Occult subcutaneous panniculitis-like T-cell lymphoma with initial presentations of cellulitis-like skin lesion and fulminant hemophagocytosis.
Occult subcutaneous panniculitis-like T-cell lymphoma with initial presentations of cellulitis-like skin lesion and fulminant hemophagocytosis.
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隐匿性皮下脂膜炎样 T 细胞淋巴瘤，最初表现为蜂窝组织炎样皮肤病变和暴发性噬血细胞增多症。
DOI：
10.1016/s0929-6646(09)60354-5
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发表时间：
2007
期刊：
影响因子：
--
通讯作者：
G. Chou
中科院分区：
文献类型：
--
作者：
Huey;C. Teng;Youngsen Yang;Ji‐Hsiung Young;G. Chou
噬血细胞综合征（HPS）可能是原发或继发于恶性肿瘤、感染、自身免疫性疾病或药物。在大多数情况下，HPS发生在诊断潜在恶性肿瘤或复发的同时。在罕见的情况下，肿瘤可以隐匿，甚至超过十年。我们报告一例28岁的妇女入院，因为HPS。依托泊苷治疗暴发性HPS是有效的。4个月后，由于腿部新出现皮下结节和蜂窝组织炎样皮肤病变，她再次住院。皮下结节切除活检显示皮下泛膜炎样t细胞淋巴瘤。依托泊苷、固美醇、阿拉伯糖胞嘧啶、顺铂方案化疗后，皮肤病变及皮下结节均消失。总之，对于表现为HPS和蜂窝组织炎样皮肤病变的患者，应考虑隐匿性淋巴瘤，可能数月甚至数年无法诊断。暴发性HPS患者应尽快开始适当的治疗，包括针对恶性淋巴瘤的类固醇和化疗，因为其致命的过程。[J]中华医学杂志，2007；106 (2): S55-S59]
Hemophagocytic syndrome (HPS) may be primary or secondary to malignancies, infections, autoimmune diseases, or drugs. In most cases, HPS occurs at the same time as the diagnosis of underlying malignancy or when it relapses. In rare situations, the neoplastic disease can be occult, even for more than a decade. We report the case of a 28-year-old woman admitted because of HPS. Treatment with etoposide for fulminant HPS was effective. Four months later, she was admitted again because of newly developed subcutaneous nodules and cellulitis-like skin lesions over her legs. Excisional biopsy of subcutaneous nodule showed subcutaneous panniculitis-like T-cell lymphoma. After etoposide, solumedrol, cytosine arabinoside, and cisplatin regimen chemotherapy, both skin lesions and subcutaneous nodules disappeared. In conclusion, for patients presenting with HPS and cellulitis-like skin lesions, occult lymphoma should be considered, which might not be diagnosed for months, even years. Adequate treatment, including steroid and chemotherapy against malignant lymphoma, should be started as soon as possible for patients with fulminant HPS because of its fatal course. [J Formos Med Assoc 2007;106(2 Suppl):S55-S59]