Acquired factor VII deficiency associated with aplastic anaemia: correction with bone marrow transplantation.
We report a patient with severe aplastic anaemia found to have a prolonged prothrombin time due to acquired factor VII deficiency. No evidence for a factor VII inhibitor or inactivator was demonstrable. Laboratory studies identified deficiency both of factor VII activity and factor VII antigen. The factor VII deficiency persisted from clinical presentation until approximately 50 d after allogeneic marrow transplantation when restoration of factor VII activity and antigen was noted. The patient's serum could be depleted of factor VII activity by in vitro incubation with Protein A bound to Sepharose, suggesting the presence of an IgG or IgG containing complex able to bind factor VII, but not neutralize its procoagulant activity. A dual specificity solid phase immunoassay identified a factor VII binding immunoglobulin which was detectable throughout the course of factor VII deficiency. The concordant appearance of this factor VII reactive immunoglobulin and the factor VII deficiency suggested the pathologic role of this immunoglobulin in the aetiology of the factor VII deficiency. This factor VII binding immunoglobulin may have induced rapid plasma clearance of the factor VII molecule or, alternatively, may have modified factor VII synthesis. The immunosuppressive therapy and subsequent lymphohaematopoietic engraftment following allogeneic marrow transplant was accompanied by complete resolution of the factor VII deficiency.
Weisdorf
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Department of Medicine, University of Minnesota, Minneapolis 55455.
Hasegawa
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Fair
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DS
D000293:Adolescent; D000741:Anemia, Aplastic / Q000097:blood / Q000150:complications*; D016026:Bone Marrow Transplantation; D005167:Factor VII / Q000032:analysis / Q000276:immunology; D005168:Factor VII Deficiency / Q000097:blood / Q000209:etiology / Q000628:therapy*; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis
D007136:Immunoglobulins; D005167:Factor VII