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DDAVP challenge tests in boys with mild/moderate haemophilia A *
Author(s) -
RevelVilk Shoshana,
Blanchette Victor S.,
Sparling Cathy,
Stain Ann Marie,
Carcao Manuel D.
Publication year - 2002
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.2002.03507.x
Subject(s) - medicine , haemophilia , desmopressin , haemophilia a , gastroenterology , pediatrics
Summary. Desmopressin (DDAVP) increases plasma factor VIII coagulant activity (FVIII:C) levels in patients with mild/moderate haemophilia A. In some subjects, FVIII can be increased to haemostatic levels, thereby avoiding use of factor VIII concentrates. We reviewed our hospital's experience with 62 boys with FVIII:C levels 0·01–0·3 IU/ml who had a DDAVP challenge test (i.v. 0·3 µg/kg) following diagnosis. A therapeutic response was defined as a 1 h post‐FVIII:C increase at least twofold over baseline and > 0·3 IU/ml. Of the total group, 29 (47%) boys responded to DDAVP, all of them with mild haemophilia (baseline FVIII:C ≥ 0·05 IU/ml), yielding a response rate of 57% in this subgroup. Boys who responded to DDAVP had higher baseline FVIII:C levels (mean ± SEM, 0·17 ± 0·01 vs 0·10 ± 0·01 IU/ml, P < 0·01) and were older (5·2 ± 0·8 vs 3 ± 0·4 years, P = 0·02) than those who failed to do so. The association between DDAVP response and age, however, remains unclear: seven boys who failed the initial challenge test responded to re‐challenge after a mean of 6·3 years (median 4·9, range 0·5–12·5), increasing the response rate in boys with mild haemophilia to 71%. Age and FVIII:C association with DDAVP response are both important in boys with mild/moderate haemophilia A. Absence of response to DDAVP should therefore be confirmed by later re‐challenge.