Desmopressin in haemophilia: The need for a standardised clinical response and individualised test regimen

Abstract

We suggest standardised desmopressin response based on clinically relevant FVIII:C levels, e.g. 0.30 and 0.50 IU/mL. In addition, patients with <0.30 IU/mL FVIII:C after 1 hour (non-responder) or ≥0.80 IU/mL (sustained responder) do not require subsequent blood sampling. However, patients with ≥0.30-0.79 IU/mL FVIII:C after 1 hour should undergo blood sampling after 6 hours to additionally determine response sustainability.

Desmopressin in non‐severe haemophilia A: Test‐response and clinical outcomes in a single Canadian centre review

“…Stoof et al suggested incorporating FVIII level at 6‐hour post‐DDAVP administration into the test responsiveness definition. The 1‐hour hr peak FVIII level was used to define non‐responders (<30 IU/dL) and “sustained responders” (≥80 IU/dL).…”

“…The 1‐hour hr peak FVIII level was used to define non‐responders (<30 IU/dL) and “sustained responders” (≥80 IU/dL). Those with 1‐hour FVIII level 30 to <80 IU/dL were further classified based on the 6‐hour post‐DDAVP FVIII level as “sustained responders” if levels remained ≥30 IU/dL, and “short‐term responder” if levels were <30 IU/dL . The 6‐hour post‐DDAVP FVIII level is a time point selected by Stoof et al for the definition of “sustained responders” based on their testing protocol.…”

“…after administration of a test dose. What constitutes a response varies among investigators, and at least 10 different definitions of test‐response have been reported (Summarized in Table ) …”

“…Variations to define test‐response arise from the use of different cut‐off values for plasma levels of FVIII post‐DDAVP administration, as well as the inconsistent use of FVIII‐fold increase at a certain time from pre‐administration levels. While clustering of similar definitions into one set of guidelines has decreased some of the variability, patients may be classified differently depending on which grouping is used.…”

“… Not included in the table is the definition of Stoof et al incorporating the FVIII level at 6‐hour post‐DDAVP in the formula (see text). …”