Waldenström’s macroglobulinemia

Waldenstr ö m 巨球蛋白血症
  • 文章类型: Case Reports
    冷球蛋白是在低于37°C的温度下沉淀并在再加热时溶解的免疫球蛋白。它们可以诱发肾脏受累的小血管血管炎。冷球蛋白血症性肾小球肾炎是一种罕见的表现,发生在单克隆丙种球蛋白病患者中。特别是Waldenström的巨球蛋白血症。我们介绍了一个52岁的患者,有皮肤血管炎和甲状腺功能减退症的病史,出现全身性水肿的人,中度贫血,高胆固醇血症,肾病范围蛋白尿12.69克/天,微血尿,动脉高血压,通过经典途径和低补体血症,无急性肾损伤,血清学研究阴性,冷球蛋白阳性。血清和尿蛋白电泳和免疫固定研究显示IgM和κ轻链的单克隆带。肾活检符合冷球蛋白血症性肾小球肾炎。在异常蛋白血症和冷球蛋白血症性肾小球肾炎的情况下,进行骨髓穿刺和活检,导致Waldenström巨球蛋白血症的诊断。已描述了与I型冷球蛋白血症相关的单克隆丙种球蛋白病。这种描述的关联并不常见,这就是为什么我们提出这个案例,以及文献综述。
    Cryoglobulins are immunoglobulins that precipitate at temperatures below 37 °C and dissolve upon reheating. They can induce small-vessel vasculitis with renal involvement. Cryoglobulinemic glomerulonephritis is a rare manifestation that occurs in patients with monoclonal gammopathy, specifically Waldenström\'s macroglobulinemia. We present the case of a 52-year-old patient with a history of cutaneous vasculitis and hypothyroidism, who presented with generalized edema, moderate anemia, hypercholesterolemia, nephrotic range proteinuria of 12.69 g/day, microhematuria, arterial hypertension, and hypocomplementemia via the classical pathway, without acute kidney injury and with negative serological studies and positive cryoglobulins in the second determination. Serum and urine protein electrophoresis and immunofixation studies showed a monoclonal band of IgM and kappa light chain. Renal biopsy was consistent with cryoglobulinemic glomerulonephritis. In the context of dysproteinemia and cryoglobulinemic glomerulonephritis, bone-marrow aspiration and biopsy were performed, leading to the diagnosis of Waldenström\'s macroglobulinemia. Monoclonal gammopathies have been described in association with type I cryoglobulinemias. This described association is uncommon, which is why we present this case, along with a review of the literature.
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  • 文章类型: Case Reports
    与严重急性呼吸综合征冠状病毒2(SARS-CoV-2)感染相关的实质性肺脓肿发展是一种罕见的并发症,文献中只报道了六起主要病例。我们介绍了一例Waldenström巨球蛋白血症患者,该患者在原发性SARS-CoV-2感染后发展为肺脓肿。我们介绍了一名63岁的男性患者,患有SARS-CoV-2感染,并有Waldenström巨球蛋白血症的病史,他在入院两周后出现了空洞性实质内肺脓肿,右下叶有空气-液体水平。患者出现败血症并出现急性呼吸衰竭,需要机械通气和重症监护。他接受了广谱抗生素治疗和吸入性引流,但不幸的是,由于他的严重临床状况,他在初次入院20天后死亡。COVID-19患者肺脓肿的发展,虽然罕见,可能是相当妥协的,甚至是致命的,尤其是免疫功能低下的患者。临床医生应该意识到这种潜在的并发症。
    Intraparenchymal lung abscess development associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is a rare complication, with only half a dozen primary cases having been reported in the literature. We present the case of a patient with Waldenström\'s macroglobulinemia who developed a lung abscess subsequent to a primary SARS-CoV-2 infection. We present a 63-year-old male patient with SARS-CoV-2 infection and a history of Waldenström\'s macroglobulinemia who developed a cavitating intraparenchymal lung abscess with an air-fluid level in his right lower lobe two weeks following admission to hospital. The patient became septic and developed acute respiratory failure requiring mechanical ventilation and intensive care. He was managed with broad-spectrum antibiotic therapy and aspiration drainage, but unfortunately due to his severe clinical condition died 20 days after his initial admission. The development of a lung abscess in patients with COVID-19, although rare, can be quite compromising and even prove fatal, especially in immunocompromised patients. Clinicians should be aware of this potential complication.
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  • 文章类型: Case Reports
    背景:Waldenström巨球蛋白血症(WM)与结间边缘区淋巴瘤(INMZL)并存很少,通常与预后不良有关。
    方法:我们介绍了一例因WM和INMZL导致继发性轻链淀粉样变性的中国女性患者,并对其全身治疗提供了意见。一名65岁的女性在6年前被诊断为WM,并接受布鲁顿酪氨酸激酶抑制剂单药治疗两年。她的INMZL由于左颈部淋巴结肿大而被证实。病人一年前出现双下肢水肿,并被诊断为继发性轻链淀粉样变性。BC方案治疗(利妥昔单抗375mg/m2,每月治疗6-8个疗程,和苯达莫司汀90mg/m2/天×2,每月六个疗程)开始,但由于毒副作用而不能耐受。给予以硼替佐米为基础的治疗两个月,包括硼替佐米,地塞米松,还有Zanubrutinb.双下肢水肿缓解,治疗效果评估为部分缓解。
    结论:建议进行详细的临床评估和积极的病因鉴定,以避免漏诊和误诊。
    BACKGROUND: The co-existence of Waldenström\'s macroglobulinemia (WM) with internodal marginal zone lymphoma (INMZL) is rare and often associated with poor prognosis.
    METHODS: We present a Chinese female patient who developed secondary light chain amyloidosis due to WM and INMZL and provides opinions on its systemic treatment. A 65-year-old woman was diagnosed with WM 6 years ago and received Bruton tyrosine kinase inhibitor monotherapy for two years. Her INMZL was confirmed due to left cervical lymphadenopathy. The patient presented with oedema in both lower limbs one year ago, and was diagnosed with secondary light chain amyloidosis. Treatment with the BC regimen (rituximab 375 mg/m2 monthly for 6-8 courses, and bendamustine 90 mg/m2 per day × 2, monthly for six courses) was initiated, but not tolerated due to toxic side effects. Bortezomib-based therapy was given for two months, including bortezomib, dexamethasone, and zanubrutinb. Oedema in both lower limbs was relieved and treatment efficacy was evaluated as partial remission.
    CONCLUSIONS: A detailed clinical evaluation and active identification of the aetiology are recommended to avoid missed diagnosis and misdiagnosis.
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  • 文章类型: Case Reports
    与利妥昔单抗或利妥昔单抗联合化疗相关的迟发性中性粒细胞减少症(LON)定义为在最后一次利妥昔单抗给药后至少四周开始的无法解释的绝对中性粒细胞计数≤1.5×109/L。LON很少见,其病理生理学仍然未知。对于发展为LON的患者的最佳管理,没有指南或共识策略。大多数患者在没有特殊治疗的情况下迅速康复,只有一些病例需要用粒细胞集落刺激因子(G-CSF)治疗,通常是快速反应。这里,我们描述了一名69岁的Waldenström巨球蛋白血症患者,在利妥昔单抗加苯达莫司汀后出现严重LON的脓毒症事件.通过骨髓检查确定粒细胞缺乏症的诊断。有趣的是,发现与患者粒细胞结合的抗中性粒细胞抗体提示自身免疫机制。患者对G-CSF无反应,但在高剂量的静脉内免疫球蛋白和全白细胞恢复后获得了快速反应。
    Late onset neutropenia (LON) related to rituximab or rituximab plus chemotherapy is defined as an unexplained absolute neutrophil count of ≤1.5 × 109/L starting at least four weeks after the last rituximab administration. LON is infrequent and its pathophysiology remains unknown. There are no guidelines or consensus strategies for the optimal management of patients developing LON. The majority of the patients recover promptly with no specific treatment and only some cases need to be managed with granulocytic colony stimulating factor (G-CSF), usually with a rapid response. Here, we describe a 69-year-old patient with Waldenström\'s macroglobulinemia who presented a septic event in the context of severe LON after rituximab plus bendamustine. The diagnosed of agranulocytosis was established by bone marrow examination. Interestingly, anti-neutrophil antibodies bound to the patient\'s granulocytes were found suggesting an autoimmune mechanism. The patient did not respond to G-CSF but achieved a rapid response after high doses of intravenous immunoglobulins with full white blood cell recovery.
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  • 文章类型: Case Reports
    BACKGROUND: Waldenström\'s macroglobulinemia is a rare B-cell lymphoma. The gold standard treatment for Waldenström\'s macroglobulinemia is an anti-CD20 antibody (rituximab) in combination with alkylating agents and dexamethasone. Treatment targeting the B-cell receptor such as ibrutinib (but not idelalisib) is currently approved for treatment of patients with relapsed or refractory Waldenström\'s macroglobulinemia.
    METHODS: We report a case of a 71-year-old white French man with Waldenström\'s macroglobulinemia who presented with acute renal failure and hyperviscosity syndrome. His Waldenström\'s macroglobulinemia was refractory to first-line treatment with rituximab, cyclophosphamide, and dexamethasone. Because of his hemorrhagic syndrome and medical history of recent myocardial infarction, we decided to treat him with idelalisib 150 mg twice daily instead of ibrutinib. We observed a very quick improvement in the patient\'s clinical status without need for dose adjustment.
    CONCLUSIONS: Our patient\'s case provides the first evidence, to the best of our knowledge, that idelalisib may be an efficient treatment option for patients with Waldenström\'s macroglobulinemia complicated by anuric renal failure and in whom ibrutinib is contraindicated.
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