INTRODUCTION TO SET FORTH APROACH TO MANAGING ADULTS TO SET FORTH APROACH PRIMARY ( PRIMARY AUTOIMMUNE) AUTOIMMUNE ITP FROM ASH & THE BRITISH

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1 HOW I TREAT IDIOPATHIC THROMBOCYTOPENIC PURPURA Dauglas B. Cines, James B Bussel. Blood Oktober 2005:106(7):

2 INTRODUCTION TO SET FORTH APROACH TO MANAGING ADULTS PRIMARY (AUTOIMMUNE) ITP FROM ASH & THE BRITISH COMMITTEE FOR STANDARDS IN HAEMATOLOGY GENERAL HAEMATOLOG Y TASK FORCE : 1.WHO DEVELOPS ITP? 2.HOW DIAGNOSIS ITP? 3.WHO WE TREAT ITP 4.ITP AND PREGNANCY -

3 WHO DEVELOPS ITP? TYPICAL ITP : ADULT WOMAN AGE : BETWEEN 18 AND 40 YEARS TWO PUBLICATION HAVE QUESTIONED THIS PERCEPTION : DENMARK (SURVEY) -THE FEMALE-MALE RATIO =1. 7 : 1 -MEDIAN AT DIAGNOSIS 56 YEARS ENGLAND (PROSPECTIVE COHORT) (PLATELET (PLT) < X10 9 /L -THE FEMALE-MALE RATIO =1.2 : 1 -HIGHEST >60 YEARS Frederiksen H, Schmidt. The Incidence of Idiophatic Thrombocytopenic Purpura in Adult Increase with age. ASH 1999: (1)

4 Cines D.B. Blanchette V.S. Immune Thrombocytopenic. N Engl J Med. March,2002: George JN, el Harake MA, Aster RH. Thrombocytopenia due to enhanced platelet destruction By immunologic mechanism, in Beutler E, Litchmann MA, Coller BS, Kipps TJ. (eds): William Hematology Ne York NY McGra Hill 1995:p1315

5 HOW WE DIAGNOSE ITP - DIAGNOSIS OF EXCLUSION - ESSENSIAL ELEMEN : ISOLATED THROMBOCYTOPENIA, PERIFERAL SMEAR (UNREMARKABLE), - PHYSICAL EXAMINATION (BLEEDING CONSISTENT WITH PLT COUNT)

6 Stasi R, Provan D. Management of Immune Thrombocytopenic Purpura in Adult Mayo Clin Proc. 2004;79:

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10 - BMP (RUTINE) > TYPICAL PATIENT >60 YEARS > DON`T SHOW A ROBUST RESPONSE (PLT>50.000) > PRIOR SPLENECTOMY > EVALUATION OF RESPONSE IVIG, anti-d > POOR RESPONSE TREATMENT

11 WHO WE TREAT PLT <20.000/MM3 WITH BLEEDING MANIFESTATION OR NOT 10-YEAR STUDY OF 310 PT (PLT<30.000) 1 HEMORRHAGIC DEATH META-ANALYSIS OF 17 STUDIES, THE AGE ADJUSTED RISK OF FATAL HEMORHAGE WITH PLT < <40 Y 0,4%, Y 1,2%, >60 Y 13% 5Y MORTALITY 2,22 TO 47,8%

12 TREATMENT AT PRESENTATION PRINCIPLES OF MANAGEMENT PLT<20.000, WITH PETECHIAE OR PURPURA, THE ONSET MORE OFTEN INSIDIOUS THAN PREVIOUSLY PLT<10.000, SEVERE CUTANEOUS BLEEDING,PROLONGE EPISTAXIS, GINGGIVA BLEEDING, OVERT HEMATURIA, OR MENORRHAGIA PLT COUNTS : , SPONTANEOUS BLEEDING TO , MAY NOT EASY BRUISING >50.000, 000, DISCOVERED INCIDENTALLY

13 PLT COUNT : INITIAL GOAL OF TREATMENT PLT TO IMMIDIATE TH/ IS NOT REQUIRED. IN ABSENCE OF BLEEDING OR PREDISPOSING COMORBID UNCONTROLED HT, ACTIVE PEPTIC ULCER DISEASE, ANTICOAGULATION, RECENT SURGERY OR HEAD TRAUMA. PLT TO , RECOMMENDED, REQUIRING ASPRIN, NSAID, WARFARIN, OR ATHER ANTITHROMBOTICS.

14 Cines D.B. Blanchette V.S. Immune Thrombocytopenic. N Engl J Med. March,2002:

15 Cines D.B. Blanchette V.S. Immune Thrombocytopenic. N Engl J Med. March,2002:

16 HOSPITALIZATION AND EMERGENCY THERAPY HOSPITALIZED : HOSPITALIZED : 1.PROFOUND MUCUCUTANEOUS OR INTERNAL BLEEDING 2.PLT BLEEDING & HISTORY OF SIGNIFICANT COMPLIANCE RESPON TH/ HAS NOT BEEN ESTABLISHED

17 REDUCE RISK OF BLEEDING (GENERAL): -CESSATION OF DRUG THAT IMPAIR PLT FUNCTION -CONTROL BP -MINIMIZE TRAUMA -REDUCE MUCOSAL BLEEDING E-AMINOCAPROIC ACID ( 100mg/KB LOADING DOSE UP TO MAX OF 5 gr IV OVER MNT FOLLOW UP BY 5 gr EVERY 6 H IV OR ORALLY (MAX DOSE=24 gr/day) TRANEXAMIC ACID DESMOPRESSIN ACETAT (DDAVP; 0,3 ug/kb)

18 INITIAL THERAPY FOR NONEMERGENT INDICATIONS THERE IS NO CONSENSUS OPTIMAL DURATION OF CORTICOSTEROID CONTINUE FULL DOSE FOR 3 to 4 WEEKS TAPERRING PREDNISON SLOWLY, ONCE DOSES OF 10 MG/DAY ARE REACHED RESPON RATE 50-90% STABEL REMISI 10-30%

19 PERSISTEN ITP THROMBOCYTOPENIA RECURS WHEN CORTICOSTEROID ARE TAPERED TARGET PLT > to MAYOR DECISION : SPLENECTOMY

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