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多发性骨髓瘤相关淀粉样变性是一个独立的高风险预后因素。

Multiple myeloma-associated amyloidosis is an independent high-risk prognostic factor.

作者信息

Vela-Ojeda J, García-Ruiz Esparza M A, Padilla-González Y, Sánchez-Cortes E, García-Chávez J, Montiel-Cervantes L, Reyes-Maldonado E, Majluf-Cruz A, Mayani H

机构信息

Hematology Department, Unidad Médica de Alta Especialidad, Hospital de Especialidades Centro Médico Nacional La Raza, IMSS, México DF, México.

出版信息

Ann Hematol. 2009 Jan;88(1):59-66. doi: 10.1007/s00277-008-0554-0. Epub 2008 Jul 22.

Abstract

Several prognostic factors have been recognized in patients with multiple myeloma (MM). Among the most important are: the serum levels of beta2-microglobulin, albumin, and LDH; the labeling index; and an abnormal karyotype. Patients with amyloidosis (AL) have poor prognosis; however, little is known concerning the prognostic significance of AL associated to MM. In 201 consecutive patients with de novo MM, we performed a fat-pad biopsy needle aspiration (FPBNA) that was stained with Congo red. Sixty eight (34%) patients had AL and a poorer prognosis disease: lower performance status, presence of B symptoms, higher LDH and calcium values, and worse response to chemotherapy. Cox regression model for overall survival detected three variables having independent prognostic significance: the presence of AL (RR = 3.4, P < 0.004), serum albumin levels <3.5 g/dl (RR 3.2, p < 0.005), and patients not achieving complete remission or very good partial remission (RR 2.9, p < 0.02). In 28% of patients with de novo MM, FPBNA was useful to detect incidental amyloidosis. During follow-up, 69% of these patients had symptoms of AL. Excluding 16 patients with obvious symptoms of AL at diagnosis, overall survival was worse in patients who developed later symptoms of AL. MM-associated AL represents a poorer prognosis disease even in the absence of symptoms at diagnosis, and this specific association may be considered as an independent high-risk prognostic factor. The routine study of periumbilical fat-pad tissue should be mandatory in all patients with MM.

摘要

多发性骨髓瘤(MM)患者中已确认了几个预后因素。其中最重要的是:β2-微球蛋白、白蛋白和乳酸脱氢酶(LDH)的血清水平;标记指数;以及异常核型。淀粉样变性(AL)患者预后较差;然而,关于与MM相关的AL的预后意义知之甚少。在201例初发MM患者中,我们进行了刚果红染色的脂肪垫穿刺活检(FPBNA)。68例(34%)患者有AL且疾病预后较差:体能状态较低、存在B症状、LDH和钙值较高,以及对化疗的反应较差。总生存的Cox回归模型检测到三个具有独立预后意义的变量:存在AL(风险比[RR]=3.4,P<0.004)、血清白蛋白水平<3.5g/dl(RR 3.2,p<0.005),以及未达到完全缓解或非常好的部分缓解的患者(RR 2.9,p<0.02)。在28%的初发MM患者中,FPBNA有助于检测偶发性淀粉样变性。在随访期间,这些患者中有69%出现了AL症状。排除诊断时具有明显AL症状的16例患者,后来出现AL症状的患者总生存更差。即使在诊断时无症状,MM相关的AL也代表预后较差的疾病,这种特定关联可被视为一个独立的高危预后因素。所有MM患者均应常规检查脐周脂肪垫组织。

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