[A patient with anemia, hemorrhagic diathesis and anuria].
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Five patients had a histiocytic, cytophagic panniculitis and a febrile, progressive course, which included hepatosplenomegaly, pancytopenia, liver function abnormalities, and a hemorrhagic death. The terminal hemorrhage was characterized by features of intravascular coagulation and liver failure. The histiocytosis could be found at times also in bone marrow, lymph nodes, liver and spleen, and serosal tissues, as well as in skin and subcutaneous tissue. Adequate biopsy diagnosis will prevent such cases from being labeled as Weber-Christian disease with bleeding, and offer an opportunity for appropriate treatment of this new syndrome of histiocytic, cytophagic panniculitis.
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A 10-month-old Simmental heifer was examined because of a 10-day history of epistaxis and aural hematomas. Examination of the calf also revealed hemarthrosis. Initial laboratory data indicated that platelet count, platelet size, prothrombin time, and partial thromboplastin time were not different from a clinically normal (control) cow. Mucosal bleeding time was prolonged, and platelet adhesion to glass beads was less than expected. The clinical signs, prolonged bleeding time, and platelet adhesion defect were corrected by infusion of bovine plasma. Subsequent laboratory testing revealed that the affected calf had a truncated multimeric structure of von Willebrand factor (vWF), low vWF activity, and impaired platelet aggregation in response to adenosine diphosphate, but concentration of vWF was not different from that in clinically normal control animals. These data were consistent with a diagnosis of variant von Willebrand disease. The relationship of this disease to the thrombopathy of Simmental cattle is unclear.
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Twenty infants aged 2 weeks to 3 months with the diagnosis of bleeding disorder secondary to low prothrombin complex level were studied. Sixty children of the control group were matched to the cases by age +/- 2 weeks, sex and race. The ratio of boys to girls was 2.3:1. The median, mean, and range of age of the cases and controls were 43.5 days, 43.7 days, 21-73 days and 43.5 days, 46.8 days, 26-28 days respectively. Most of them were pale with a mean hematocrit of 23.55%. The partial thromboplastin time and prothrombin time were markedly prolonged. The means of vitamin K dependent coagulation factors II, VII, IX and X were 1.10%, 5.87%, 2.86%, and 4.47% of adult activity, respectively. The clinical manifestations related to the bleeding of the cases were drowsiness and convulsion (95%), pallor (85%), and apparent bleeding (10%). The sites of the bleeding were demonstrated in the cranial cavity (95%), gastrointestinal tract and oral cavity (15%), and skin (5%). Nineteen patients with intracranial hemorrhage had bleeding in the subdural space (79%), intracerebral (42%), intraventricular (32%), and subarachnoid space (5.2%). The mortality rate and permanent brain damage occurred in 10% and 45%, respectively. Only 45% of the cases recovered normally. The permanent neurological sequelaes were hemiparesis (44.4%), microcephaly (33.3%), convulsive disorder (33.3%), mental retardation (33.3%), spasticity (22.2%), and hydrocephalus (11.1%). Breast feeding alone up to the day of study (OR = 7.0, p < 0.005) was found to be a significant risk factor for bleeding in these infants.(ABSTRACT TRUNCATED AT 250 WORDS)
Dysplastic scoliosis often occurs with parallel hemostasis disorders and is fraught with the risk of intra- or postoperative risk of hemorrhagic complications. For early correction of hemostasis and adequate management of the patients, hemostasis parameters should be found out. Administration of indirect-action anticoagulants is risky. The use of heparin or fraxiparin is more valid. The latter of them maintains the threat of postoperative hemorrhage onset in the least degree.
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