Baraniskin Alexander, Jähnert Andreas, Ahle Guido, Theile Anja, Kretzschmar Hans, Schmiegel Wolff, Schroers Roland
Department of Medicine, Hematology and Oncology, Ruhr-University of Bochum, Germany.
Onkologie. 2010;33(4):174-7. doi: 10.1159/000290674. Epub 2010 Mar 19.
Intravascular lymphoma (IVL) is an uncommon disease characterized by atypical lymphoid cells growing inside the lumina of small vessels. The diversity of clinical presentation due to possible involvement of multiple organs often complicates its diagnosis.
Here, we report on a case of IVL with rapidly progressive dementia and Coombs-negative hemolytic anemia. Interestingly, the erythrocytes exhibited a decreased osmotic resistance. Bone marrow histopathology revealed increased erythropoiesis and, finally, a small monoclonal B lymphocyte population. Cerebral magnetic resonance imaging (MRI) demonstrated few micro-bleedings. Computed tomography (CT) showed bilateral ground-glass opacity of the lungs. Within a few days, the patient developed respiratory failure and died. On post-mortem examination, intravascular large B-cell lymphoma with almost complete infiltration of the brain and lungs was diagnosed.
IVL should be considered early in situations of unexplained neuropsychiatric disease along with markedly elevated levels of lactic dehydrogenase, anemia, and hemolysis.
血管内淋巴瘤(IVL)是一种罕见疾病,其特征为非典型淋巴细胞在小血管腔内生长。由于可能累及多个器官,临床表现多样,常使诊断复杂化。
在此,我们报告一例伴有快速进展性痴呆和库姆斯试验阴性溶血性贫血的IVL病例。有趣的是,红细胞表现出渗透抵抗力下降。骨髓组织病理学显示红细胞生成增加,最终发现一小群单克隆B淋巴细胞。脑磁共振成像(MRI)显示少量微出血。计算机断层扫描(CT)显示肺部双侧磨玻璃样混浊。数天内,患者出现呼吸衰竭并死亡。尸检诊断为血管内大B细胞淋巴瘤,脑和肺几乎完全被浸润。
在出现无法解释的神经精神疾病,同时伴有乳酸脱氢酶水平显著升高、贫血和溶血的情况下,应尽早考虑血管内淋巴瘤。