Afiat Thanh-Phuong, Zhang Xiaohui, Zhang Hailing, Ayala Ernesto, Zhang Ling, Sokol Lubomir
Department of Internal Medicine, College of Medicine, University of South Florida, 12902 USF Magnolia Drive, Tampa, FL 33612, USA.
Department of Hematopathology and Laboratory Medicine, Moffitt Cancer Center, Tampa, FL, USA.
Leuk Res Rep. 2018 May 1;9:72-75. doi: 10.1016/j.lrr.2018.04.006. eCollection 2018.
Posttransplant lymphoproliferative disorders (PTLDs) of T-cell orgin are rare biologically heterogeneous diseases of mature lymphoid cells manifesting in immunosuppressed patients. Only a few cases of mycosis fungoides diagnosed post allogeneic hematopoietic cell transplant (alloHSCT) have been described so far. We present a patient with myelodysplastic syndrome (MDS) post matched unrelated donor alloHSCT who was on long-term immunosuppressive therapy due to graft versus host disease. Three years after an alloHSCT, she developed generalized erythroderma and peripheral blood lymphocytosis. Both skin biopsy and peripheral blood flow cytometry revealed atypical CD4+ T-cell population consistent with diagnosis of Sezary syndrome. Chimerism studies revealed 100% donor engraftment. Therapy with extracorporeal photopheresis resulted in complete response in blood and skin.
T细胞起源的移植后淋巴增殖性疾病（PTLD）是成熟淋巴细胞罕见的生物学异质性疾病，见于免疫抑制患者。迄今为止，仅描述了少数几例异基因造血细胞移植（alloHSCT）后诊断为蕈样肉芽肿的病例。我们报告1例接受匹配无关供者alloHSCT后的骨髓增生异常综合征（MDS）患者，因移植物抗宿主病接受长期免疫抑制治疗。alloHSCT三年后，她出现泛发性红皮病和外周血淋巴细胞增多。皮肤活检和外周血流式细胞术均显示非典型CD4+T细胞群，符合Sezary综合征的诊断。嵌合研究显示供者100%植入。体外光化学疗法治疗后血液和皮肤均完全缓解。
