HOW I TREAT IDIOPATHIC

THROMBOCYTOPENIC
PURPURA

Dauglas B. Cines, James B Bussel.

Blood Oktober 2005:106(7):2244-2251
 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) < 50.000X109/L
-THE FEMALE-MALE RATIO =1.2 : 1
-HIGHEST >60 YEARS
 PATHOGENESIS

1. PLATELET AUTO ANTIBODY

2. CELL-MEDIATED IMMUNE REGULATION
3. GENETIC
4. IMPAIRMENT OF MEGAKARYOCYTE
MATURATION AND PLATELET PRODUCTION
5. PLATELET KINETIC STUDY

Yang R, Han Z.C. Pathogenesis and management of chronic Idiopathic

Thrombocytopenic
Cines D.B. Blanchette V.S. Immune Thrombocytopenic. N Engl J Med. March
 HOW WE DIAGNOSE ITP

- DIAGNOSIS OF EXCLUSION
- ESSENSIAL ELEMEN : ISOLATED
THROMBOCYTOPENIA,
PERIFERAL SMEAR , PHYSICAL
EXAMINATION (BLEEDING CONSISTENT WITH
PLT
COUNT)

- EXCLUDED OF OTHER CAUSES :

- EXPOSURE TO DRUG,
- HERB, FOODS,
- OTHER SUBSTANCES (EG, QUININI) ,
- ASSOCIATED WITH THROMBOCYTOPENIA;
PSEUDOTHROMBOCYTONIA; GIANT PLATELET,
FAMILY HISTORY CONSISTENT WITH INHERITED
THROMBOCYTOPENIA,
SYMPTOMS/ SIGNS SUGGEST OF AN UNDERLYING
THAT MAY CAUSE SECONDARY IMMUNE
THROMBOCYTOPENIA

- HIV OR HEPATITIS (RISK POPULATION)

- HAEMOPHILUS PYLORI (ITALY & JAPAN)

- COEXISTING IMMUNE HEMOLYTIC ANEMIA (1%),

- IMMUNOGLOBULIN LEVEL (RECURRENT INFECTION
OR
ALERGY) IF THERE IS REACTION TO IV IG OR
PRIOR
TRANFUSION

- CLONALITY & CYTOGENETICS

- ANTIPHOSPHOLIPID ANTIBODIES (aps)
(HISTORY THROMBOSIS, GASTASIONAL FAILURE,
PROLONG APTT)

- SLE (5-10%)
(SECONDARY IMMUNE THROMBOCYTOPENIA)
- ANTIPLATELET ANTIBODIES DON NOT RELY MAKE
OR
EXCLUDE A DIAGNOSIS OF ITP
DETECTED IN 10-20% OF PTS CHRONIC LIVER
DISEASE, MDS
(NONIMMUNE THROMBOCYTOPENIAS)
 BMP

> TYPICAL PATIENT >60 YEARS

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

PLT <20.000/MM3
WITH BLEEDING MANIFESTATION OR
NOT

10-YEAR STUDY OF 310 PTS

(PLT<30.000) : 1 HEMORRHAGIC DEATH
 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 :
10.000-20.000, SPONTANEOUS BLEEDING,
30.000 TO 50.000, MAY NOT EASY BRUISING
>50.000, DISCOVERED INCIDENTALLY
Cines D.B. Blanchette V.S. Immune Thrombocytopenic. N Engl J Med. March
Cines D.B. Blanchette V.S. Immune Thrombocytopenic. N Engl J Med. March
 TREATMENT OF CHRONIC ITP

PRINCIPLES OF TREATMENT

30-40% PLT <50.000 AFTER SPLENECTOMY

NOT RESPON OR RELAPSE

THE CONCUR WITH SEVERAL OF RECOMM OF BHTF

MINOR SURGERY PLT >50.000
MAYOR SURGERY PLT>80.000
HIGHER COUNTS ARE ADVISABLE FOR CARDIAC
BYPASS SURGERY, NEUROSURGERY, AND COMPLEX
ORTHOPEDIC OR PLASTIC SURGERY.
PRINCIPLES OF TREATMENT

ALKOLATING AGENT SHOULD BE AVOID or MINIMIZE

total dose CYCLOPHOSPAMIDE <2000mg/m2
THEY PRACTIC: LOOK FOR AN ASCESSORY SPLENEN
WITH MRI or ANATHER SENSITIVE SCAN (HEAT-
DAMAGE RED CELL) IF THE RESPONSE TO
SPLENECTOMY WAS DURABLE.
TREATMENT OF CHRONIC REFRACTORY ITP

5% CHRONIC REFRACTER ITP

DEFINE AS FAILURE OF ANY MODALITY TO KEEP
PLT >20.000

THEY GENERALLY RESERVE ; THE TREATMENTS

RFRACTORY :
SPLENECTOMY, DANAZOLE, RITUXIMAB,or
IMMUNOSUPRESSANTS

ONE OPTION :
CsA 1,25 2,5 mg/kg/ DOSE PO EVERY 12 HOURS
EXPERIMENTAL THERAPY

THROMBOPOETIC FACTOR
STUDY : 2 OF 4 PTS DEVELOPED TEMPORARY
THROMBOCYTOSIS
2 PLACEBO-CONTROL TRIAL AMG-531 (DOSE >3,0
ug/Kg)
-8 OF 12 PTS AND -7 OF 8 PTS
SHOWED TEMPORARY BUT SUBSTANTIAL
INCREASES PLT
THERAPY :
IVFG
IV anti-D (safe and efective) (limited experience)
SPLENECTOMY.
Danazol, Cyclophosphamide, anti-CD20, vinca
alkaloid avoid
(AZHATHIOPRIN possible exception for Renal
transplant)