A registry of suspected cases of cancer-associated hemolytic-uremic syndrome (C-HUS) was established in May 1984. Records of 85 patients from the registry, all with history of cancer, hematocrit less than or equal to 25%, platelet count less than 100,000, and serum creatinine greater than or equal to 1.6 mg/dL were subjected to in-depth analysis. Eighty-nine percent of patients had adenocarcinoma, including 26% with gastric cancer. Microangiopathic hemolysis was reported in 83 patients; coagulation studies were normal with rare exception. Bone marrow examination ruled out chemotherapy-induced myelosuppression in 68 of 85. Thirty-five percent of patients were without evident cancer at time of syndrome development. Mitomycin (MMC) was part of the treatment regimen in 84 patients; all but nine received a cumulative dose greater than 60 mg. Pulmonary edema, generally noncardiogenic, developed in 65% of patients, often after blood product transfusions. C-HUS has a high mortality: over 50% of patients died of or with syndrome, most within 8 weeks of syndrome development. Conventional treatment was ineffective, although ten of 21 treated with staphylococcal protein A (SPA) immunopheresis showed significant responses. Statistical analysis found only absence of obvious tumor and treatment with SPA to suggest favorable prognosis. C-HUS is distinguishable from related syndromes such as childhood HUS, thrombotic thrombocytopenic purpura (TTP), consumption coagulopathy, and microangiopathic hemolysis associated with advanced carcinoma. MMC is likely involved in the development of C-HUS; the risk of developing C-HUS after treatment with MMC is between 4% and 15%. However, possible bias in patients referred to the registry and reports of non-MMC C-HUS cases must be remembered. Recommendations include careful monitoring of renal and hematologic function in patients treated with MMC, aggressive nontransfusion in patients with suspected C-HUS, and consideration of treatment with SPA immunopheresis in patients with definite syndrome.
Accuracy of certification of underlying cause of death and implications for US mortality statistics were assessed among 257 autopsied cases collected during the calendar year 1970 at a short-stay general hospital in Atlanta, GA. Clinicopathologic cause of death (CPCD) certificates, with assignment of underlying cause of death based on autopsy findings in combination with pertinent clinical data, were prepared by a pathologist and were employed as a standard of comparison against which the accuracy of the underlying cause of death on the original death certificate was measured. Results suggest that autopsy findings are not necessarily used to supplement clinical data in filling out death certificates. Improper recording of underlying cause of death was found in 42% of the autopsied cases. Malignant neoplasms were found to be underreported and vascular diseases overreported, each by approximately 10%, when original certificates were compared to CPCD certificates. The confirmation rate for original death certificate diagnoses was 89%. In the case of a confirmed diagnosis, the underlying cause of death was substantiated by postmortem findings as having existed regardless of its role in the sequence of events leading to death. The underlying cause of death as assigned by the pathologist was listed on the original death certificate among the sequence of events leading to or contributing to death at the rate of 72% (i.e., this rate measures the sensitivity of the death certificate).
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