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Clinical Significance of Inhibitors in Acquired von Willebrand
Syndrome
Hiroshi Mohri,
Shigeki Motomura,
Heiwa Kanamori,
Michio Matsuzaki,
Shin-ichiro Watanabe,
Atsuo Maruta,
Fumio Kodama, and
Takao Okubo
From the First Department of Internal Medicine, Division of Blood
Transfusion and Laboratory Medicine, Yokohama City University,
Yokohama, Japan and the Department of Hematology/Chemotherapy, Kanagawa
Cancer Center, Yokohama, Japan.
Of 260 patients enrolled, 25 patients (9.6%) were associated with
acquired von Willebrand syndrome (AvWS). We studied 25 patients with
AvWS, retrospectively. AvWS was diagnosed by reduced levels of von
Willebrand factor (vWF) (decrease of von Willebrand factor antigen
[vWF:Ag] and von Willebrand ristocetin cofactor
[vWF:RCoF]), a decrease of ristocetin-induced platelet
agglutination (RIPA), sometimes decreased high-molecular-weight
multimers, and prolonged bleeding time with neither prior nor family
histories of bleeding problems and the evidence of normal vWF:RCoF in
their families. The inhibitor of vWF was determined by mixing patient
plasma with pooled normal plasma. Eight patients in this study had the
inhibitors to vWF that were of the IgG class; the subclasses were
IgG1 (7 cases) and IgG2 (1 case). Multimeric
analysis of vWF showed selective loss of large multimers in most
patients with AvWS similar to that of congenital type-2 von Willebrand
disease (vWD). All inhibitors blocked ristocetin-mediated vWF binding
to platelets. Five out of 6 IgGs evaluated here recognized the 39/34-kD
fragment (residues 480/481-718) and Fragment III (residues 1-1365) that
implied binding domain of glycoprotein Ib (GPIb), whereas 1 recognized
Fragment I (residues 911-1365). A close relationship was found
between the presence of the inhibitor and bleeding tendency. Of the 7 patients with inhibitors, 6 patients (86%) had a bleeding tendency, as
well as 1 of the 15 patients without inhibitors (6%). The efficacy of
treatment of underlying diseases and/or therapy with deamino D-arginine vasopressin (DDAVP) for the treatment of AvWS
also depends on the presence of an inhibitor. Four of 8 patients with inhibitors (50%) had poor response to treatment of the underlying disease and/or therapy with DDAVP, as well as 1 of the 16 patients without inhibitors (6%). These results indicate that
patients with AvWS developing inhibitors to vWF are likely to have
bleeding problems and might be resistant to treatment of underlying
diseases and/or therapy with DDAVP for bleeding to AvWS. We
also showed evidence that intravenous immunoglobulin therapy (0.3 g/kg,
3 days) was effective to correct a hemostatic defect and manage severe
bleeding in a patient with AvWS developing inhibitors. We might
consider an additional treatment including expensive high-dose
immunoglobulin therapy when uncontrollable bleeding is continued after
the treatment of the underlying diseases and/or therapy with
DDAVP.
Blood, Vol. 91 No. 10 (May 15), 1998:
pp. 3623-3629
© 1998 by The American Society of Hematology.
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