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多发性骨髓瘤:对62例连续尸检病例的临床病理研究。

Multiple myeloma: a clinicopathologic study of 62 consecutively autopsied cases.

作者信息

Kapadia S B

出版信息

Medicine (Baltimore). 1980 Sep;59(5):380-92.

PMID:7432154
Abstract

The clinical, laboratory and pathologic findings were studied in 62 consecutively autopsied patients with multiple myeloma between 1954 and 1975. All patients were 40 years of age or older. Bone pain was the initial symptom in 2/3 of patients. Anemia (81%), thrombocytopenia (29%), azotemia (41%), hypercalcemia (46%) and hyperuricemia (52%), were common laboratory findings at diagnosis. Ninety-seven percent had a monoclonal protein in serum or urine. Extensive plasma cell replacement of marrow was invariably seen at autopsy although in 15% of patients no abnormality was found on skeletal survey. Extraskeletal spread (67%) was due to direct extension to paraosseous tissue resulting from cortical destruction and to distant organ involvement mainly of splenic red pulp and hepatic sinusoids. The patients were susceptible to bacterial infection, mainly gram-negative, of the lung (56%), urinary tract (35%), and blood (24%). Fungal infection was less frequent and usually consisted of superficial candidal overgrowth of gastrointestinal tract ulcerations (18%). Amyloidosis (10%) was perivascular and associated with light chain proteinuria. Renal failure as a cause of death (21%) was secondary only to infection (52%). Severity of histologic findings in the kidney at autopsy had little correlation to initial BUN concentration. The median survival was 11.5 months with alkylating agent therapy (responders, 29 months; non-responders, 6 months), and 6 months with urethan. Initial azotemia (greater than 80 mg/dl) and hypercalcemia (greater than 12 mg/dl) were important prognostic indicators (median survival, less than 1 month and 3 months, respectively). A good response to alkylating agent therapy, initial BUN less than 40 mg/dl and serum calcium less than 12 mg/dl were favorable to prognostic indicators.

摘要

对1954年至1975年间连续62例多发性骨髓瘤尸检患者的临床、实验室及病理检查结果进行了研究。所有患者年龄均在40岁及以上。2/3的患者首发症状为骨痛。贫血(81%)、血小板减少(29%)、氮质血症(41%)、高钙血症(46%)和高尿酸血症(52%)是诊断时常见的实验室检查结果。97%的患者血清或尿液中有单克隆蛋白。尸检时均可见骨髓有广泛的浆细胞替代,尽管15%的患者骨骼检查未发现异常。骨外播散(67%)是由于皮质破坏导致直接蔓延至骨旁组织以及远处器官受累,主要是脾红髓和肝血窦。患者易发生细菌感染,主要为革兰阴性菌,肺部感染(56%)、尿路感染(35%)和血液感染(24%)。真菌感染较少见,通常为胃肠道溃疡表面念珠菌过度生长(18%)。淀粉样变性(10%)为血管周围性,与轻链蛋白尿有关。肾衰竭作为死亡原因(21%)仅次于感染(52%)。尸检时肾脏组织学检查结果的严重程度与初始血尿素氮浓度相关性较小。烷化剂治疗的中位生存期为11.5个月(有反应者为29个月,无反应者为6个月),脲烷治疗的中位生存期为6个月。初始氮质血症(大于80mg/dl)和高钙血症(大于12mg/dl)是重要的预后指标(中位生存期分别小于1个月和3个月)。对烷化剂治疗反应良好、初始血尿素氮小于40mg/dl和血清钙小于12mg/dl是有利的预后指标。

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