中华血液学杂志
中華血液學雜誌
중화혈액학잡지
Chinese Journal of Hematology
2013年
4期
341-344
,共4页
樊建玲%傅卫军%石昊天%周帆%袁振刚%张春阳%韦苇%叶飞%张慧
樊建玲%傅衛軍%石昊天%週帆%袁振剛%張春暘%韋葦%葉飛%張慧
번건령%부위군%석호천%주범%원진강%장춘양%위위%협비%장혜
免疫球蛋白,M型%多发性骨髓瘤%原位杂交,荧光%治疗结果%预后
免疫毬蛋白,M型%多髮性骨髓瘤%原位雜交,熒光%治療結果%預後
면역구단백,M형%다발성골수류%원위잡교,형광%치료결과%예후
Immunoglobulin%IgM%Multiple myeloma%Fluorescence in situ hybridization%Treatment%Prognosis
目的 提高对IgM型多发性骨髓瘤(MM)患者临床及实验室特征的认识.方法 收集4例IgM型MM患者的临床资料,对其临床及实验室特征进行总结、分析.结果 4例患者符合IgM型MM的诊断.4例均为男性,年龄54 ～ 69岁,初发症状包括骨痛、高黏滞血症、出血,3例轻链为κ型,1例为λ型,Durie-Salmon (D-S)分期均为ⅢA期,国际分期系统(ISS)分期:Ⅰ期1例,Ⅱ期3例.平均IgM 83.6(52.9 ～111.0)g/L,HGB 79.5(61.0 ～ 105.0) g/L,血钙3.20(2.11 ～6.00) mmol/L,肌酐104.3(56.0～ 171.0) μmol/L,骨髓浆细胞比例0.558(0.290 ～0.775),3例存在骨质破坏.3例患者行免疫表型检测均表达CD38、CD138,不表达CD19、CD20、CD117.染色体、荧光原位杂交检查结果显示4例患者均有IgH易位和1q21扩增,2例伴有13q和17p缺失,3例明确为t(11;14).3例予以硼替佐米为主的方案诱导化疗,达到部分反应至非常好的部分反应.随访至2012年11月30日,无进展生存时间仅6.0(2.5～7.0)个月,总生存时间仅为17.5(2.5 ～27.0)个月.结论 IgM型MM非常罕见,在所有类型MM患者中不到0.5％,细胞遗传学异常以t(11；14)和1q21阳性多见,硼替佐米为主的方案治疗有效,但患者病情进展迅速,总体预后较差.
目的 提高對IgM型多髮性骨髓瘤(MM)患者臨床及實驗室特徵的認識.方法 收集4例IgM型MM患者的臨床資料,對其臨床及實驗室特徵進行總結、分析.結果 4例患者符閤IgM型MM的診斷.4例均為男性,年齡54 ～ 69歲,初髮癥狀包括骨痛、高黏滯血癥、齣血,3例輕鏈為κ型,1例為λ型,Durie-Salmon (D-S)分期均為ⅢA期,國際分期繫統(ISS)分期:Ⅰ期1例,Ⅱ期3例.平均IgM 83.6(52.9 ～111.0)g/L,HGB 79.5(61.0 ～ 105.0) g/L,血鈣3.20(2.11 ～6.00) mmol/L,肌酐104.3(56.0～ 171.0) μmol/L,骨髓漿細胞比例0.558(0.290 ～0.775),3例存在骨質破壞.3例患者行免疫錶型檢測均錶達CD38、CD138,不錶達CD19、CD20、CD117.染色體、熒光原位雜交檢查結果顯示4例患者均有IgH易位和1q21擴增,2例伴有13q和17p缺失,3例明確為t(11;14).3例予以硼替佐米為主的方案誘導化療,達到部分反應至非常好的部分反應.隨訪至2012年11月30日,無進展生存時間僅6.0(2.5～7.0)箇月,總生存時間僅為17.5(2.5 ～27.0)箇月.結論 IgM型MM非常罕見,在所有類型MM患者中不到0.5％,細胞遺傳學異常以t(11；14)和1q21暘性多見,硼替佐米為主的方案治療有效,但患者病情進展迅速,總體預後較差.
목적 제고대IgM형다발성골수류(MM)환자림상급실험실특정적인식.방법 수집4례IgM형MM환자적림상자료,대기림상급실험실특정진행총결、분석.결과 4례환자부합IgM형MM적진단.4례균위남성,년령54 ～ 69세,초발증상포괄골통、고점체혈증、출혈,3례경련위κ형,1례위λ형,Durie-Salmon (D-S)분기균위ⅢA기,국제분기계통(ISS)분기:Ⅰ기1례,Ⅱ기3례.평균IgM 83.6(52.9 ～111.0)g/L,HGB 79.5(61.0 ～ 105.0) g/L,혈개3.20(2.11 ～6.00) mmol/L,기항104.3(56.0～ 171.0) μmol/L,골수장세포비례0.558(0.290 ～0.775),3례존재골질파배.3례환자행면역표형검측균표체CD38、CD138,불표체CD19、CD20、CD117.염색체、형광원위잡교검사결과현시4례환자균유IgH역위화1q21확증,2례반유13q화17p결실,3례명학위t(11;14).3례여이붕체좌미위주적방안유도화료,체도부분반응지비상호적부분반응.수방지2012년11월30일,무진전생존시간부6.0(2.5～7.0)개월,총생존시간부위17.5(2.5 ～27.0)개월.결론 IgM형MM비상한견,재소유류형MM환자중불도0.5％,세포유전학이상이t(11；14)화1q21양성다견,붕체좌미위주적방안치료유효,단환자병정진전신속,총체예후교차.
Objective To improve the understanding of the clinical and laboratory features of the IgM multiple myeloma (MM).Methods The clinical data of four cases of IgM MM patients were collected,their clinical and laboratory features were summarized and analyzed.Results Four patients met the criteria of IgM MM.They were all male.The age at the diagnosis ranged from 54 to 69 years.The primary symptoms included bone pain,hyperviscosity and bleeding.Three cases had κ-chain and only one case had λ-chain.They were all staged ⅢA according to the Durie-Salmon staging system (DSS).One case staged Ⅰ and three cases staged Ⅱ according to the international staging system (ISS).The average value of IgM,hemoglobin,serum calcium,creatinine and the proportion of bone marrow plasma cells were 83.6 (52.9-111.0) g/L,79.5 (61.0-105.0) g/L,3.20(2.11-6.00) mmol/L,104.3 (56.0-171.0) μmol/L and 0.558 (0.290-0.775),respectively.Bone destruction was found in 3 cases.Immunophenotypes of bone marrow plasma cells were analyzed in 3 patients.Results showed that these cells expressed CD38 and CD138,and did not express CD19,CD20 and CD117.Chromosome and fluorescence in situ hybridization (FISH) analysis were carried out in 4 cases and found that all of them had IgH translocations and 1q21 amplification,2 cases had 13q and 17p deletion,and 3 cases had t(11 ;14).Three patients received bortezomib-based regimens as induction therapy and reached partial response (PR)-very good partial response (VGPR).Followed up to November 30,2012,the median progress-free survival (PFS) and overall survival (OS) of the 4 cases were only 6.0(2.5-7.0) months and 17.5(2.5-27.0) months,respectively.Conclusions IgM MM is very rare and is no more than 0.5％ in all types of MM.IgM MM have frequent t (11 ; 14) and amp(1 q21).Bortezomib-based regimens are effective for it,however,the disease progresses rapidly and has poor prognosis.
