Download The Spleen

SPLENOMEGALY
THE SPLEEN
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Largest lymphoid tissue of the body
Serves two main functions
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Filters blood to remove damaged/old RBC- red pulp
Serves as secondary lymphoid tissue by removing infectious agents and
using them to activate lymphocytes- white pulp
A significant reservoir for T lymphocytes
Plays an active role in the production of IgM antibodies and
complement
Has significant role in the functional maturation of antibodies
ANATOMY OF
SPLEEN
ANATOMY OF
SPLEEN
120mm
SPLEEN STRUCTURE
The white pulp is circular in
structure and is made up mainly
of lymphocytes. It functions in a
way similar to the nodules of the
lymph node.
The red pulp surrounds the white
pulp and contains mainly red blood
cells and macrophages. The main
function of the red pulp is to
phagocytize old red blood cells.
FUNCTION
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During fetal development the spleen produces red and
white blood cells
Reticulocytes loose their nuclear remnants and excess
membrane before entering the circulation
RBCs or PLTs coated with IgG and IgM are removed
and destroyed
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The spleen is the site of destruction in autoimmune disease
states (ITTP and hemolytic anemia)
Parasites such as malaria can be removed as well
The spleen is involved in specific and nonspecific
immune responses (promotes phagocytosis and
destruction of bacteria)
SPLENOMEGALY
1.
Palpable = significant, clear, usually clinically
important. Usually after 1,5-2x increase in
size.
2.
Not palpable (Ultrasounf or CT diagnostics
only) = not significant, usually clinically not
important
CAUSES OF SPLENOMEGALY
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Infection
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Bacterial: Typhoid fever, endocarditis, septicemia, abscess
Viral:E-B virus, CMV, and others
Protozoal: Malaria, toxoplasmosis
Hematology benign disorders
Hemolytic anemia: Congenital: her. Spherocytoisis etc., acquired : AIHA
 Extramedullary hematopoiesis: thalassemia, osteopetrosis, (myelofibrosis)
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Neoplasms
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Metabolic diseases
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Malignant haematology dioseases + other metastatic tumors
Benign: Hemagioma, hamartoma
Lipidosis: Niemann-Pick, Gaucher disease
Mucopolysaccharidosis infiltration: Histiocytosis
Congestion (eg right heart failure, thrombosis of v. lienalis)
Cirrhosis
Cysts + abscesses
Miscellaneous
CAUSES OF SPLENOMEGALY IN MALIGNANT
HAEMATOLOGY
1.
2.
3.
4.
5.
Myeloprolipherative diseases (MPD):
- Primary myelofibroisis – PMF
- Chronic myelogenous leukemia – CML
- Polycythaemia vera – PV
- Essential thrombocythaemia – ET
Leukemia: Acute lymphoblastic leukemia, chronic lymphocytic leukemia
(CLL), hairy cell leukemia (HCL), myelodysplastic syndromes (MDS)
Lymphoma: Hodgfkin lymphoma, non-Hodgkin lymphoma
Histiocytic diaseases
Systemic mastocytosis
HYPERSPLENISM
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Refers to a variety of ill effects resulting from
increased splenic function that may be improved by
splenectomy
The criteria for diagnosis included:
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Anemia, leukopenia, thrombocytopenia or a combination of
the three
Compensatory bone marrow hyperplasia
Splenomegaly
Hypersplenism can be categorized as primary or
secondary
FELTY’S SYNDROME
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Is a syndrome consisting of severe rheumatoid
arthritis, granulocytopenia and splenomegaly
It usually occurs in patients with a long history of
rheumatoid arthritis
Severe, persistent and recurrent infections are
characteristic
Moderate splenomegaly is common
Splenectomy is effective in most patients
INFECTIOUS MONONUCLEOSIS
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A disease characterized by fever, sore throat,
lymphadenopathy and atypical lymphocytes
Most patients are young
Clinical symptoms are similar to those of a severe
upper respiratory tract infection
The spleen is enlarged and palpable in over 50% of
patients
Splenic rupture may occur
DIAGNOSIS OF SPLENOMEGALY
Palpable vs non palpable
 US and/or CT scan (PET)
 Crucial point: cause of splenomegaly
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DIAGNOSIS OF SPLENOMEGALY
Homogenous
Palpable vs non palpable
 US and/or CT scan (PET)
Focally non homogenous
 Crucial point: cause of splenomegaly
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DIAGNOSIS OF SPLENOMEGALY
Homogenous
Palpable vs non palpable
 US and/or CT scan (PET)
Focally non homogenous
 Crucial point: cause of splenomegaly
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Not obvious
Further investigation:
Residual after sepsis?
Primary splenic lymphoma?
Abscesses?
……
Gucher disease?
Obvious: MPDs, NHL,
HL, HCL, liver cirhosis,
AIHA, h. spherocytosis,
IM, malaria, etc.
GAUCHER’S DISEASE
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Is a disorder of lipid metabolism that may result in
massive splenomegaly and hypersplenism
Commonly found in the Jewish population
Diagnosis is made by finding the typical Gaucher’s
cells in biopsy tissue
Massive splenomegaly is usually the most common
form of presentation
The adult form is the most common form
Splenectomy (subtotal) shows great benefits
SPLENECTOMY
= Removal of the spleen
 Incidental versus Elective
 Main indications:
 Trauma
Incidental
 Diagnostic splenectomy (ML – MZL, some PMF )
 Therapeutic splenectomy: her. Spherocytosis,
immune thrombocytopenia (ITP), etc.
SPLENECTOMY
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Prior to removing the spleen specific
preoperative preparation is necessary
 All
patients should receive polyvalent
pneumococcal vaccine, polyvalent meningococcal
vaccine and Haemophilus influenzae type b
conjugant vaccine
 Blood and blood products should be available well
in advance of surgery
BLOOD COMPOSITIONAL CHANGES IN
THE ASPLENIC OR HYPOSPLENIC
PATIENT
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The absence of functional splenic tissue results in
characteristic changes in the circulating blood
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Some of these are predictable and desirable results
These changes are considered a measure of its success
when splenectomy is performed for a hematologic disease
Howell-Jolly bodies (nuclear remnants) and
thrombocytosis (desired result)
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Other findings include: target cells, acanthocytes (spur cells),
Heinz bodies (denatured hemoglobin) and stippled red cells
HOWELL JOLLY BODIES
Howell-Jolly
bodies are
round, purple
staining nuclear
fragments of
DNA in the red
blood cell
COMPLICATIONS OF SPLENECTOMY
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Surgery related: relatively high morbidity and
mortality associated in MPD (PMF). In other types of
diseases low complication rate.
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Long life OPSI risk
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Earleir onset of atherosclerosis.
POSTSPLENECTOMY SEPSIS (OPSI)
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Asplenic patients have an increased susceptibility to
the development of overwhelming infection caused by
encapsulated microorganisms.
The risk of sepsis is approximately 60 times greater
than normal after splenectomy
The risk is greatest in children younger than four years
of age
The risk of sepsis is higher among patients requiring
splenectomy for inherited diseases
The risk of sepsis after splenectomy is lowest after
trauma
POSTSPLENECTOMY SEPSIS
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The most common bacteria: Streptococcus
pneumoniae, Neisseria meningitidis or Haemophilus
influenzae
Because half of the patients develop sepsis from strep
pneumoniae, penicillin can be administered
immediately with onset of a febrile URI
Patients are instructed to obtain and wear a Medic
alert tag
POSTSPLENECTOMY SEPSIS
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The most common bacteria: Streptococcus
pneumoniae, Neisseria meningitidis or Haemophilus
influenzae
Because half of the patients develop sepsis from strep
pneumoniae, penicillin can be administered
immediately with onset of a febrile URI
Patients are instructed to obtain and wear a Medic
alert tag
All patients should be vaccinated
Thank You very much