van Dongen Helena M, Koomen Bregje M, Jurgens Jordy, Leavis Helen L
Department of Rheumatology & Clinical Immunology, University Medical Center Utrecht, Utrecht, the Netherlands.
Department of Pathology, University Medical Center Utrecht, Utrecht, the Netherlands.
Case Rep Hematol. 2025 Sep 11;2025:9919411. doi: 10.1155/crh/9919411. eCollection 2025.
Intravascular large B-cell lymphoma (IVLBCL) has a high mortality rate, partly due to its heterogeneous presentation and rarity. We present a case of a 73-year-old woman who came into the emergency room in need of fluid resuscitation, interpreted as septic shock. However, broad-spectrum antibiotics gave no resolution, and no causative agent was found. Further physical examination showed proximal muscle weakness, Raynaud's phenomenon, and calcinosis cutis. During 3 weeks of admission, vasopressor support was required continuously due to a capillary leak syndrome. The patient passed away. The underlying malignancy was only revealed at autopsy. To the best of our knowledge, this is the first case of IVLBCL with hypovolemic shock due to systemic capillary leak syndrome in combination with a wide range of immunological phenomena.
血管内大B细胞淋巴瘤（IVLBCL）死亡率很高，部分原因是其表现多样且罕见。我们报告一例73岁女性病例，该患者因液体复苏需求进入急诊室，最初被诊断为感染性休克。然而，广谱抗生素治疗无效，未发现病原体。进一步体格检查发现近端肌无力、雷诺现象和皮肤钙质沉着。住院3周期间，由于毛细血管渗漏综合征，患者持续需要血管升压药支持。患者最终死亡。潜在的恶性肿瘤仅在尸检时才被发现。据我们所知，这是首例因系统性毛细血管渗漏综合征合并多种免疫现象导致低血容量性休克的IVLBCL病例。
