Di Lucca-Chrisment J, Maubec E, Grossin M, Marinho E, Varet B, Maillard H, Crickx B
Service de dermatologie, hôpital Bichat-Claude-Bernard, AP-HP, université Paris VII Denis-Diderot, 46, rue Henri-Huchard, 75877 Paris cedex 18, France.
Ann Dermatol Venereol. 2009 Nov;136(11):800-5. doi: 10.1016/j.annder.2009.04.017. Epub 2009 Jul 25.
Mycosis fungoides during large cell transformation to lymphoma has a poor prognosis with mean survival of 36 months. Autologous stem cell transplantation is rarely proposed in this indication. We report the case of a young man still in complete remission for transformed mycosis fungoides 14 years after autologous stem cell transplantation.
A 25-year-old man presenting eczema-like patches since childhood was treated by chemotherapy for multiple lymphadenopathies considered as Hodgkin's lymphoma. He was referred with diffuse skin tumours and infiltrated patches. Histology of tumour samples revealed atypical T-cell infiltrate with epidermotropism and presence of more than 25% of large CD30-positive cells. Non-infiltrated patches showed small T-cell lymphoma with epidermotropism. Histological verification of a previous lymphadenopathy confirmed the diagnosis of transformed mycosis fungoides. Despite multiple courses of chemotherapy, the disease progressed, with neurological involvement in particular. Because of tumour aggressiveness, autologous stem cell transplantation was performed and resulted in rapid regression of the tumours, lymphadenopathy and neurological symptoms. Non-transformed mycosis fungoides patches persisted but were controlled with topical mechlorethamine. This patient is still in complete remission for tumour and extracutaneous lesions 14 years after the autograft.
This was probably a case of juvenile mycosis fungoides diagnosed and transformed in adult age. Neurological involvement by mycosis fungoides is rare and usually carries a drastic prognosis. To our knowledge, this is the longest remission of transformed mycosis fungoides seen after autograft. It highlights the value of this method in aggressive transformed mycosis fungoides, especially in patients ineligible for allograft.
蕈样肉芽肿发生大细胞转化为淋巴瘤时预后较差，平均生存期为36个月。在这种情况下很少建议进行自体干细胞移植。我们报告了一例年轻男性的病例，自体干细胞移植14年后，转化型蕈样肉芽肿仍处于完全缓解状态。
一名25岁男性自幼出现湿疹样斑块，因多处淋巴结肿大被视为霍奇金淋巴瘤接受化疗。他因弥漫性皮肤肿瘤和浸润性斑块前来就诊。肿瘤样本的组织学检查显示非典型T细胞浸润伴亲表皮性，且存在超过25%的大CD30阳性细胞。未浸润的斑块显示为亲表皮性小T细胞淋巴瘤。先前淋巴结病的组织学检查证实了转化型蕈样肉芽肿的诊断。尽管进行了多疗程化疗，疾病仍进展，尤其出现了神经受累。由于肿瘤侵袭性，进行了自体干细胞移植，肿瘤、淋巴结病和神经症状迅速消退。未转化的蕈样肉芽肿斑块持续存在，但通过外用氮芥得到控制。自体移植14年后，该患者的肿瘤和皮肤外病变仍处于完全缓解状态。
这可能是一例幼年型蕈样肉芽肿在成年期被诊断并发生转化的病例。蕈样肉芽肿累及神经罕见，通常预后严重。据我们所知，这是自体移植后转化型蕈样肉芽肿最长的缓解期。它凸显了这种方法在侵袭性转化型蕈样肉芽肿中的价值，尤其是在不符合异体移植条件的患者中。
