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寡分泌型和非分泌型多发性骨髓瘤的临床特征和转归。

Clinical characteristics and outcomes of oligosecretory and non-secretory multiple myeloma.

机构信息

Department of Clinical Therapeutics, Alexandra Hospital, National and Kapodistrian University of Athens, Vasilissis Sofias 80, 11528, Athens, Greece.

Hematology division, Tel Aviv Sourasky Medical Center, Sackler faculty of medicine, Tel Aviv University, 6 Weizmann Street, 6423906, Tel Aviv, Israel.

出版信息

Ann Hematol. 2020 Jun;99(6):1251-1255. doi: 10.1007/s00277-020-03984-w. Epub 2020 Apr 19.

Abstract

Secretion of monoclonal immunoglobulins (MIg) detected in the serum and/or urine is one of the typical features of multiple myeloma (MM). However, some patients secrete MIg in quantities below "measurable" (termed oligosecretory MM) and others have no detectable MIgs by standard serum and urine immunofixation (termed non-secretory MM). In a cohort of 852 consecutive patients with active myeloma, we identified 100 (11.7%) patients with oligo/non-secretory MM, including 20 (2.3%) with non-secretory MM. Compared to patients with secretory MM, these were younger, less anemic, and had less often renal dysfunction and less extensive bone marrow infiltration. Presence and extent of bone disease were similar, however, hypercalcemia was less common and more often is ISS (International Staging System)-1 and, in those with available FISH (Fluoresense In Situ Hybridization) , high-risk cytogenetics were less common. FLCs (Free Light Chains) were available in 17 patients with non-secretory MM: only 3 had normal FLC ratio; the others had abnormal ratio and 9/14 had involved FLC ≥ 100 mg/L. The 4-year OS for patients with oligo/non-secretory disease was 64% vs 58% for secretory MM. In multivariate analysis, oligo/non-secretory disease was not an independent prognostic factor per se. Thus, 12% of MM patients present with oligo/non-secretory disease at diagnosis and have different biologic characteristics but similar outcome to other MM patients.

摘要

单克隆免疫球蛋白 (MIg) 在血清和/或尿液中的分泌是多发性骨髓瘤 (MM) 的典型特征之一。然而,一些患者分泌的 MIg 数量低于“可测量”(称为寡分泌性 MM),而另一些患者通过标准血清和尿液免疫固定电泳法无法检测到 MIg(称为非分泌性 MM)。在一组 852 例活动性骨髓瘤患者中,我们确定了 100 例(11.7%)寡分泌性/非分泌性 MM 患者,其中 20 例(2.3%)为非分泌性 MM。与分泌性 MM 患者相比,这些患者年龄较小,贫血程度较轻,肾功能不全和骨髓浸润程度较低。然而,存在和程度的骨病相似,高钙血症不常见,ISS(国际分期系统)分期 1 更常见,并且在有可用 FISH(荧光原位杂交)的患者中,高危细胞遗传学更常见。在 17 例非分泌性 MM 患者中可获得 FLC(游离轻链):只有 3 例具有正常的 FLC 比值;其余患者的比值异常,9/14 例患者受累 FLC≥100mg/L。寡分泌性/非分泌性疾病患者的 4 年 OS 为 64%,而分泌性 MM 患者为 58%。在多变量分析中,寡分泌性/非分泌性疾病本身并不是一个独立的预后因素。因此,12%的 MM 患者在诊断时表现为寡分泌性/非分泌性疾病,具有不同的生物学特征,但与其他 MM 患者的预后相似。

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