Hypofibrinogenemia and thrombocytopenia in familial hemophagocytic reticulosis

Familial hemophagocytic reticulosis (FHR) is a lethal disorder of early infancy characterized by infiltration of many tissues by histiocytes. A severe bleeding disorder frequently accompanies the disease. Thrombocytopenia associated with an absence of fibrinogen was demonstrated in six patients. The coagulation abnormality was not caused by disseminated intravascular clotting, because plasma levels of all other clotting factors were normal and there was no proof of increased fibrinolysis. If present, this unusual coagulation disturbance is helpful in establishing the diagnosis of FHR during life.