Fatal Hemophagocytic Syndrome in a Patient with Panniculitis-like T-cell Lymphoma and No Clinical Evidence of Disease
Fatal Hemophagocytic Syndrome in a Patient with Panniculitis-like T-cell Lymphoma and No Clinical Evidence of Disease
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脂膜炎样 T 细胞淋巴瘤患者出现致命噬血细胞综合征且无疾病临床证据
DOI：
10.1080/1042819031000104042
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发表时间：
2003
影响因子：
2.6
通讯作者：
O. Paltiel
中科院分区：
文献类型：
--
作者：
N. Goldschmidt;G. Amir;M. Krieger;L. Gilead;O. Paltiel
脂膜炎样T细胞淋巴瘤是一种少见的结节性T细胞淋巴瘤，临床表现为皮下结节。临床过程可以是无痛的或快速进展的，通常并发噬血细胞综合征。我们报告一位原发性皮下疾病的病人，对联合化疗有完全反应。该患者经历了早期复发，对挽救化疗有反应。然而，她不久后死于噬血细胞综合征，多微生物败血症和全身真菌感染。尸检时，骨髓或其他器官中没有淋巴瘤的证据。我们要强调的是，尽管本例尸检显示淋巴瘤临床活动性极小，甚至没有证据，但仍可能发生致命的噬血细胞综合征。
Panniculitis-like T-cell lymphoma is an uncommon type of extranodal T-cell lymphoma which presents clinically with subcutaneous nodules. The clinical course can either be indolent or rapidly progressive, often complicated by hemophagocytic syndrome. We report a patient with primary subcutaneous disease and initial complete response to combination chemotherapy. The patient experienced an early relapse which responded to salvage chemotherapy. However, she died shortly thereafter with hemophagocytic syndrome, polymicrobial sepsis and systemic fungal infection. At autopsy there was no evidence of lymphoma in the bone marrow or other organs. We emphasize that a fatal hemophagocytic syndrome can occur despite minimal or even without evidence of clinically active lymphoma as demonstrated by autopsy in this case.
影响因子：
3.5
作者：
E. Jaffe;L. Krenács;S. Kumar;D. Kingma;M. Raffeld
通讯作者：
E. Jaffe;L. Krenács;S. Kumar;D. Kingma;M. Raffeld