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1例与边缘区B细胞淋巴瘤相关的混合型II型冷球蛋白血症性血管炎

A Case of Mixed Type II Cryoglobulinemic Vasculitis Associated With Marginal Zone B-cell Lymphoma.

作者信息

Farrukh Larabe, Rosenberg Lisa, Waqar Hafiza H, Tehzeeb Javaria, Akhtar Muhammad F, Chaukiyal Pooja

机构信息

Internal Medicine, Albany Medical Center, Albany, USA.

Oncology Hematology, Albany Medical Center, Albany, USA.

出版信息

Cureus. 2022 Dec 27;14(12):e33001. doi: 10.7759/cureus.33001. eCollection 2022 Dec.

Abstract

Our patient is a male in his 40s with a past medical history of sickle cell trait, factor V Leiden mutation, marginal zone B-cell lymphoma, gastric mucosa-associated lymphoid tissue (MALT) lymphoma, and sarcoidosis who presented with the complaint of hemoptysis, dyspnea, abdominal pain, arthralgias, peripheral edema of the lower extremities with petechial rash, and oliguria. Investigations revealed acute kidney injury and bilateral transudative pleural effusion. Serology was positive for elevated rheumatoid factor, low complement components, and cryoglobulins. Renal biopsy showed membranoproliferative cryoglobulinemic glomerulonephritis with deposition of monoclonal IgM and IgG3 with kappa light chain and C3 component. The patient was diagnosed with mixed type II cryoglobulinemic vasculitis in the setting of untreated marginal B-cell lymphoma. He had a complex clinical course, requiring multiple intubations, hemodialysis, and treatment with intravenous immunoglobulin, plasmapheresis, steroids, and chemotherapy, to which he initially responded. During treatment, he developed cardiomyopathy associated with congestive heart failure and passed away due to cardiac arrest. We present a rare case of mixed type II cryoglobulinemic vasculitis secondary to untreated marginal zone B-cell lymphoma in a hepatitis C virus (HCV) negative patient, which has not been reported before.

摘要

我们的患者是一名40多岁的男性,既往有镰状细胞性状、因子V莱顿突变、边缘区B细胞淋巴瘤、胃黏膜相关淋巴组织(MALT)淋巴瘤和结节病病史,因咯血、呼吸困难、腹痛、关节痛、下肢伴有瘀点皮疹的外周水肿及少尿前来就诊。检查发现急性肾损伤和双侧漏出性胸腔积液。血清学检查显示类风湿因子升高、补体成分降低及冷球蛋白阳性。肾活检显示膜增生性冷球蛋白血症性肾小球肾炎,有单克隆IgM和IgG3伴κ轻链及C3成分沉积。该患者在未治疗的边缘B细胞淋巴瘤背景下被诊断为混合型II型冷球蛋白血症性血管炎。他的临床过程复杂,需要多次插管、血液透析以及静脉注射免疫球蛋白、血浆置换、类固醇和化疗治疗,最初对这些治疗有反应。在治疗期间,他发生了与充血性心力衰竭相关的心肌病,并因心脏骤停去世。我们报告了一例在丙型肝炎病毒(HCV)阴性患者中,由未治疗的边缘区B细胞淋巴瘤继发的混合型II型冷球蛋白血症性血管炎的罕见病例,此前尚未见报道。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9448/9879283/655d427682f9/cureus-0014-00000033001-i01.jpg

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