Transcript 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