Download ITP

IDOPATHIC
THROMBOCYTOPENIA
PURPURA
April Alexander
Diagnosis of ITP
 ITP
is an autoimmune bleeding disorder
which is characterized by abnormally low
level of platelets
 1735
– Paul Gottlieb Werlhof
(Dameron & Puzanov, 2012) (Cines & Leibman, 2009)
(http://bmchp.kramesonline.com/235014.img)
Diagnosis of ITP

American society of hematology

History: Isolated bleeding symptoms in conjugation with
thrombocytopenia without constitutional symptoms

PE: Signs of bleeding in the absence of hepatosplenomegaly,
lymphadenopathy, or other congenital conditions.

Diagnostic tests:


CBC

Platelet count below 100,000 mcL

Normal RBC + WBC
Peripheral blood smear:

Platelets should be normal to large in size

Normal RBC + WBC
(American Society of Hematology, 2011)
Pathogenesis

Antiplatelet antibodies begin to bind to the plasma membrane on
platelets; as a result, platelet destruction by mononuclear phagocytes
in the spleen occurs. The rate of platelet destruction happens so
rapidly that it exceeds the bone marrows ability to produce more.

Secondary ITP has been linked to a viral illness especially in children

Autoimmune diseases

Genetics
(Johnsen, 2012)(http://itpanswers.com)
Clinical Manifestations


History:

Spontaneous bruising

Hemorrhagic bullae of the mucous membrane/gingival bleeding

Prolonged bleeding from minor injury

Epistaxis

Heavier than normal menstrual bleeding

Dark urine + stool
Important questions to ask:

Recent illness, recent hospitalization or surgery, medication list, travel
history, blood disorders, leukemia, or autoimmune disorders
Review of systems

General: fevers? Weight loss? Night sweats?

Skin: spontaneous bruising? swollen lymph nodes?

HEENT: Change in vision or hearing?

GI: Rectal bleeding? Dark stool?

Urinary: Dark urine?

Genital: Females- amount of menstrual bleeding?

Musculoskeletal: Bone pain?

Neurological: Changes in orientation, attention, or memory?
Headaches? Dizziness?
Clinical Manifestations


Physical Exam:

Patients are going to feel well

petechiae, purpura, and ecchymosis on arms, legs, and trunk

Hemorrhagic bullae/gingival bleeding

Tympanic membrane hemorrhage

Retinal hemorrhage

Dried blood in nose from epistaxis

Vaginal and rectal exam may reveal bleeding
WNL

Afebrile, Lymph nodes, spleen, liver, no change in mentation
(http://healthfixit.com)
(http://e-ijd.org)
Diagnostic tests



CBC

Platelet count below 100,000 mcL

Normal RBC + WBC
Peripheral blood smear:

Platelets should be normal to large in size

Normal RBC + WBC
Bone Marrow Aspiration


Megakaryocytes in normal or increased numbers
Urinalysis

Microscopic hematuria

HIV + HCV

H. Pylori
(American Society of Hematology, 2011)
Differential Diagnosis

Von Willebrand Disease

Henoch Scholien Purpura

Acute Leukemia

Aplastic Anemia
Treatment

Observation (monitored by Hematologist)

First line therapy for patients with platelet count <30,000 mcL

Corticosteroids


Prednisone 0.5-2 mg/kg/day x 4 weeks

IVIg

Anti-D
Second line

Adults: splenectomy, Rituximab, Thrombopoetin Receptor Agonist

Children: splenectomy, Rituximab, high dose Dexamethasone
(American Society of Hematology, 2011)
High-does Dexamethasone vs Prednisone for treatment of adult ITP
(http://drugster.info/drug/medicament/7041/)
Patient Education
Avoiding injury, NSAIDS, + Aspirin
 Children are going to need to avoid contact sports
until disease resolution

(Frey & Oberleitner, 2011)
Outcomes

80% of patients with acute ITP will make a full
recovery

Most patients diagnosed with chronic ITP will
achieve long term remission
References

American Society of Hematology. (2011). Retrieved from www.hematology.org

Cines, D.B., & Leibman, H.A. (2009). The Immune thrombocytopenia syndrome: A Disorder of diverse pathogenesis and clinical presentation. HematologyOncology Clinics of North America, 23(6), 1155-1161. doi: 10.1016/j.hoc.2009.09.003

Dameron, A., & Puzanov, I. (2012). Idiopathic thrombocytopenia purpura. In. H.B. Hawley (Ed.), Infectious disease and conditions (Vol. 2, pp. 556-557).
Salem, MA: EBSCO Publishing Inc. Retrieved from
http://go.galegroup.com/ps/i.do?id=GALE%7CCX4003300289&v=2.1&u=psucic&it=r&p=GVRL&sw=w&asid=dbf4951622073b8d9965a06b292642a5

Dexamethasone tablet. [online image]. Retrieved April 26, 2016 from http://drugster.info/drug/medicament/7041/

Frey, R.J., & Oberleitner, M.G. (2011). Idiopathic Thrombocytopenia Purpura. In. L.J. Fundukian (Ed.), Gale encyclopedia of medicine, (Vol 3(4) pp 22792282). Detroit, MI: Cengage learning. Retrieved from
http://go.galegroup.com/ps/i.do?id=GALE%7CCX1919600908&v=2.1&u=psucic&it=r&p=GVRL&sw=w&asid=b216c2764701bd1ec6e50a1c7ecb9ba1

Hemorrhagic bullae. [online image] Retrieved on April 25,2016 from http://e-ijd.org

Idiopathic thrombocytopenia purpura. [online image] Retrieved on April 25, 2016 from http://itpanswers.com

Johnsen, J. (2012). Pathogenesis in immune thrombocytopenia: New insights. American Society of Hematology education book, 1, 306-312. Retrieved from
http://asheducationbook.hematologylibrary.org/content/2012/1/306.full

Petechiae. [online image]. Retrieved on April 25, 2016 from http://healthfixit.com

Prednisone tablet. [online image]. Retrieved April 26, 2016 from http://drugster.info/drug/medicament/7041/

Thrombocytopenia. [online image]. Retrieved on April 26, 2016 from http://bmchp.kramesonline.com/235014.img

Yu, W., Xue-bin, J., Ya-wen, W., Jing-xia, W., En-qin, Y., Zheng-cheng, W., Yu-qi, S., Zuo-mu, B., Cui-ai, R., Fang, Z., Guo-qiang, L., Jun, P., & Ming, H.
(2016). High-dose Dexamethasone vs Prednisone for treatment of adult immune thrombocytopenia: A Prospective multicenter randomized trial. Blood,
127(3), 296-302. doi: 10.1182/blood-2015-07-659656