Modarressi Amir, Kuriyan Mercy, Harvey Gail, Strair Roger
Department of Medicine, Division of Hematology, UMDNJ- Robert Wood Johnson Medical School, New Brunswick, NJ 08901, USA.
J Clin Apher. 2003;18(4):190-3. doi: 10.1002/jca.10073.
Cryoglobulins are immunoglobulins that have tendency to precipitate in temperatures below 37 degrees C and dissolve with rewarming. Monoclonal cryoglobulins are usually associated with a distinct hematological disorder and often are asymptomatic. Heat insoluble cryoglobulin has been described with Sjogren's syndrome and glomerulonephritis but, not with multiple myeloma. Severe sensitivity to cold occurs with high thermal insolubility of the cryoprotein, with dramatic symptoms when exposed to minimal lowering of the temperature. We report a case of a 49 year old man with multiple myeloma and an unusual type I cryoglobulin that caused occlusive gangrene. The cryoglobulin appeared as a milky white precipitate that was resistant to re-suspension and did not dissolve at 37 degrees C. Immunoelectrophoresis of the cryoglobulin, which dissolved at 56 degrees C, showed it to be composed of a monoclonal IgG kappa protein (3.5 g/dl). Unlike most high thermal insoluble cryoglobulin, cold associated symptoms were not seen. In addition to steroids, plasmapheresis was initiated thrice a week with albumin fluid replacement. Plasmapheresis caused a marked decline in cryocrit levels from 21% to less than 0.5% in 9 days after 4 procedures with resolution of the gangrene of the feet and after 6 treatments, vasculitic symptoms improved dramatically. The cryoglobulin test was negative 2 weeks after initiation of treatment. The patient was treated for the myeloma and there was no recurrence of occlusive symptoms. Proper laboratory procedure and careful examination and handling of cryoglobulinemic samples facilitate detection of unusual cryoglobulins. This is a unique report of multiple myeloma with gangrene of lower extremities that has a heat insoluble cryoglobulin.
冷球蛋白是一种免疫球蛋白，在温度低于37摄氏度时容易沉淀，复温后溶解。单克隆冷球蛋白通常与一种独特的血液系统疾病相关，且往往无症状。热不溶性冷球蛋白已在干燥综合征和肾小球肾炎中有所描述，但未见于多发性骨髓瘤。冷蛋白的高热不溶性会导致严重的冷敏感，暴露于温度稍有降低时就会出现明显症状。我们报告一例49岁男性多发性骨髓瘤患者，其有一种不寻常的I型冷球蛋白，导致了闭塞性坏疽。冷球蛋白呈乳白色沉淀，难以重新悬浮，在37摄氏度时不溶解。在56摄氏度时溶解的冷球蛋白免疫电泳显示，它由一种单克隆IgG κ蛋白（3.5 g/dl）组成。与大多数高热不溶性冷球蛋白不同，未观察到与寒冷相关的症状。除了使用类固醇外，每周进行三次血浆置换，并补充白蛋白液。经过4次治疗后9天内，血浆置换使冷球蛋白水平从21%显著下降至低于0.5%，足部坏疽消退，6次治疗后血管炎症状明显改善。治疗开始2周后冷球蛋白检测呈阴性。该患者接受了骨髓瘤治疗，闭塞性症状未复发。正确的实验室操作以及对冷球蛋白血症样本的仔细检查和处理有助于检测不寻常的冷球蛋白。这是一份关于多发性骨髓瘤合并下肢坏疽且伴有热不溶性冷球蛋白的独特报告。
