0521779407-10 CUNY1086/Karliner 0 521 77940 7 June 7, 2007 18:40
Idiopathic (Immune) Thrombocytopenic Purpura (ITP) 809differential diagnosis
■Leukemia, tumor infiltration of bone marrow, aplastic anemia, but
ITP is the most likely diagnosis if platelets <20,000/mm^3 and all other
cell lines are normal.
■Drug-induced thrombocytopenia
■HIV
■Evan’s syndrome with positive Coombs
■TTP, HUS: microangiopathic changes on peripheral smear
■Congenital quantitative platelet disorder. TAR (thrombocytopenia
with absent radii) syndrome or, more likely if moderate throm-
bocytopenia, MHY9-associated thrombocytopenia (May-Hegglin
anomaly)
■Wiskott-Aldrich syndrome: male infant with isolated thrombocy-
topenia, microplatelets, eczema, recurrent infections
management
■No aspirin
■Avoid intramuscular injections.
■Head injury precautions, no contact sports, parental education on
controlling nosebleeds
■Hospitalization if mucocutaneous bleeding, life-threatening bleed-
ing, parents unable to restrict child’s activity
■Consider no therapy in platelet count >10,000/mm^3 and only minor
bruising with emphasis on education about head trauma.
■Treatment for patients with extensive mucocutaneous bleeding, life-
threatening bleeding (treatment does NOT decrease the duration of
the disease)
➣IVIG: side effects include severe headache, aseptic meningitis,
anaphylaxis (especially in IgA deficiency)
➣Corticosteroids: orally (for 1–2 weeks); some experts recommend
bone marrow examination prior to steroid use, especially in the
setting of poor response to initial therapy
➣IV anti-Rh(D) immunoglobulin if patient’s blood type is Rh(D) –
positive. Rarely, renal failure secondary to severe intravascular
hemolysis and hemoglobinuria can occur. Also, recent reports in
adults have associated life-threatening DIC after treatment with
IV anti-Rh(D) for ITP.
➣Splenectomy for patients with life-threatening bleeding or chro-
nic ITP. Pneumovax and penicillin coverage post-operatively.
➣Vinca alkaloids: vincristine, vinblastine IV weekly for 4 weeks.
Side effects include peripheral neuropathy and abdominal and
jaw pain.