Saka Bulent, Kalayoglu-Besisik Sevgi, Ozturk Gulistan Bahat, Dogan Oner, Erten Nilgun
Department of Internal Medicine, Faculty of Medicine, Istanbul University, Istanbul, Turkey.
J Formos Med Assoc. 2008 Feb;107(2):185-90. doi: 10.1016/S0929-6646(08)60133-3.
Patients with primary biliary cirrhosis (PBC) are at increased risk for various malignancies including lymphoproliferative diseases. In this report, we describe a 52-year-old man with the simultaneous diagnosis of PBC and IgG-kappa multiple myeloma (MM). Serum cholestatic enzyme (alkaline phosphatase and gamma-glutamyltransferase) levels decreased after three courses of vincristine, adriamycin and dexamethasone treatment followed by six courses of melphalan and methylprednisolone, given for MM. Pruritus also disappeared. He did not show any progression during the next 24 months of clinical follow-up. The coexistence of PBC and MM is rare and the pathogenetic mechanism under this association remains unclear. Clinical improvement of both diseases in this patient after chemotherapy may indicate an association; however, absence of concurrency in previous large series increases the possibility of coincidence in this case. When compared with the previous single case reports, this case provided long-term results about response after chemotherapy.
原发性胆汁性肝硬化(PBC)患者发生包括淋巴增殖性疾病在内的各种恶性肿瘤的风险增加。在本报告中,我们描述了一名52岁男性,同时诊断为PBC和IgG-κ多发性骨髓瘤(MM)。给予MM进行三个疗程的长春新碱、阿霉素和地塞米松治疗,随后进行六个疗程的美法仑和甲基强的松龙治疗后,血清胆汁淤积酶(碱性磷酸酶和γ-谷氨酰转移酶)水平下降。瘙痒也消失了。在接下来24个月的临床随访中,他没有出现任何病情进展。PBC和MM并存的情况很少见,这种关联背后的发病机制仍不清楚。该患者化疗后两种疾病均有临床改善,这可能表明存在关联;然而,之前的大型系列研究中不存在并发情况,增加了本例为巧合的可能性。与之前的单例报告相比,本例提供了化疗后反应的长期结果。