Chronic thrombocytopenia in an immunodeficient patient with hemophilia A.
Coincident hemophilia and idiopathic thrombocytopenia has been rarely observed. We report here on a young man with severe hemophilia A who was treated with concentrates of lyophilized antihemophilic factor for several years before he developed thrombocytopenia. An isolated thrombocytopenia coincident with reduced platelet survival, ample megakaryocytes in the bone marrow, elevated platelet-associated IgG, as well as remission after treatment with prednisone and splenectomy, suggest the idiopathic form of thrombocytopenia. In addition, defects in cellular immunity became obvious. A causal relationship between the application of blood-derived products and the development of the platelet disorder as well as the impairment of the T-cell system remain to be demonstrated.
D000328:Adult; D000614:Aminocaproates / Q000627:therapeutic use; D005169:Factor VIII / Q000627:therapeutic use; D006467:Hemophilia A / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D007116:Immunization, Passive; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D008297:Male; D011241:Prednisone / Q000627:therapeutic use; D013156:Splenectomy; D013921:Thrombocytopenia / Q000150:complications* / Q000628:therapy
D000614:Aminocaproates; D005169:Factor VIII; D011241:Prednisone