Radojković Milica, Ristić Slobodan, Colović Natasa, Cemerikić-Martinović Vesna, Colović Milica
Srp Arh Celok Lek. 2011 Jan-Feb;139(1-2):95-8. doi: 10.2298/sarh1102095r.
Waldenström's macroglobulinaemia is a rare B cell lymphoproliferative disorder characterized by lymphoplasmocyte bone marrow infiltration and monoclonal IgM gammopathy. In the majority of cases, Waldenström's macroglobulinaemia is a chronic disease with variable course. Therapy consists of alkylating agents, purine analogs and antiCD20 monoclonal antibody. In the literature, there have been descriptions of rare cases of progression of Waldenström's macroglobulinaemia to aggressive lymphoma, as well as secondary carcinoma in the patients after treatment of macroglobulinaemia.
A 63-year-old patient was diagnosed with serum monoclonal IgM kappa gammopathy (Waldenström's macroglobulinaemia). Chemotherapy was applied and a good clinical and haematological response had been achieved. Ten years later, the patient was diagnosed with colon adenocarcinoma as a secondary malignancy, and operated on. Within one month, the patient rapidly developed a large neck tumour mass. Tumour biopsy revealed the diagnosis of diffuse large B-cell lymphoma with the expression of monoclonal lambda chain, which more likely pointed out to coexistence of two different B cell lymphoproliferative disorders, rather than the transformation of Waldenström's macroglobulinaemia to aggressive lymphoma. The patient was treated with chemotherapy following R-CHOP protocol, and clinical remission was achieved. Seven months later, despite the successful treatment of lymphoproliferative disorder, dissemination of adenocarcinoma led to the lethal outcome.
The patient was diagnosed with a rare occurrence of three neoplastic diseases: Waldenstrom's macroglobulinaemia, colon adenocarcinoma and diffuse large B cell lymphoma. The possible mechanisms of the combined appearance of lymphoproliferative and other malignant diseases include the previous treatment with alkylating agents, genetic, immunomodulatory and environmental factors.
华氏巨球蛋白血症是一种罕见的B细胞淋巴增殖性疾病，其特征为淋巴浆细胞浸润骨髓和单克隆IgM丙种球蛋白病。在大多数情况下，华氏巨球蛋白血症是一种病程多变的慢性疾病。治疗方法包括烷化剂、嘌呤类似物和抗CD20单克隆抗体。文献中曾有关于华氏巨球蛋白血症进展为侵袭性淋巴瘤的罕见病例描述，以及巨球蛋白血症患者治疗后发生继发性癌症的报道。
一名63岁患者被诊断为血清单克隆IgMκ型丙种球蛋白病（华氏巨球蛋白血症）。接受了化疗并取得了良好的临床和血液学反应。十年后，该患者被诊断出患有继发性恶性肿瘤结肠腺癌，并接受了手术。在一个月内，患者颈部迅速出现一个巨大肿瘤肿块。肿瘤活检显示诊断为弥漫性大B细胞淋巴瘤，伴有单克隆λ链表达，这更可能表明两种不同的B细胞淋巴增殖性疾病并存，而非华氏巨球蛋白血症转变为侵袭性淋巴瘤。该患者按照R-CHOP方案接受化疗，实现了临床缓解。七个月后，尽管淋巴增殖性疾病治疗成功，但腺癌的播散导致了致命结局。
该患者被诊断出罕见地同时患有三种肿瘤性疾病：华氏巨球蛋白血症、结肠腺癌和弥漫性大B细胞淋巴瘤。淋巴增殖性疾病与其他恶性疾病合并出现的可能机制包括先前使用烷化剂治疗、遗传、免疫调节和环境因素。
