Department of Internal Medicine C, Wolfson Medical Center, 61 Halochamim Street, 58100, Holon, Israel.
Department of Intensive Care, Wolfson Medical Center, Holon, Israel.
Clin Rheumatol. 2019 May;38(5):1257-1262. doi: 10.1007/s10067-018-04423-y. Epub 2019 Jan 10.
Type I cryoglobulinemia is associated with B cell proliferative diseases, whereas essential mixed cryoglobulinemia is classically associated with infections, malignancy, and autoimmune diseases, but may be idiopathic. Prognosis in patients with grave manifestations and renal involvement is often poor. We report a case of a 40-year-old woman, 2 weeks post-partum for pre-eclampsia who was hospitalized with nephritic syndrome and acute renal failure. The patient harbored type I and type II cryoglobulinemia. Renal and cutaneous biopsies confirmed the diagnosis; however, an underlying etiology was not established. A bone marrow biopsy suggested monoclonal gammopathy of undetermined source (MGUS). Despite therapy with intravenous cyclophosphamide, rituximab, plasmapheresis, dialysis, and bortezomib, the patient succumbed after 8 months of hospitalization. We suggest that an overlap entity of types I and II cryoglobulinemia with severe multi-organ involvement not only is rare but also may be resistant to conventional therapy and fatal.
I 型冷球蛋白血症与 B 细胞增殖性疾病相关，而原发性混合性冷球蛋白血症经典地与感染、恶性肿瘤和自身免疫性疾病相关，但也可能是特发性的。有严重表现和肾脏受累的患者预后通常较差。我们报告了一例 40 岁女性，因子痫前期产后 2 周，因肾病综合征和急性肾衰竭住院。该患者患有 I 型和 II 型冷球蛋白血症。肾和皮肤活检证实了诊断；然而，未确定潜在病因。骨髓活检提示意义未明的单克隆丙种球蛋白病（MGUS）。尽管接受了静脉注射环磷酰胺、利妥昔单抗、血浆置换、透析和硼替佐米治疗，但患者在住院 8 个月后死亡。我们建议，I 型和 II 型冷球蛋白血症的重叠实体伴有严重的多器官受累不仅罕见，而且可能对常规治疗有抗性，是致命的。
