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[多发性骨髓瘤的临床表现]

[The clinical picture of multiple myeloma].

作者信息

Ludwig H

出版信息

Onkologie. 1986 Aug;9(4):202-4, 206-8. doi: 10.1159/000216007.

DOI:10.1159/000216007
PMID:3531947
Abstract

Diagnosis of multiple myeloma is based on the triad paraproteinemia, osteolytic bone lesions and bone marrow plasma cell infiltration. Clinically, rheumatoid-like pain induced by osteolytic skeletal lesions often prevails. Occasionally, foudroyant bacterial infections - the most frequent cause of death in myelomatosis - or acute/subacute renal failure or rarely, acute hemi- or paraparesis precede diagnosis. Establishment of diagnosis early in the course of the disease and improved cytostatic and symptomatic treatment has led to a decrease in episodes of hyperviscosity-syndromes. Severe renal insufficiency due to Bence-Jones proteinuria prevails in 20% of patients already at time of diagnosis. With increasing duration of the disease, frequency of renal insufficiency further increases. Hypercalcemia with consecutive dehydration and renal insufficiency usually is a complication of long-standing disease. Anemia, leukopenia and thrombo-cytopenia are not only side effects of cytostatic treatment, but also consequences of tumor-induced suppression of hematopoiesis. Polyneuropathies are common in myelomatosis. They probably are the result of specific and/or unspecific binding of paraproteins to myelin sheaths. Effective treatment for this complication is not available at present. Thrombohemorrhagic complications are more frequent in patients with myeloma than in the control group of other hospitalized patients. Non-secretory myeloma, osteoblastic myeloma and Takatsuki syndrome are variants of myelomatosis. Solitary and extramedullary plasmocytoma are different, potentially curable entities. Prognosis is especially poor in patients with plasma cell leukemia and poor in primary amyloidosis.

摘要

多发性骨髓瘤的诊断基于以下三联征

副蛋白血症、溶骨性骨病变和骨髓浆细胞浸润。临床上,溶骨性骨骼病变引起的类风湿样疼痛较为常见。偶尔,暴发性细菌感染(骨髓瘤病最常见的死亡原因)、急性/亚急性肾衰竭,或很少见的急性半身轻瘫或截瘫在诊断之前出现。在疾病过程中早期确立诊断以及改进细胞毒性和对症治疗已导致高黏滞综合征发作减少。诊断时已有20%的患者因本-周氏蛋白尿出现严重肾功能不全。随着疾病病程延长,肾功能不全的发生率进一步增加。高钙血症伴连续脱水和肾功能不全通常是长期疾病的并发症。贫血、白细胞减少和血小板减少不仅是细胞毒性治疗的副作用,也是肿瘤诱导的造血抑制的后果。多发性神经病在骨髓瘤病中很常见。它们可能是副蛋白与髓鞘特异性和/或非特异性结合的结果。目前尚无针对该并发症的有效治疗方法。骨髓瘤患者的血栓出血并发症比其他住院患者的对照组更常见。非分泌型骨髓瘤、成骨性骨髓瘤和高月综合征是骨髓瘤病的变异型。孤立性和髓外浆细胞瘤是不同的、可能治愈的实体。浆细胞白血病患者的预后尤其差,原发性淀粉样变性的预后也较差。

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