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Nancy Caroline’s Emergency Care in the Streets, Seventh Edition
Chapter 24: Hematologic Emergencies
Chapter 24
Hematologic Emergencies
Unit Summary
This chapter provides a basic understanding of the hematopoietic system (the blood components
and the organs involved in their development and production) and hematologic disorders. The
hematopoietic system consists of organs and tissues, primarily bone marrow, spleen, and lymph
nodes, involved in the production of blood components.
National EMS Education Standard Competencies
Medicine
Integrates assessment findings with principles of epidemiology and pathophysiology to formulate
a field impression and implement a comprehensive treatment/disposition plan for a patient with a
medical complaint.
Hematology
Anatomy, physiology, pathophysiology, assessment, and management of
• Sickle cell crisis (pp 1252-1253)
• Clotting disorders (pp 1256-1257)
Anatomy, physiology, epidemiology, pathophysiology, psychosocial impact, presentations,
prognosis, and management of common or major hematological diseases and/or emergencies
• Sickle cell crisis (p 1252)
• Blood transfusion complications (pp 1257-1258)
• Hemostatic disorders (p 1257)
• Lymphomas (p 1255)
• Red blood cell disorders (pp 1253, 1256)
• White blood cell disorders (pp 1254-1255)
• Coagulopathies (pp 1256-1257)
Knowledge Objectives
1. Discuss the composition and functions of blood’s essential components: red blood cells,
white blood cells, and platelets. (pp 1245-1248)
2. Summarize the role of white blood cells in the normal inflammatory process.
(pp 1245, 1246- 1247)
3. Distinguish between cell-mediated immunity and humoral immunity. (p 1247)
4. Define hemostasis, and discuss the mechanisms essential to its maintenance in the body.
(pp 1248-1249)
5. Outline the steps in the primary assessment and management of a patient with a
hematologic disorder. (pp 1249-1250)
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6. Describe the pathophysiology, assessment, and management of sickle cell disease. (pp
1252-1253)
7. Define three types of sickle cell crisis. (p 1252)
8. Outline the pathophysiology, assessment, and management of other common diseases and
conditions of the blood, including anemia, leukopenia, thrombocytopenia, leukemia,
lymphomas, polycythemia,disseminated intravascular coagulation (DIC), hemophilia, and
multiple myeloma. (pp 1253-1257)
9. Discuss the causes, symptoms, assessment, and management of blood transfusion
complications. (pp 1257-1258)
Skills Objectives
There are no skill objectives for this chapter.
Readings and Preparation
Review all instructional materials including Chapter 24 of Nancy Caroline’s Emergency Care in
the Streets, Seventh Edition, and all related presentation support materials.
Support Materials
• Lecture PowerPoint presentation
• Case Study PowerPoint presentation
Enhancements
• Direct students to visit the companion website to Nancy Caroline’s Emergency Care in the
Streets, Seventh Edition, at http://www.paramedic.emszone.com for online activities.
• Leukemia information from the national cancer society:
http://www.cancer.gov/cancertopics/types/leukemia
• Tour a dialysis center (if available) to discuss cleansing methods and processes involved with
mechanically cleansing the blood of toxins.
• Tour a blood bank (if available) to discuss blood typing, cross-matching, and plasma separation.
• Content connections: Hematology is the basis for cellular respiration and the effective function
of all body systems. Content from every chapter can be added to the discussion of hematology.
• Cultural considerations: Sickle cell conditions typically are related to ethnicity. Typically men
are hemophiliacs more than women. Discuss the cultural implications as necessary.
Teaching Tips
Hematology can be a very “dry” topic. Use local resources such as blood banks, hospital labs,
and cancer centers to bring real-world experience to the discussion.
Unit Activities
Writing activities: Have students select an organ involved in the production of blood
components. Students should prepare a paper discussing the organ, how it works to supply a
blood component and how the organ works within the hematologic system.
Student presentations: Assign a hematologic condition to the students. Students will research
and prepare a presentation covering typical signs, symptoms, and patient chief complaint types.
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Chapter 24: Hematologic Emergencies
Group activities: Develop simulated lab results for a number of patients. Provide each group
with the lab results. The group will need to determine if the simulated lab values are within
normal limits. If the result is not normal, the group will need to give the normal range and
discuss any potential conditions the patient may delevop.
Visual thinking: Have students draw the circulatory system as a review of the anatomy chapter.
Students will need to add the organs to the circulatory system drawing to indicate blood
production points.
Pre-Lecture
You are the Medic
“You are the Medic” is a progressive case study that encourages critical-thinking skills.
Instructor Directions
Direct students to read the “You are the Medic” scenario found throughout Chapter 24.
• You may wish to assign students to a partner or a group. Direct them to review the
discussion questions at the end of the scenario and prepare a response to each question.
Facilitate a class dialogue centered on the discussion questions and the Patient Care Report.
• You may also use this as an individual activity and ask students to turn in their comments
on a separate piece of paper.
Lecture
I. Introduction
A. Most EMS systems rarely respond to hematologic emergencies.
1. Hematologic disorder: Any disorder of the blood
a. Hemolytic disorders
i. Disease processes that cause the breakdown of red blood cells
b. Hemostatic disorders
i. Bleeding and clotting abnormalities
2. A basic understanding of the hematopoietic system and hematologic disorders will help
you to respond to these kinds of emergencies.
a. The hematopoietic system consists of organs and tissues involved in the production
of blood components.
i. Primarily bone marrow, spleen, and lymph nodes
II. Anatomy and Physiology
A. Blood and plasma
1. Blood performs the following functions:
a. Respiratory function
i. Transports oxygen from the lungs to the tissues
ii. Transports carbon dioxide from the tissues to the lungs
b. Nutritional function
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Chapter 24: Hematologic Emergencies
i. Carries nutrients (glucose, proteins, fats) from the digestive tract to cells
c. Excretory function
i. Ferries metabolic waste from the cells to the excretory organs
d. Regulatory function
i. Transports hormones to their target organs
ii. Transmits excess internal heat to the surface of the body to be dissipated
e. Defensive function
i. Carries defensive cells and antibodies, which protect the body against foreign
organisms
2. Blood is made up of two components:
a. Plasma
i. 92% water and 6% to 7% proteins
ii. Accounts for 55% of total blood volume
b. Formed elements
i. Account for 45% of total blood volume
ii. Include:
(a) Red blood cells (RBCS), or erythrocytes
(b) White blood cells (WBCs), or leukocytes
(c) Platelets, or thrombocytes
iii. 99% are RBCs
3. The production of RBCs occurs within stem cells.
a. Stimulated by erythropoietin, a protein secreted by the kidneys in response to
circulatory need.
b. RBCs may take as long as 5 days to mature and have an average life of 4 months.
c. Within RBCs, iron-rich hemoglobin carries oxygen to the tissues.
i. Oxygen gives blood its red color.
4. Three laboratory tests are commonly performed on blood:
a. RBC count
i. Measures the number of RBCs in a blood sample
b. Hemoglobin level
i. Identifies the amount of hemoglobin found within the RBCs
c. Hematocrit measurement
i. Gives the overall proportion of RBCs in the blood.
ii. Blood is considered balanced if:
(a) The hemoglobin level is one third of the hematocrit level
(b) The RBC count is one third of the hemoglobin level
5. White blood cells
a. Larger than RBCs
b. Provide immunity against “foreign invaders.”
c. Derived from stem cells
i. Cells that develop into other types of cells in the body
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d. Several types exist and perform a specific task to maintain the immune system.
6. Platelets
a. Smallest of the formed elements
b. Responsible for clotting the blood
i. Form the initial plug following a vascular injury.
ii. Clotting proteins then toughen and complete the blood clot.
c. Hemostasis is the process that allows the body to stop bleeding.
i. Vascular spasm
ii. Coagulation
iii. Platelet plugging
d. Approximately two thirds circulate through the blood.
i. The rest are stored in spleen.
e. Average life span: 5 to 11 days
B. Blood-forming organs and RBC production
1. The major players in the hematologic system are:
a. Bone marrow
i. Primary site for cell production
ii. Found in most of the long bones and the pelvis, skull, and vertebrae
b. Liver
i. Produces the clotting factors found in the blood
ii. Filters the blood
iii. Removes toxins
iv. Essential to normal metabolism and homeostasis
v. Highly vascular
c. Spleen
i. Also vascular
ii. Filters and breakdowns erythrocytes
iii. Assists with lymphocyte production
iv. Helps provide homeostasis and infection control
v. If removed, the platelets formed afterward remain in the blood throughout their
life span.
C. The inflammatory process
1. At birth, all of the body’s cells and blood contain antigens.
a. Antigens: Substances that can activate the immune system
i. The body keeps these in memory.
ii. If a foreign antigen is not identified, the body produces antibodies to counteract
it.
iii. Autoimmune diseases cause the body to identify its own antigen as a foreign
body.
D. The immune system
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1. Processes protect the body from pathogens and other unwanted material.
a. Must distinguish the body’s own tissue from outside organisms
2. Consists of two types of immunity:
a. Native (innate) immunity
i. Provides a nonspecific, maximal response to any disturbance
ii. Operates as the first line of defense
iii. Usually associated with the initial inflammatory response
b. Acquired immunity
i. Specific to vertebrates
ii. Provides a pathogen-specific response when the body is exposed to a foreign
substance or disease and produces antibodies
3. Immune system responses can be categorized as:
a. Humoral immunity
i. The secretion of antibodies (immunoglobulins) that recognize a specific antigen
b. Cell-mediated immunity
i. Macrophages and T cells attack and destroy pathogens or foreign substances.
4. WBCs are produced in the bone marrow.
a. A small number are always circulating in the bloodstream.
b. A larger reserve is ready whenever pathogens are detected.
c. Laboratory values can provide information on the status of the immune system.
i. Higher levels generally indicate the possible presence of infection.
ii. In severe infections the count can drop as destruction exceeds production.
d. The baseline count is measured when the body is in a normal state.
i. Normal range is 5,000 to 10,000 cells/µL.
ii. Women usually have higher counts, due to changes during menstrual periods.
E. Blood classifications
1. Developed to ensure compatibility and prevent medical problems during blood
component replacement
2. ABO system
a. RBC classification types are “O,” “A,” “B,” and “AB”
i. Indicate the antigens found in the plasma membrane
b. Type O blood
i. Has no ABO antigens, so it can be given to anyone
ii. “Universal donor”
c. Type AB blood
i. Has no ABO antibodies
ii. Can receive blood from any donor without having an ABO reaction
iii. “Universal recipient”
3. Blood contains a secondary antigen called the Rh antigen
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a. In the U.S., 85-90% of Caucasians and African Americans have it
b. In pregnancy, a woman without the antigen (Rh-) who is carrying a fetus with the
antigen (Rh+) might develop antibodies against the fetus.
i. Results in hemolytic disease
F. Hemostasis
1. The process of stopping bleeding through:
a. Vasoconstriction (immediate response)
i. Narrowing of the blood vessel to cut off flow at the affected site
ii. Locally, vasoconstrictors are released.
iii. If the hemorrhage is a significant, the adrenal glands release epinephrine.
(a) A potent vasoconstrictor
(b) Leads to systemic vasoconstriction
b. Platelet plugging
i. Platelets are small cellular fragments that stick to collagen and become activated.
(a) Release chemicals to draw additional platelets to the injury site.
(b) Causes a plug to form, helping to stop the bleeding
c. Coagulation
i. Involves a dozen clotting factors that are activated when the body is injured
(a) Each requires the presence and activation of the preceding factor to work.
ii. Clots are made up of fibrin.
(a) Calcium holds the fibrin fibers together.
2. Clotting cascade
a. The process by which clotting factors work together to form fibrin
b. Can be initiated through:
i. Intrinsic pathway: Triggered by elements within the body
ii. Extrinsic pathway: Activated by tissue damage outside of the blood vessels
themselves
c. Coagulopathy: Any process that interferes with the clotting cascade or hemostasis
i. Bleeding disorders that can lead to heavy or prolonged bleeding
III. Patient Assessment
A. During the primary assessment of a patient suspected of having a hematologic
disorder, note any signs and symptoms that may be immediately life threatening.
1. Ask in-depth and relevant questions about the patient’s history and SAMPLE history.
a. Follow up on the responses.
b. A nonjudgmental approach is essential to assure patients they will not be treated
differently because of their condition.
B. Scene size-up
1. Ensure the scene is safe for entry.
2. Consider the mechanism of injury.
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3. Determine the number of patients.
4. Assess for hazards and the need for additional help.
5. Gloves and eye protection are the standard minimum precautions.
C. Primary assessment
1. An African American or Mediterranean patient who reports severe pain may have
undiagnosed sickle cell disease.
a. Perform cervical spine stabilization, if necessary.
b. Even if a person has sickle cell disease, it may not be what is causing the current
problem.
c. Always perform a thorough, careful primary assessment.
2. Form a general impression.
a. Perform a rapid scan of the patient.
i. How does the patient look?
(a) Anxious, restless, or listless?
(b) Apathetic or irritable?
b. Determine the level of consciousness.
3. Airway and breathing
a. Patients showing signs of inadequate breathing or altered mental status should
receive:
i. High-flow oxygen at 12 to 15 L/min via a nonrebreathing mask or
ii. Ventilation via bag-mask
b. Patients experiencing a sickle cell crisis may have:
i. Increased respirations from severe pain
ii. Signs of pneumonia
4. Circulation
a. An increased pulse rate may indicate a compensatory mechanism.
i. “Forcing” the sickled cells through smaller vasculature
b. Look for signs of shock.
c. Manage life-threatening conditions, and provide transport to an appropriate facility.
d. If hemophilia is suspected, watch for:
i. Signs of acute blood loss
ii. Any bleeding of unknown origin
iii. Signs of hypoxia or shock
5. Transport decision
a. Depends on severity of condition and patient’s wishes
b. If a patient is experiencing a sickle cell crisis or uncontrolled bleeding:
i. Transport to the closest appropriate facility.
D. History taking
1. You may need to be overly inquisitive about the patient’s history and SAMPLE history.
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2. Do not take a call for a sickle cell crisis lightly.
a. Patients are often in life-threatening situations, characterized by:
i. Shortness of breath
ii. Signs of pneumonia
iii. Skin showing inadequate perfusion
iv. Accompanying hypotension
v. Swelling of the fingers and toes
vi. Priapism
vii. Jaundice
3. Look for:
a.
b.
c.
d.
e.
f.
g.
h.
i.
j.
k.
Changes in level of consciousness
Vertigo
Feelings of fatigue
Syncopal episodes
Dyspnea
Chest pain
Changes in pulse rate and rhythm
Coughing up blood
Visual disturbances
Muscle pain
Stiffness
4. Ascertain whether pain is isolated or felt throughout the entire body.
5. Has the patient experienced:
a.
b.
c.
d.
e.
f.
Skin changes?
Bleeding?
History of liver problems?
Pain for unknown reasons?
Problems with the genitourinary system?
Gastrointestinal problems?
6. In a patient with known sickle cell disease, obtain a SAMPLE history and ask:
a.
b.
c.
d.
Have you had a crisis before?
When was the last time you had a crisis?
How did it resolve?
Have you had any illness, unusual amount of activity, or stress lately?
E. Secondary assessment
1. May be performed on scene, en route, or not at all
a. Depends on transport time and patient condition
2. When treating a patient with a blood disorder, perform a physical exam.
a. Systematically examine the patient.
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b. Focus on major joints where cells congregate.
3. Obtain baseline vital signs.
a. Oxygen saturation reading may be inaccurate due to an anemic state.
4. Evaluate and document mental status using the AVPU scale.
5. In patients experiencing a sickle cell crisis:
a. Respirations are normal to rapid.
b. Pulse is weak and rapid.
c. Skin is pale and clammy with a low blood pressure.
F. Reassessment
1. Reassess frequently to determine if their condition has changed.
2. Administer supplemental oxygen via a nonrebreathing mask at 12 to 15 L/min.
a. Hypersaturates the remaining hemoglobin
b. Increases level of perfusion
3. Provide ventilation when respirations are insufficient.
4. Place the patient in a position of comfort, and maintain body temperature.
5. According to local protocol, administer:
a. IV fluid for hydration
b. Nitrous oxide for pain
6. Contact the hospital to help sort signs and symptoms of a true sickle cell crisis from other
causes of pain.
7. Prehospital care for a patient with hemophilia can include IV therapy to treat
hypotension.
a. Provide appropriate supplemental oxygen.
b. Cover the patient to maintain body temperature.
8. Inform hospital staff about:
a.
b.
c.
d.
Patient history
Present situation
Assessment findings
Interventions and their results
9. Thoroughly document:
a.
b.
c.
d.
e.
Each assessment
Your findings
Treatment
Time of interventions
Any changes in the patient’s condition.
IV. Emergency Medical Care
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A. Emergency medical care for problems related to a blood disorder should
include:
1. Oxygen
a. Determined by the severity of the patient’s condition and respiratory status
2. Fluids
3. Electrocardiogram (ECG)
4. Transport
5. Comfort
6. Pharmacology
7. Psychological support
V. Pathophysiology, Assessment, and Management of
Specific Emergencies
A. Hematologic disorders are serious and often life threatening.
1. Over 245,000 people in the United States are afflicted with hematologic disorders.
B. Sickle cell crisis
1. Pathophysiology
a. Leading inherited blood disorder.
i. Primarily affects African American, Puerto Rican, and European populations
ii. In the U.S., 70,000-100,000 Americans have sickle cell disease.
iii. Average life expectancy is 45 years.
(a) In general, women tend to live slightly longer than men.
b. Begins with a gene defect of the adult-type hemoglobin (HbA)
i. Can be inherited from both parents (HbSS) or one parent (HbS)
(a) When inherited from both parents, there is a high probably offspring will
have the disease or carry the mutation.
c. Defective RBCs have an oblong shape instead of a smooth, round shape.
i. Makes the RBC a poor oxygen carrier, increasing risk of hypoxia
ii. Have a much shorter life span, increasing risk of anemia
d. Sickle cell disease can lead to:
i. Aplastic crisis: Body temporarily stops RBC production, causing the patient to
become easily tired, anemic, pale, and short of breath
ii. Hemolytic crisis: Acute red blood cell destruction leading to jaundice
e. The odd shape can cause RBCs to lodge in small blood vessels.
i. Leads to thrombosis
f. May manifest in several ways:
i. Vaso-occlusive crisis: Blood flow to an organ becomes restricted.
(a) Causes pain, ischemia, and often organ damage
(b) Usually lasts between 5-7 days
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(c) Spleen is frequently affected due to its narrow vessels and function of
removing damaged RBCs
ii. Acute chest syndrome: Vaso-occlusive crisis associated with pneumonia
(a) Common signs and symptoms include chest pain, fever, and cough.
iii. Splenic sequestration crisis: Sickle cells within the spleen block blood from
leaving the spleen.
(a) Results in painful, acute enlargement of the spleen and a hard, bloated
abdomen
(b) Acute splenic sequestration syndrome: RBCs become trapped in the spleen.
(1) Causes a dramatic fall in hemoglobin available in circulation
(2) Patients present with a painful, acute abdomen and sudden weakness,
pallor, tachypnea, and tachycardia.
(3) Usually occurs in infants or toddlers
2. Assessment
a. Characterizations of a sickle cell crisis may include:
i. Shortness of breath
ii. Signs of pneumonia
iii. Inadequate perfusion of the skin, accompanied by hypotension
iv. Signs of jaundice
v. Yellowing in the eye (icteric sclera)
vi. Mild dehydration
b. In acute crises, patients may have significant pain and multiple system involvement.
c. Pediatric patients typically present with initial pain in the hands and feet.
d. Adults typically report back and proximal extremity pain.
3. Management
a.
b.
c.
d.
Administer high levels of oxygen to prevent further destruction from hypoxia.
Rapidly transport patient to an appropriate facility.
Consider IV fluid therapy if necessary.
Maintain the patient’s body temperature.
i. Cold can contribute to sickling of cells.
e. Patients may have a high pain threshold and require a higher level of analgesia.
f. Recommend that the patient rest during transport.
C. Anemia
1. Pathophysiology
a. Hemoglobin or erythrocyte level that is lower than normal
i. Usually associated with:
(a) Underlying disease process
(b) Acute or chronic blood loss
(c) Decreased production or increased destruction of erythrocytes
(d) Preexisting hemolytic disorder
b. Iron deficiency anemia
i. The most common type of anemia
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ii. Typical causes include:
(a) Gastrointestinal blood loss
(b) Menstrual bleeding
(c) Blood loss from frequent donations or diagnostic tests
(d) Premature birth or low birth weight (in children)
c. May be caused by an inherited hemolytic disorder (sickle cell disease or thalassemia)
i. RBCs develop rigid and deformed membranes.
(a) May become lodged in small blood vessels, leading to thrombosis
d. May be caused by hematologic disorders resulting from deficiency of glucose-6phosphate dehydrogenase
i. Enzyme helps protect RBCs during infections.
e. The most common type of acquired anemia develops when the RBC flow is disrupted
from problems with blood vessel linings or blood clots.
i. Hypoxia
ii. Difficulty breathing
iii. Chest pain
2. Assessment
a. Most patients will complain of:
i. Feeling worn down
ii. Having no energy
iii. Feeling as if they overexerted themselves
iv. Being unable to “catch their breath.”
b. Patients may also have:
i. Anginal-type chest pain related to reduced oxygen to the heart
ii. Leukopenia (reduction in WBCs)
iii. Thrombocytopenia (reduction in platelets)
3. Management
a. Check and monitor the airway and breathing closely.
i. Administer high-flow oxygen when necessary.
b. Check vital signs frequently.
c. In cases of chest pain, apply a cardiac monitor.
i. A 12-lead ECG may be needed.
d. Blood pressure management may be needed, along with fluid replacement therapy.
i. IV fluids can induce unwanted or unexpected bleeding.
e. Allow the patient to rest in a comfortable position.
f. Transport to the closest appropriate facility.
g. Consider rapid transport if any of the following develop:
i. An abrupt change in consciousness
ii. Hypotension
iii. Other significant perfusion inadequacies
D. Leukemia
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1. Pathophysiology
a. Cancer that develops in the lymphoid system
b. Blood cells (particularly WBCs) develop abnormally and/or excessively.
c. Can cause:
i. Anemia
ii. Thrombocytopenia (decrease in platelets)
iii. Leukocytosis (increased WBCs)
d. Patients experience frequent bleeding, bruising, infections, and fever.
e. Can be classified as acute or chronic
i. Develops more frequently in people 65 years or older
ii. Acute leukemia: Bone marrow is replaced with abnormal lymphoblasts.
iii. Chronic leukemia: Abnormal mature lymphoid cells accumulate in the bone
marrow, lymph nodes, spleen, and peripheral blood.
(a) Typically found by chance during routine blood tests
f. Survival depends on several factors:
i. What stage the disease is detected
ii. The patient’s underlying medical condition
iii. Response to treatment
g. Treated with chemotherapy and radiation therapy
i. In most cases, treatment results in remission.
ii. Approximately 80% of children will be cured when diagnosed and treated early.
2. Assessment
a. Patient presentation depends on:
i. The stage of leukemia
ii. Current treatment
b. Patients may present with:
i. Fatigue
ii. Headaches
iii. Dyspnea
iv. Signs of neurologic defects
v. Fever
vi. Bone pain
vii. Diaphoresis
viii. Feeling full
ix. Soreness in the mid part of the chest
x. Unexplained bleeding
c. Monitor all basic vital signs and the cardiac rhythm.
i. Hypotension and tachycardia are often present.
3. Management
a. Provide airway support and oxygen therapy as appropriate.
b. IV fluid therapy and analgesics may be needed.
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c. Patients may need constant positive support.
d. You may be called because the patient’s condition has deteriorated and loved ones
are uncertain about what to do.
i Discuss the situation with medical control.
ii. Document all findings before leaving.
iii. Have a refusal and/or release form signed if transport is declined.
e. Be alert to rapid changes in condition.
f. If you transport, be aware the patient could go into arrest.
i. Make sure you understand the patient’s and family’s wishes about what to do.
E. Lymphomas
1. Pathophysiology
a. Group of malignant diseases that arise within the lymphoid system
b. Classified in two categories:
i. Non-Hodgkin lymphoma
(a) Can occur at any age
(b) Can be hereditary
(c) May be characterized based on progression: Indolent, aggressive, or highly
aggressive
(1) Indolent: Disease may never leave the lymphoid system
(2) Highly aggressive: May affect multiple organs in a short period
ii. Hodgkin lymphoma
(a) Painless, progressive enlargement of the lymphoid glands
(b) Most commonly affects the spleen and lymph nodes
(c) Highly rare form
(d) Expected to have some hereditary components
(e) Incidence has two peaks: Between 15-35 years of age and after age 55-60
(f) Twice as common in men as in women
(g) Symptoms may not occur for years, but may include:
(1) Night sweats
(2) Chills
(3) Persistent cough
(4) Swelling of various lymph nodes
(5) Loss of appetite
(6) Significant weight loss
(7) Generalized itching
(8) Fatigue
(9) Bone pain
(h) With aggressive treatment, symptoms may disappear for long periods.
2. Assessment
a. Require specialized levels of chemotherapy or radiation
b. During assessment, ask questions like:
i “What type of cancer do you have?”
ii. “What type of treatment are you receiving?”
c. Signs and symptoms may include:
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Chapter 24: Hematologic Emergencies
i.
ii.
iii.
iv.
Pallor
Feeling hot and then cold or both in different areas of the body
Inadequate perfusion
Abnormal ECG rhythms
3. Management
a.
b.
c.
d.
e.
Pain management may need to be aggressive.
Treat inadequate perfusion with fluid therapy, and provide supplemental oxygen.
Treat any abnormal heart rhythms.
If the condition does not improve or deteriorates, initiate rapid transport.
You may be called to offer support but not transport.
i. Be supportive
ii. Discuss your findings with medical control.
iii. Explain options to the patient and family, and allow them to make a decision.
F. Polycythemia
1. Pathophysiology
a. An overabundance or overproduction of RBCs
b. Can be caused by:
i. A rare disorder originating in a single stem cell
ii. An existing disease
iii. Living in high-altitude areas for long periods
c. May lead to other conditions such as:
i. Strokes
ii. Transient ischemic attacks
iii. Headaches
iv. Abdominal pain
d. Cases more frequent in adults 60-80 years old
e. Clinical treatment usually include phlebotomy to maintain hematocrit levels.
i. Less than 45% in men
ii. Less than 42% in women
f. Other treatments include cancer-type therapy.
g. Survival is less than 18 months when the disease goes untreated.
2. Assessment
a. Findings may vary widely, and may include:
i. Altered levels of consciousness
ii. Hypoxia
iii. Respiratory distress
iv. Changes in peripheral pulses, pulse rate, and skin color
v. Tachycardia
vi. Purplish skin with red hands and feet
b. Note the extent and duration of dyspnea.
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Chapter 24: Hematologic Emergencies
c. Has the patient experienced uncontrolled itching (pruritus)?
d. Has the patient noted changes in skin temperature?
e. Obtain a thorough medical history.
3. Management
a. Prehospital care consists of supportive care and transporting to an appropriate
facility.
b. Administer oxygen as needed.
c. Establish IV access for possible pharmacologic interventions.
G. Disseminated intravascular coagulation
1. Pathophysiology
a. May be caused by a number of life-threatening conditions
b. Progresses in two stages.
i. First stage: Free thrombin and fibrin deposits in the blood increase.
(a) Platelets begin to aggregate.
(b) Defibrination (breakdown of the fibrin clots) occurs.
ii. Second stage: Uncontrolled hemorrhage results from the reduction in clotting
factors.
c. Mortality is high, especially in acute cases.
i. Some studies have shown up to 75% mortality.
ii. Relates to uncontrolled bleeding, hypotension, and shock
2. Assessment
a. Identify signs and symptoms associated with DIC or progression toward it.
b. Patients may have:
i. Episodes of respiratory difficulty
ii. Signs of shock
iii. Skin changes
3. Management
a.
b.
c.
d.
e.
f.
Identify the underlying cause of the condition, and establish treatment early.
Maintain an airway.
Administer supplemental oxygen.
Treat the patient for shock.
Pharmacologic interventions may include pain management and treatment for
abnormal heart rhythms.
Be optimistic but honest.
H. Hemophilia
1. Pathophysiology
a. A bleeding disorder in which clotting does not occur or occurs insufficiently
i. Usually associated with an X-linked recessive inheritance pattern
b. Classified into two primary types:
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i.
Type A: Due to low levels of factor VIII (antihemophilic globulin and
antihemophilic factor)
ii. Type B: A deficiency of factor IX (plasma thromboplastin component/the
Christmas factor)
c. Primarily found in males
d. Signs and symptoms are the same and may include:
i. Acute and chronic bleeding
ii. Spontaneous intracranial bleeding
e. Patients with significant acute bleeding episodes may require:
i. Hospitalization for transfusion
ii. Infusion of factors VIII and IX
2. Assessment
a. Take care of the ABCs.
b. Be alert for signs of acute blood loss.
i. Pallor
ii. Weak pulse
iii. Hypotension
c. Note any bleeding of unknown origin.
i. Nosebleeds
ii. Bloody sputum
iii. Blood in the urine or stool (melena)
d. Patients may show signs of hypoxia.
3. Management
a. Any patient complaining of respiratory problems should receive high-flow oxygen.
b. Note ECG findings.
i. Treat symptomatic dysrhythmias as appropriate.
c. In cases of unstable hypotension, IV therapy may be necessary.
d. Analgesics may be appropriate.
e. If the bleeding stopped before you arrived, suggest the patient seek immediate
follow-up.
I. Multiple myeloma
1. Pathophysiology
a. The number of plasma cells in the bone marrow increases abnormally, forming
tumors in the bone.
i. Impairs normal bone marrow function
ii. RBC, WBC, and platelet formation is decreased.
iii. Results in anemia and susceptibility to infection.
b. Neoplastic cells may also accelerate protein development in the bloodstream.
i. Leads to organ failure
c. Disease occurs mostly in patients older than 40.
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d. As disease progresses, patients may have weakness in the bones.
i. Results in spontaneous fractures
ii. Pain in the bones and back is common.
e. In advanced cases, chemotherapy and other anticancer-type treatment may be given.
2. Assessment
a. Findings depend on the stage of the disease.
i. Early stage complaints may be fatigue or mild pain.
ii. Later-stage may be evidenced by:
(a) Unexplained hemorrhage
(b) Significant weight loss
(c) Frequent bone fractures
(d) Pain in various locations
3. Management
a.
b.
c.
d.
e.
IV fluid therapy
Pain management
Supportive care
Don’t assume the patient is ready to or is going to die.
Definitive care may improve the patient’s condition.
J. Transfusion reactions
1. Pathophysiology
a. Occur in approximately 1 in 250,000 RBC transfusions
b. Similar to an anaphylactic reaction
i. Occur rapidly
ii. Can cause severe circulatory collapse
c. Monitor a patient receiving a blood transfusion very closely for the first 30-60
minutes.
d. Determine a patient’s blood type and the type of blood received.
i. A transfusion reaction occurs when a patient receives a blood type different than
their own.
2. Assessment
a. Signs and symptoms may be subtle in an unresponsive or intubated patient.
b. In the acute reaction, the patient experiences a rapid onset of:
i. Chills
ii. Fever
iii. Back pain
iv. Vomiting
v. Tachycardia
vi. Hypotension
c. Reactions may be delayed up to 7 days.
d. Complications include the following categories:
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i.
ii.
iii.
iv.
v.
vi.
Hemolytic
(a) The greatest threat during blood transfusion
(b) Primarily caused by incompatibility between recipient and donor blood
Febrile
(a) Most common complication
(b) Usually benign
(c) Treated with an antipyretic and observation.
Allergic
(a) Anaphylactic reaction to preservatives or other agents in the product being
transfused
(b) Onset is usually within the first few minutes of transfusion.
Transfusion-related lung injury
(a) Noncardiogenic pulmonary edema caused by increased capillary
permeability post transfusion
(b) Treatment is focused on supporting the ABCs.
Circulatory overload
(a) Typically occurs in patients with preexisting cardiomyopathy or ventricular
dysfunction
(b) Treatment consists of relieving the system of its excess fluids through
diuresis and the use of oxygen, nitrates, and morphine.
Bacterial infection
(a) Typically a result of poor blood product handling or contamination during
the infusion process
(b) Can lead to full systemic sepsis
3. Management
a. The severity of the reaction correlates to the amount of blood volume transfused.
b. Care is centered on:
i. Immediately stopping the transfusion
ii. Providing hemodynamic supportive care to counteract shock
iii. Maximizing kidney perfusion
c. A patient in hemodynamically unstable condition requires:
i. Early invasive monitoring
ii. Vasopressors
iii. Promotion of diuresis with isotonic fluids and a loop diuretic (furosemide)
d. Dopamine may be helpful.
e. High-flow oxygen should be administered.
f. Administration of epinephrine and diphenhydramine should be considered.
VI. Summary
A. Most EMS systems rarely respond to hematologic emergencies.
B. Blood performs respiratory, nutritional, excretory, regulatory, and defensive
functions.
C. Blood is made up of plasma and formed elements, including RBCs, WBCs, and
platelets.
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D. Common lab blood tests are red blood cell count, hemoglobin level, and
hematocrit measurement.
E. Blood tests measuring subtypes of WBCs can provide valuable information
about the status of the immune system.
F. The ABO system is commonly used to classify blood types.
G. During the primary assessment of a patient with a hematologic disorder, note
any signs and symptoms that may be immediately life threatening.
H. While taking a history and during the secondary assessment, look for changes in
the level of consciousness, including vertigo, fatigue, or syncopal episodes.
I. General blood disorder management should include oxygen, fluids, ECG,
transport, medications, and psychological support.
J. Hematologic disorders include sickle cell crisis, anemia, leukopenia,
thrombocytopenia, leukemia, lymphomas, polycythemia, DIC, hemophilia,
multiple myeloma, and complications of blood transfusions.
K. A patient experiencing a sickle cell crisis will experience significant pain due to
congested vessels and may have a serious infection that could lead to sepsis and
death.
L. A patient with anemia has a low hemoglobin or RBC level. Anemia may be
caused by an underlying hematologic or hemolytic disorder.
M. Leukopenia is a reduction in the number of WBCs. Thrombocytopenia is a
reduction in the number of platelets. Both are often seen in patients with anemia
or leukemia.
N. Leukemia is a cancer that affects the production of WBCs. Patients often
experience bleeding, bruising, infections, and fever.
O. Lymphomas are a group of malignant disorders that occur within the lymphoid
system. The two types are non-Hodgkin (most common) and Hodgkin
lymphoma.
P. Polycythemia is an overabundance or overproduction of RBCs, which causes
hyperviscosity of the circulatory system.
Q. Disseminated intravascular coagulation (DIC) may result from a massive injury,
sepsis, or obstetric complications. In the first stage, too much blood clotting
results from an overactivated coagulation system. In the second, the body’s
natural reaction to breaking up the clots causes uncontrolled hemorrhage.
R. Hemophilia is a bleeding disorder found primarily in males in which clotting
does not happen or is insufficient. Type A is due to a low level of factor VIII, and
type B is due to a deficiency in factor IX.
S. Multiple myeloma is a cancer of the bone marrow caused by malignant plasma
cells.
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T. Complications of blood transfusions are similar to anaphylactic reactions. They
are caused by a mismatch of the patient’s blood type to that received or an
allergic reaction to preservatives or agents in the transfused product.
Post-Lecture
This section contains various student-centered end-of-chapter activities designed as
enhancements to the instructor’s presentation. As time permits, these activities may be presented
in class. They are also designed to be used as homework activities.
Assessment in Action
This activity is designed to assist the student in gaining a further understanding of issues
surrounding the provision of prehospital care. The activity incorporates both critical thinking and
application of paramedic knowledge.
Instructor Directions
1. Direct students to read the “Assessment in Action” scenario located in the Prep Kit at the
end of Chapter 24.
2. Direct students to read and individually answer the quiz questions at the end of the
scenario. Allow approximately 10 minutes for this part of the activity. Facilitate a class
review and dialogue of the answers, allowing students to correct responses as may be
needed. Use the quiz question answers noted below to assist in building this review.
Allow approximately 10 minutes for this part of the activity.
3. You may wish to ask students to complete the activity on their own and turn in their
answers on a separate piece of paper.
Answers to Assessment in Action Questions
1. Answer: B.
antigens
Rationale: At birth, all of the body’s cells and blood contain antigens—substances
within the body that can activate the immune system. Typically, the body keeps these
antigens in memory and, when exposed to something for which it already has antibodies,
will not initiate a reaction (see the chapter on Pathophysiology). However, when the body
is exposed to something it cannot identify in its base antigens, it produces antibodies to
counteract the foreign or unidentified antigen.
2. Answer: A.
ABO
Rationale: To ensure compatibility and prevent medical problems during blood
component replacement, blood type classifications have been developed. In the ABO
system, the RBC classification types are “O,” “A,” “B,” and “AB”; they indicate which
antigens are found in the plasma membrane.
3. Answer: D.
Rh antigen
Rationale: Blood contains a secondary antigen, known as the Rh antigen (the name
signifies that the antigen was first found in the Rhesus monkey). In the United States,
85% to 90% of all whites and African Americans have this antigen. Thus, if a person has
the blood type A-positive (A+), the blood contains the Rh antigen.
4. Answer: B.
anaphylaxis
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Rationale: Blood reactions are similar to an anaphylactic reaction—they occur rapidly
and can cause severe circulatory collapse and even death. When a patient receives a
blood transfusion, it is important to monitor the patient very closely for the first 30 to 60
minutes because transfusion reactions typically begin within this time frame. In general,
the severity of the reaction directly correlates to the amount of blood volume transfused.
5. Answer: B.
stop transfusion, counteract shock, kidney perfusion
Rationale: While there is no specific antidote or remedy for a transfusion reaction, care
is centered on immediately stopping the transfusion process, hemodynamic supportive
care to counteract shock, and maximizing kidney perfusion. In a patient in
hemodynamically unstable condition, early invasive monitoring, vasopressors, and the
promotion of diuresis with isotonic fluids and a loop diuretic (furosemide) are indicated.
6. Answer: A.
2 to 4 µg/kg/min
Rationale: The effects of dopamine are dose-dependent. A dopaminergic response,
meaning dilation of the vessels in the kidneys and increased urine output, is produced by
2 to 4 µg/kg/min. A dose of 4 to 10 µg/kg/min creates a beta-adrenergic response,
meaning there will be positive chronotropic and inotropic effects; 10 to 20 µg/kg/min
creates an adrenergic response: primary alpha stimulation and vasoconstriction will
occur. A dose greater than 20 µg/kg/min results in reversal of renal effects and an
override of alpha effects. This information can be reviewed in the chapter on Medication
Formulary.
Additional Questions
7. Rationale: Sickle cell disease is—by far—the leading inherited blood disorder. Although
it primarily affects African American, Puerto Rican, and European populations, it can
occur in anyone. In the United States, about 1,000 infants each year are born with this
disease. The numbers are much greater in Africa, where in Nigeria alone it is estimated
that 45,000 to 90,000 infants are born with the disease each year. Also, 30% of all
newborns in Africa carry the sickle cell trait, which means they can pass the disease on to
future generations, ensuring its continuation.
8. Rationale: Patients with lymphoma may be in constant extreme pain; therefore, if pain
management is needed and available, it may have to be aggressive because the patient
will likely already be following a high-dose analgesic regimen. You may need to contact
medical control for direction of dosage. Treat inadequate perfusion with fluid therapy,
and provide supplemental oxygen. If necessary, treat abnormal heart rhythms. If the
patient’s condition does not improve or even deteriorates following these measures,
initiate rapid transport to the closest facility.
Assignments
A. Review all materials from this lesson and be prepared for a lesson quiz to be
administered (date to be determined by instructor).
B. Read Chapter 25, Immunologic Emergencies, for the next class session.
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Unit Assessment Keyed for Instructors
1. What are the five functions of blood?
Answer: (1) Respiratory—Blood transports oxygen from the lungs to the tissues and
transports carbon dioxide from the tissues to the lungs. (2) Nutritional—Blood carries
nutrients from the digestive tract to cells throughout the body. (3) Excretory—Blood
moves the waste products from the cells to the excretory organs. (4) Regulatory—Blood
transports hormones to their target organs and transmits internal heat to the surface of the
body. (5) Defensive—Blood carries defensive cells and antibodies.
(p 1245)
2. What is hemostasis?
Answer: Hemostasis is a highly complex process that allows the body to stop bleeding
through vasoconstriction, platelet plugging, and coagulation. Vasoconstriction is the
narrowing of the blood vessel to cut off blood flow at the affected site. Platelets are then
activated, releasing chemicals that cause the aggregation of additional platelets to the site
of injury. Eventually these platelets form a plug to help stop the bleeding. Finally,
thrombin converts fibrinogen to fibrin, which combined with calcium makes up a clot.
The clotting cascade refers to the process by which clotting factors work together to form
fibrin.
(pp 1248-1249)
3. What are the two classifications of immune system response?
Answer: Immune system responses can be classified into humoral immunity and cellmediated immunity. Humoral immunity refers to the secretion of anitbodies called
immunoglobulins, which recognize a specific anitgen. Cell-mediated immunity refers to
macrophages and T cells attacking and destroying pathogens or foreign substances.
(p 1247)
4. What is the ABO system?
Answer: To ensure compatibility and prevent medical problems during blood component
replacement, blood-type classifications using the ABO system was developed. The RBC
classification types in this system are “O,” “A,” “B,” and “AB”, which indicate the
antigens found in the plasma membrane. Type O blood has no ABO antigens and can be
given to anyone; people with this blood are referred to as the “universal donor.” AB
blood has no ABO antibodies, so a person with this type of blood can receive blood from
any donor without having an ABO reaction, which is why they are referred to as the
“universal recipient.”
(p 1248)
5. What is the most common inherited blood disorder?
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Chapter 24: Hematologic Emergencies
Answer: Sickle cell disease is—by far—the leading inherited blood disorder. Although
it primarily affects African American, Puerto Rican, and European populations, it can
occur in anyone. In the United States, approximately 70,000 to 100,000 Americans have
sickle cell disease. The numbers are much greater in Africa, where the World Health
Organization estimates that 200,000 infants are born with the disease each year. Mortality
at younger ages is common, with an average life expectancy of 45 years. In general,
women tend to live slightly longer than men with this disease.
(p 1252)
6. Where does leukemia develop in the body?
Answer: Leukemia is a disease that develops in the lymphoid system. In this type of
cancer, blood cells—particularly WBCs—develop abnormally and/or excessively.
Leukemia can cause anemia, thrombocytopenia (decrease in platelets), and leukocytosis
(increased WBCs). In acute leukemia, bone marrow is replaced with abnormal
lymphoblasts. In chronic leukemia, abnormal mature lymphoid cells accumulate in the
bone marrow, lymph nodes, spleen, and peripheral blood.
(p 1254)
7. What condition is characterized by an overabundance or overproduction of RBCs?
Answer: Polycythemia is characterized by an overabundance or overproduction of
RBCs. The increased RBC production can be caused by a rare disorder originating in a
single stem cell or an existing disease such as congestive heart failure or hypertension. It
can also arise in persons who live in high-altitude areas for long periods. The disease
essentially causes hyperviscosity of the circulatory system. The overabundance of the
blood components associated with polycythemia may lead to many other conditions, such
as strokes, transient ischemic attacks, headaches, and abdominal pain.
(p 1256)
8. What is disseminated intravascular coagulation (DIC)?
Answer: DIC may result from a massive injury, sepsis, or obstetric complications. In the
first stage, too much blood clotting results from an overactivated coagulation system. In
the second stage, the body’s natural reaction to breaking up these clots causes
uncontrolled hemorrhage.
(p 1256)
9. What is the name of a bleeding disorder in which clotting does not occur or occurs
insufficiently?
Answer: Hemophilia is a bleeding disorder in which clotting does not occur or occurs
insufficiently. It is usually associated with an X-linked recessive inheritance pattern,
albeit one that is poorly understood. The disease is classified into two primary types: type
A, which is due to low levels of factor VIII (antihemophilic globulin and antihemophilic
factor), and type B, which is associated with a deficiency of factor IX (plasma
thromboplastin component, also known as the Christmas factor). This disease is primarily
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Chapter 24: Hematologic Emergencies
found in the male population. The levels of factors VIII and IX determine the severity of
the disease.
(p 1257)
10. Name four categories of complications that may occur due to transfusion reactions.
Answer: Complication categories may include any four of the following:
o Hemolytic—This type of complication is the greatest threat to the patient during
blood transfusion. The primary cause is incompatibility between the recipient and the
blood donor.
o Febrile—This type of complication is the most common transfusion complication. It
is usually benign, and is treated with an antipyretic and observation.
o Allergic—A patient may experience an anaphylactic reaction to the preservatives or
other agents in the product being transfused.
o Tranfusion-related lung injury—This type of complication is a noncardiogenic
pulmonary edema caused by increased permeability post transfusion.
o Circulatory overload—This type of complication may occur due to rapid infusion of
blood products, but typically only occurs in patients with preexisting cardiomyopathy
or ventricular dysfunction.
o Bacterial infection—This complication is typically a result of poor blood product
handling or contamination during the infusion process.
(p 1258)
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Unit Assessment
1. What are the five functions of blood?
2. What is hemostasis?
3. What are the two classifications of immune system response?
4. What is the ABO system?
5. What is the most common inherited blood disorder?
6. Where does leukemia develop in the body?
7. What condition is characterized by an overabundance or overproduction of RBCs?
8. What is disseminated intravascular coagulation (DIC)?
9. What is the name of a bleeding disorder in which clotting does not occur or occurs
insufficiently?
10. Name four categories of complications that may occur due to transfusion reactions.
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