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IDOPATHIC THROMBOCYTOPENIA PURPURA

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1 IDOPATHIC THROMBOCYTOPENIA PURPURA
April Alexander

2 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) (

3 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)

4 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)(

5 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

6 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?

7 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

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10 Diagnostic tests CBC Platelet count below 100,000 mcL Normal RBC + WBC
Peripheral blood smear: Platelets should be normal to large in size Bone Marrow Aspiration Megakaryocytes in normal or increased numbers Urinalysis Microscopic hematuria HIV + HCV H. Pylori (American Society of Hematology, 2011)

11 Differential Diagnosis
Von Willebrand Disease Henoch Scholien Purpura Acute Leukemia Aplastic Anemia

12 Treatment Observation (monitored by Hematologist)
First line therapy for patients with platelet count <30,000 mcL Corticosteroids Prednisone 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)

13 High-does Dexamethasone vs Prednisone for treatment of adult ITP
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14 Patient Education Avoiding injury, NSAIDS, + Aspirin
Children are going to need to avoid contact sports until disease resolution (Frey & Oberleitner, 2011)

15 Outcomes 80% of patients with acute ITP will make a full recovery
Most patients diagnosed with chronic ITP will achieve long term remission

16 References American Society of Hematology. (2011). Retrieved from Cines, D.B., & Leibman, H.A. (2009). The Immune thrombocytopenia syndrome: A Disorder of diverse pathogenesis and clinical presentation. Hematology- Oncology Clinics of North America, 23(6), doi: /j.hoc Dameron, A., & Puzanov, I. (2012). Idiopathic thrombocytopenia purpura. In. H.B. Hawley (Ed.), Infectious disease and conditions (Vol. 2, pp ). Salem, MA: EBSCO Publishing Inc. Retrieved from Dexamethasone tablet. [online image]. Retrieved April 26, 2016 from Frey, R.J., & Oberleitner, M.G. (2011). Idiopathic Thrombocytopenia Purpura. In. L.J. Fundukian (Ed.), Gale encyclopedia of medicine, (Vol 3(4) pp ). Detroit, MI: Cengage learning. Retrieved from Hemorrhagic bullae. [online image] Retrieved on April 25,2016 from Idiopathic thrombocytopenia purpura. [online image] Retrieved on April 25, 2016 from Johnsen, J. (2012). Pathogenesis in immune thrombocytopenia: New insights. American Society of Hematology education book, 1, Retrieved from Petechiae. [online image]. Retrieved on April 25, 2016 from Prednisone tablet. [online image]. Retrieved April 26, 2016 from Thrombocytopenia. [online image]. Retrieved on April 26, 2016 from 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), doi: /blood

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