A CASE WITH ACUTE RENAL FAILURE COMPLICATED BY WALDENSTRÖM'S MACROGLOBULINEMIA AND CRYOGLOBULINEMIA

We encountered a 53-year-old man associated with acute renal failure caused by Waldenström's macroglobulinemia and type I cryoglobulinemia. Treatment with prednisolone and cyclophosphamide induced a rapid recovery from acute renal failure. Renal histology revealed endocapillary proliferation and lobular formation with scattered subendothelial, amorphous and periodic acid-Schiff (PAS)-positive materials in the glomerular capillaries which were positive for IgM on immunofluorescence study. Although the exact mechanism for pathophysiology of acute renal failure remains unknown, treatment with prednisolone and cyclophosphamide could induce a rapid recovery from acute renal failure accompanied by Waldenström's macroglobulinemia and type I cryoglobulinemia.     

Investigation and management of IgM and Waldenström‐associated peripheral neuropathies: recommendations from the IWWM‐8 consensus panel

Paraproteinaemic neuropathies are a heterogeneous group of disorders most frequently associated with IgM monoclonal gammopathies including Waldenström macroglobulinaemia (WM ). Their consequences are significant for affected patients, and their management challenging for their physicians. The variability in clinical presentation and time course hamper classification and management. The indications for invasive investigations such as cerebrospinal fluid analysis, nerve conduction tests and sensory nerve biopsies are unclear, and the optimum way to measure clinical response to treatment unknown. When to intervene and and how to treat, also present challenges to physicians. As part of its latest deliberations at the International Workshops on WM (IWWM ) in London, UK (August 2014), the IWWM 8 panel have proposed a consensus approach to the diagnosis and management of peripheral neuropathies associated with IgM monoclonal gammopathies, including WM . Importantly, a consensus regarding the use of clinical outcome measures and recommended models of care for this group of patients is discussed, as well as appropriate treatment interventions.     

Diffuse Interstitial Lung Disease as a First Manifestation of Waldenström's Macroglobulinemia: Case Report and Review of the Literature

Waldenström's macroglobulinemia (WM) is a lymphoid malignancy characterized by infiltration, mainly of the bone marrow and lymph nodes, by small mature lymphocytes showing plasmacytoid differentiation, associated with an IgM monoclonal band, and in general, a low degree of aggressiveness. We present the first case reported in the Spanish literature of interstitial lung disease presenting as MW and we review the literature.     

LOW MOLECULAR WEIGHT IgM IN B CELL LYMPHOPROLIFERATIVE DISORDERS

Circulating low molecular weight (LMW) IgM was demonstrated in five of 38 patients with B cell lymphoproliferative disorders. These five patients all had malignant disease and could be subdivided into two groups. In the first group were three patients, each with an associated serum IgM paraprotein; two had Waldenstrom's macroglobulinemia. and one lymphocytic lymphoma. The two patients of the second group did not have IgM paraproteins; one had lymphocytic lymphoma and one chronic lymphocytic leukemia. Both these patients also had acquired C1 esterase inhibitor deficiency, a previously recognised association with circulating LMW IgM. None of the 16 patients with benign IgM macroglobulinemia had circulating LMW IgM. In those positive sera with LMW IgM this moiety contributed between 10.5% and 37.5% of the total IgM. There was no apparent association between LMW IgM and total IgM levels, kappallambda typing or the presence of Bence Jones proteinuria. but rheumatoid factor, immune complexes and cryoglobulins occurred in many of the sera which contained LMW IgM. Pokeweed mitogen stimulated peripheral blood mononuclear cells from two patients with circulating LMW IgM secreted considerable quantities of this moiety in vitrobut this did not occur in two patients with benign IgM macroglobulinemia. We conclude that LMW IgM is found in the malignant but riot the benign forms of B cell lymphoproliferative disorders and is frequently associated with other serological abnormalities. The basic abnormality causing defective IgM polymerisation in these disorders is obscure.     

原发性巨球蛋白血症1例报告并文献复习

 目的 探讨原发性巨球蛋白血症（Waldenstrom’s macroglobulinemia,WM）的临床特点、免疫表型、遗传学改变及诊疗措施。方法 回顾分析一例原发性巨球蛋白血症患者的临床资料。结果 本病例经血清固定电泳、骨髓形态学、细胞免疫分型等检测结果证实为原发性巨球蛋白血症,患者经过8个疗程的化疗后,恢复良好,目前仍在定期随访之中。结论 WM有特殊的免疫表型及细胞遗传学的改变,在临床工作中如果遇到单克隆IgM增高的病例,建议要考虑到WM的可能性,进一步完善免疫表型和细胞遗传学检测。     

24例华氏巨球蛋白血症临床分析

目的 华氏巨球蛋白血症(Waldenstr(o)m's macroglobulinemia,WM)是一种特殊类型的非霍奇金淋巴瘤,其发病率极低,国内外相关数据较为缺乏.本研究旨在探讨WM的临床特点、预后因素及诊治方法. 方法 回顾性分析2011-01-01-2016-01-01郑州大学人民医院诊治的24例WM患者临床资料. 结果 24例WM患者,男16例,女8例,男女比例为2∶1,年龄42~79岁,中位年龄62.5岁;贫血(20例,83.3%)是最常见的临床表现,中位血红蛋白水平75(46~145) g/L;中位IgM水平32.9(6.4~79.3) g/L,其中κ型18例(75%),λ型6例(25%).16例患者行流式细胞术检测,13例(81.3%)表现为sIgM+CD5-CD10-CD19+CD20+CD22+CD23-.全组中位无进展生存时间(PFS)为7.5(1~51)个月.单因素分析结果显示,年龄、血红蛋白(Hb)、血小板(PLT)、β2微球蛋白(β2-MG)、IgM水平、白蛋白(ALB)、血清肌酐(SCr)、乳酸脱氢酶(LDH)、C反应蛋白(CRP)和合并重度免疫不全麻痹影响患者PFS,应用含有利妥昔单抗或硼替佐米的化疗方案组PFS相对较长.多因素分析结果显示,年龄(P=0.008)、IgM水平(P=0.028)和SCr(P=0.005)与预后相关. 结论 WM好发于老年男性,以IgM κ型多见,具有惰性B细胞淋巴瘤的特点,年龄、IgM水平和SCr是影响WM预后的独立危险因素,利妥昔单抗或硼替佐米的应用有望延长PFS.