Da Silva Constante V, Couvert H, Wolfromm A, Ilzkovitz M
Hematology Department, Institut Jules Bordet - Hôpital Universitaire de Bruxelles (H.U.B.), Université Libre de Bruxelles (ULB), Brussels, Belgium.
Internal Medicine Department, Institut Jules Bordet - Hôpital Universitaire de Bruxelles (H.U.B.), Université Libre de Bruxelles (ULB), Brussels, Belgium.
Leuk Res Rep. 2024 Dec 20;23:100498. doi: 10.1016/j.lrr.2024.100498. eCollection 2025.
We describe the case of a female patient with May-Hegglin syndrome who developed peripheral T-cell lymphoma not otherwise specified. The patient presents with systemic lupus erythematous phenotype and myelofibrosis secondary to T-cell lymphoma. Peripheral T-cell lymphoma not otherwise specified, represents 25 % of all peripheral T-cell lymphoma. Its diagnosis remains challenging due to the polymorphous clinical presentation and pathological heterogeneity. Myelofibrosis associated with malignant lymphomas is rare and peripheral T-cell lymphoma is even rarer. To our knowledge, this is the first case to describe an association between May-Hegglin syndrome and a peripheral T-cell lymphoma.
我们描述了一例患有May-Hegglin综合征的女性患者，该患者发生了非特指外周T细胞淋巴瘤。该患者表现出系统性红斑狼疮表型以及继发于T细胞淋巴瘤的骨髓纤维化。非特指外周T细胞淋巴瘤占所有外周T细胞淋巴瘤的25%。由于其临床表现多样和病理异质性，其诊断仍然具有挑战性。与恶性淋巴瘤相关的骨髓纤维化很罕见，外周T细胞淋巴瘤则更为罕见。据我们所知，这是第一例描述May-Hegglin综合征与外周T细胞淋巴瘤之间关联的病例。
