Rabrenović Violeta, Mijušković Zoran, Marjanović Slobodan, Rabrenović Milorad, Jovanović Dragan, Antić Svetlana, Ignjatović Ljiljana, Petrović Milica, Pilcević Dejan
Vojnosanit Pregl. 2015 Feb;72(2):196-9. doi: 10.2298/vsp140210027r.
Immunoglobulin D (IgD) myeloma is a rare disease, about 2% of all myelomas, even rarer when accompanied with another multiple myeloma in biclonal gammopathy. We presented a case of biclonal gammopathy-as-sociated manifestation of IgD myeloma and light chain disease in a patient who initially had renal failure.
37-year-old male approximately one month before hospitalization began to feel malaise and fatigue along with decreased urination. Laboratory analysis revealed azotemia. A dialysis catheter was placed and hemodialysis started. The patient was then admitted to our hospital for further tests and during admission, objective examination revealed pronounced paleness with hepatosplenomegaly and hypertension (170/95 mmHg). Laboratory analysis showed erythrocyte sedimentation rate 122 mm/h, expressed anemic syndrome (Hb 71 g/L) and renal failure dialysis rank: creatinine 1,408 micromol/L, urea 31.7 mmol/L. There was two M components in serum protein electrophoresis: IgD lambda and free light chain lambda. Proteinuria was nephrotic rank (5.4 g/24 h), whose electrophoresis revealed 2 M components--massive in alpha 2 fraction of 71%; 7% in the discrete beta fraction, beta 2M / serum 110 mg / L, in urine 1.8 mg/L--extremely high; IgL kappa I lambda index 1:13 (reference value ratio 2:1). The findings pointed to double myeloma disease: IgD myeloma and Bence Jones lambda myeloma. Bone biopsy confirmed IgD myeloma lambda 100% infiltration medulla predominantly plasmablasts. The treatment continued with hemodialysis 3 times per week with chemotherapy protocol bortezomib, doxorubicin, dexamethasone. After 4 cycles of chemotherapy, there was a decrease of IgD, lamda-light chains, reduction in proteinuria (1.03 g/24 h), so hemodialysis was reduced to once per week. Six months after treatment initiation the patient underwent autologous bone marrow transplantation. In a 2-year follow-up period double myeloma disease showed complete remission.
The presented rare form of double myeloma disease with initial renal insufficiency underscores the importance of careful observation and teamwork that can alter the course of this serious disease.
免疫球蛋白D(IgD)骨髓瘤是一种罕见疾病,约占所有骨髓瘤的2%,当与双克隆丙种球蛋白病中的另一种多发性骨髓瘤同时出现时则更为罕见。我们报告了一例IgD骨髓瘤和轻链病的双克隆丙种球蛋白病相关表现的病例,该患者最初患有肾衰竭。
一名37岁男性,在住院前约一个月开始感到不适、疲劳,尿量减少。实验室分析显示氮质血症。放置了透析导管并开始进行血液透析。随后患者因进一步检查入住我院,入院期间体格检查发现面色明显苍白,伴有肝脾肿大和高血压(170/95 mmHg)。实验室分析显示红细胞沉降率为122 mm/h,表现为贫血综合征(血红蛋白71 g/L),肾衰竭处于透析阶段:肌酐1408 μmol/L,尿素31.7 mmol/L。血清蛋白电泳中有两种M成分:IgD λ和游离轻链λ。蛋白尿为肾病范围(5.4 g/24 h),其电泳显示有两种M成分——71%大量存在于α2区带;7%存在于离散的β区带,β2微球蛋白/血清为110 mg/L,尿液中为1.8 mg/L——极高;IgL κ/λ指数为1:13(参考值比例为2:1)。这些发现指向双骨髓瘤疾病:IgD骨髓瘤和本斯·琼斯λ骨髓瘤。骨髓活检证实IgD骨髓瘤λ100%浸润骨髓,主要为成浆细胞。治疗继续每周进行3次血液透析,并采用硼替佐米、阿霉素、地塞米松化疗方案。4个化疗周期后,IgD、λ轻链减少,蛋白尿减少(1.03 g/24 h),因此血液透析减少至每周1次。治疗开始6个月后,患者接受了自体骨髓移植。在2年的随访期内,双骨髓瘤疾病显示完全缓解。
所呈现的这种伴有初始肾功能不全的罕见双骨髓瘤疾病形式强调了仔细观察和团队合作的重要性,这可以改变这种严重疾病的病程。