Lasri Najat, Lahlimi Fatimazahra, Tazi Mohammed Ilias, Rigaudeau Sophie
Service d´Hématologie Clinique et de Greffe de Moelle, Centre Hospitalier Universitaire Mohammed VI, Faculté de Médecine et de Pharmacie, Université Cadi Ayyad, Marrakech, Maroc.
Service d´Hématologie Clinique, Centre Hospitalier de Versailles André Mignot, Faculté de Médecine Xavier Bichat, Université Paris Diderot, Paris, France.
Pan Afr Med J. 2022 Apr 27;41:338. doi: 10.11604/pamj.2022.41.338.31755. eCollection 2022.
IgD myeloma is an extremely rare haemopathy with severe clinical presentation. It can be confused with non-secretory or free light chain myeloma. We here report the case of a 72-year old female patient presenting with bone pain and diffuse ecchymosis and deterioration of her general condition. Laboratory tests showed monoclonal gammopathy associated with severe acute renal failure and low total protein (TP) (48%) with factor X deficiency. Etiological assessment confirmed the diagnosis of IgD lambda myeloma stage IIIb, according to Durie and Salmon, International Staging System (ISS) score III unfavorable cytogenetics. Patient's outcome was favorable after treatment with proteasome inhibitor, anti-CD 38 and corticosteroid therapy. Adequate treatment of IgD myeloma, using new therapeutic approaches and hematopoietic stem cell autotransplantation, can improve the prognosis.
IgD骨髓瘤是一种极为罕见的血液疾病，临床表现严重。它可能会与非分泌型或游离轻链骨髓瘤相混淆。我们在此报告一例72岁女性患者，该患者出现骨痛、弥漫性瘀斑且全身状况恶化。实验室检查显示单克隆丙种球蛋白病，伴有严重急性肾衰竭、总蛋白（TP）水平低（48%）以及因子X缺乏。病因评估根据Durie和Salmon国际分期系统（ISS）评分III期、细胞遗传学不良，确诊为IIIb期IgD λ骨髓瘤。经蛋白酶体抑制剂、抗CD 38和皮质类固醇治疗后，患者预后良好。采用新的治疗方法和造血干细胞自体移植对IgD骨髓瘤进行充分治疗可改善预后。
