Abnormal Fibrinogen in Thrombotic Thrombocytopenic Purpura

Author:

RIVERO ISAAC12,RITZ NORTON D.13

Affiliation:

1. Hematology Division, Department of Medicine, Maimonides Hospital, Brooklyn, New York, and the State University of New York, Downstate Medical Center, Brooklyn, New York.

2. Maimonides Hospital, Brooklyn, New York; Chief, Section of Hematology and Immunology, Internal Medicine II, Universidad Nacional de Cuyo Mendoza, Argentina.

3. Hematology Clinic, Maimonides Medical Center, Brooklyn, New York; Clinical Assistant Professor of Medicine, State University of New York, Downstate Medical Center, Brooklyn, New York.

Abstract

Abstract An abnormal fibrinogen was demonstrated in the plasma of an eleven year old boy with thrombotic thrombocytopenic purpura during the acute disease and during one clinical relapse nine months later. The altered fibrinogen was antigenically similar to normal fibrinogen but migrated more rapidly toward the cathode. The abnormal fibrinogen was incompletely precipitated by ammonium sulfate or heat, but was clottable by thrombin. Thrombotic thrombocytopenic purpura may be associated with incomplete in vivo defibrination and a circulating partially degraded fibrinogen. A patient with this syndrome is alive and well forty months after treatment with 200 mg. of prednisone daily.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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