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

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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)

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. Hematology- Oncology 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 2279- 2282). 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