HOW I TREAT IDIOPATHICHOW I TREAT IDIOPATHICHOW I TREAT IDIOPATHIC HOW I TREAT IDIOPATHIC THROMBOCYTOPENIC PURPURATHROMBOCYTOPENIC PURPURA

Dauglas B. Cines, James B Bussel. Dauglas B. Cines, James B Bussel. Blood Oktober 2005:106(7):2244Blood Oktober 2005:106(7):2244--22512251

INTRODUCTION

TO SET FORTH APROACH TO MANAGING ADULTS TO SET FORTH APROACH TO MANAGING ADULTS PRIMARY (AUTOIMMUNE) ITP FROM ASH & THE BRITISH PRIMARY (AUTOIMMUNE) ITP FROM ASH & THE BRITISH ( )( )COMMITTEE FOR STANDARDS IN HAEMATOLOGY COMMITTEE FOR STANDARDS IN HAEMATOLOGY GENERAL HAEMATOLOG Y TASK FORCEGENERAL HAEMATOLOG Y TASK FORCE :

1.WHO DEVELOPS ITP ?1.WHO DEVELOPS ITP ?2.HOW DIAGNOSIS ITP ?2.HOW DIAGNOSIS ITP ?3 WHO WE TREAT ITP3 WHO WE TREAT ITP3.WHO WE TREAT ITP3.WHO WE TREAT ITP4.ITP AND PREGNANCY4.ITP AND PREGNANCY

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WHO DEVELOPS ITP ?

TYPICAL ITP : ADULT WOMANTYPICAL ITP : ADULT WOMANAGE : BETWEEN 18 AND 40 YEARS AGE : BETWEEN 18 AND 40 YEARS

TWO PUBLICATION HAVE QUESTIONED THISTWO PUBLICATION HAVE QUESTIONED THISTWO PUBLICATION HAVE QUESTIONED THIS TWO PUBLICATION HAVE QUESTIONED THIS PERCEPTION : PERCEPTION :

DENMARK (SURVEY) DENMARK (SURVEY) THE FEMALETHE FEMALE MALE RATIO =1 7 : 1MALE RATIO =1 7 : 1--THE FEMALETHE FEMALE--MALE RATIO =1. 7 : 1MALE RATIO =1. 7 : 1

--MEDIAN AT DIAGNOSIS 56 YEARSMEDIAN AT DIAGNOSIS 56 YEARS

ENGLAND (PROSPECTIVE COHORT)ENGLAND (PROSPECTIVE COHORT)(PLATELET (PLT) < 50.000X10(PLATELET (PLT) < 50.000X1099/L/L

--THE FEMALETHE FEMALE--MALE RATIO =1.2 : 1MALE RATIO =1.2 : 1--HIGHEST >60 YEARSHIGHEST >60 YEARS

Frederiksen H, Schmidt. The Incidence of Idiophatic Thrombocytopenic Purpura in Adult Increase with age. ASH 1999: 909-913 (1)

Cines D.B. Blanchette V.S. Immune Thrombocytopenic. N Engl J Med. March,2002:995-1008George 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

HOW WE DIAGNOSE ITPHOW WE DIAGNOSE ITP

-- DIAGNOSIS OF EXCLUSION DIAGNOSIS OF EXCLUSION -- ESSENSIAL ELEMEN : ISOLATED THROMBOCYTOPENIA,ESSENSIAL ELEMEN : ISOLATED THROMBOCYTOPENIA,ESSENSIAL ELEMEN : ISOLATED THROMBOCYTOPENIA, ESSENSIAL ELEMEN : ISOLATED THROMBOCYTOPENIA,

PERIFERAL SMEAR (UNREMARKABLE), PERIFERAL SMEAR (UNREMARKABLE), -- PHYSICAL EXAMINATION (BLEEDING CONSISTENT PHYSICAL EXAMINATION (BLEEDING CONSISTENT

WITH PLT COUNT)WITH PLT COUNT)WITH PLT COUNT)WITH PLT COUNT)

Stasi R, Provan D. Management of Immune Thrombocytopenic Purpura in AdultMayo Clin Proc. 2004;79:504-522

-- BMP (RUTINE)BMP (RUTINE)TYPICAL PATIENT 60 YEARSTYPICAL PATIENT 60 YEARS> TYPICAL PATIENT >60 YEARS> TYPICAL PATIENT >60 YEARS

> DON`T SHOW A ROBUST RESPONSE (PLT>50.000) > DON`T SHOW A ROBUST RESPONSE (PLT>50.000) > PRIOR SPLENECTOMY> PRIOR SPLENECTOMY> EVALUATION OF RESPONSE IVIG, anti> EVALUATION OF RESPONSE IVIG, anti--D D > POOR RESPONSE TREATMENT> POOR RESPONSE TREATMENT

WHO WE TREATWHO WE TREAT

PLT <20.000/MM3 WITH BLEEDING MANIFESTATION OR NOTWITH BLEEDING MANIFESTATION OR NOT

10-YEAR STUDY OF 310 PT (PLT<30.000)1 HEMORRHAGIC DEATH1 HEMORRHAGIC DEATH

META-ANALYSIS OF 17 STUDIES, THE AGE ADJUSTED ,RISK OF FATAL HEMORHAGE WITH PLT <30.000

<40 Y 0 4%<40 Y 0,4%, 40-60 Y 1,2%, >60 Y 13%5Y MORTALITY 2 2 TO 47 8%5Y MORTALITY 2,2 TO 47,8%

TREATMENT AT PRESENTATIONTREATMENT 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 MENORRHAGIAOR MENORRHAGIA

PLT COUNTS : 10.000-20.000, SPONTANEOUS BLEEDING30.000 TO 50.000, MAY NOT EASY BRUISING>50.000, DISCOVERED INCIDENTALLY50 000, SCO C

PLT COUNT : ≥30.000 INITIAL GOAL OF TREATMENTINITIAL GOAL OF TREATMENT

PLT 20 000 TO 50 000PLT 20.000 TO 50.000 IMMIDIATE TH/ IS NOT REQUIRED.IN ABSENCE OF BLEEDING OR PREDISPOSING COMORBID UNCONTROLED HT, ACTIVE PEPTIC ULCER DISEASE , ANTICOAGULATION, RECENT SURGERY OR HEAD TRAUMA.SURGERY OR HEAD TRAUMA.

PLT 40.000 TO 50.000, RECOMMENDED REQUIRING ASPRIN NSAIDRECOMMENDED, REQUIRING ASPRIN, NSAID, WARFARIN, OR ATHER ANTITHROMBOTICS.

Cines D.B. Blanchette V.S. Immune Thrombocytopenic. N Engl J Med. March,2002:995-1008

Cines D.B. Blanchette V.S. Immune Thrombocytopenic. N Engl J Med. March,2002:995-1008

HOSPITALIZATION AND EMERGENCY THERAPYHOSPITALIZATION AND EMERGENCY THERAPY

HOSPITALIZED :HOSPITALIZED :1.PROFOUND MUCUCUTANEOUS OR INTERNAL BLEEDING 2.PLT ≤20.000

BLEEDING & HISTORY OF SIGNIFICANT COMPLIANCERESPON TH/ HAS NOT BEEN ESTABLISHED

REDUCE RISK OF BLEEDING (GENERAL):( )-CESSATION OF DRUG THAT IMPAIR PLT FUNCTION-CONTROL BP-MINIMIZE TRAUMA-MINIMIZE TRAUMA

-REDUCE MUCOSAL BLEEDING E AMINOCAPROIC ACID ( 100 /KB LOADING DOSEE-AMINOCAPROIC ACID ( 100mg/KB LOADING DOSE UP TO MAX OF 5 gr IV OVER 30-60 MNT FOLLOW UP BY 5 gr EVERY 6 H IV OR ORALLY (MAX DOSE=24 gr/DAY)g ( g / )

TRANEXAMIC ACID

DESMOPRESSIN ACETAT (DDAVP; 0,3 ug/KB)

INITIAL THERAPY FOR NONEMERGENT INDICATIONSINITIAL THERAPY FOR NONEMERGENT INDICATIONS

THERE IS NO CONSENSUS OPTIMAL DURATION OF CORTICOSTEROIDCONTINUE FULL DOSE FOR 3 to 4 WEEKSTAPERRING PREDNISON SLOWLY, ONCE DOSES OF10 MG/DAY ARE REACHED10 MG/DAY ARE REACHED

RESPON RATE 50-90% STABEL REMISI 10 30%STABEL REMISI 10-30%

PERSISTEN ITPPERSISTEN ITP

THROMBOCYTOPENIA RECURS WHEN CORTICOSTEROIDTHROMBOCYTOPENIA RECURS WHEN CORTICOSTEROID ARE TAPEREDTARGET PLT > 20.000 to 30.000

MAYOR DECISION : SPLENECTOMY