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Results for “AFIBRINOGENEMIA”

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Malignant hemangioendothelioma of the heart with hematological disorders.

An autposy case of a 30-year-old house wife with malignant hemangiothelioma of the heart was reported. This case was also accompanied by marked anemia, thrombocytopenia, leukoerythremoid reaction and hypofibrinogenemia. The spread of tumor was so wide that only the alimentary tract and the skin were free from metastases of the tumor. Many of the metastatic foci showed marked hemopoiesis intra tumori. A short discussion was made on the hematological abnormalities associated with vascular tumors.

Adult↗

Hypofibrinogenemia in a girl with Langerhans cell histiocytosis during etoposide and prednisolone therapy.

A case of a girl with Langerhans cell histiocytosis who had hypofibrinogenemia during etoposide (VP-16) and prednisolone therapy is described. A patient on a three times per week schedule of 100 mg/m2 etoposide in combination with 40 mg/m2 of prednisolone every day had hypofibrinogenemia of 90 mg/dL after 12 doses of etoposide. Hypofibrinogenemia improved after discontinuing the course of etoposide. The clinical course suggested that the combination of etoposide with prednisolone caused this side-effect. Although the exact mechanism of toxicity of etoposide with prednisolone remains unknown, it is possible that the side-effect might be due to reduced production of fibrinogen in the liver. Careful monitoring of fibrinogen is mandatory in patients receiving etoposide and prednisolone.

Afibrinogenemia↗

Morphological and biochemical confirmation of gray platelet syndrome in two siblings.

A case report is presented of gray platelet syndrome in siblings. The absence of platelet alpha-granules in these patients was confirmed by electron microscopy and by analysis of the platelet protein profile using SDS-polyacrylamide gel electrophoresis. This was further confirmed by quantitation of individual alpha-granule protein constituents. The results in these two patients are compared with those of the four reported patients with this syndrome.

Adult↗

Hypofibrinogenemia due to increased fibrinolysis in two patients with acute promyelocytic leukemia.

Two patients with acute promyelocytic leukemia and severe bleeding associated with hypofibrinogenemia were studied. The markedly shortened whole blood clot lysis time and dilute clot lysis time suggested that the defect was an increase in fibrinolysis. Although disseminated intravascular coagulation could not be totally excluded as an alternative mechanism, excessive fibrinolysis was confirmed as the pathogenic cause by the prompt response to the administration of tranexamic acid. The low circulating plasminogen, alpha 2 plasmin inhibitor level and the presence of alpha 2 plasmin inhibitor-protease complex in both patients suggested that the increased fibrinolysis probably resulted from the liberation of plasminogen activator from the promyelocyte.

Adult↗