Hepatosplenic Gamma Delta T-cell Lymphoma Presenting as Progressive Hepatosplenomegaly Highlighted during Pregnancy

A. Hussam, Petkar Mahir, Z. Abdulaziz, A. Mohammed, A. Mohammad, Ahmad Rita, Eldean Mouhammad Sharaf
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Abstract

Hepatosplenic T-cell lymphoma (HSGDTL) is a rare variant of T-cell lymphoma with few reported cases in literature. It usually presents with hepatosplenomegaly and constitutional symptoms, primarily in young to middle aged patients. The prognosis is generally poor, with maximum reported survival periods of 2-3 years. There is a link to immunode-ficiency and some biological treatments. Liver biopsy with flow cytometry is required to confirm the diagnosis. We report a case of gamma delta ( γδ ) HSTCL in a 37-year-old female presenting as progressive hepatosplenomegaly with cytopenia during pregnancy. The patient underwent chemotherapy, splenectomy and bone marrow transplant and is in remission 10 months after the diagnosis. As far as we are aware, this is the first case of γδ HSGDTL reported in the Middle East and the second documented case of HSGDTL that highlighted during pregnancy.
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妊娠期肝脾γ δ t细胞淋巴瘤表现为进行性肝脾肿大
肝脾t细胞淋巴瘤(HSGDTL)是一种罕见的t细胞淋巴瘤,文献报道病例很少。它通常表现为肝脾肿大和体质症状,主要见于中青年患者。预后一般较差,据报道最长生存期为2-3年。这与免疫缺陷和一些生物治疗有关。需要肝活检和流式细胞术来确认诊断。我们报告一个37岁女性的γδ (γδ) HSTCL病例,在怀孕期间表现为进行性肝脾肿大伴细胞减少。患者接受了化疗、脾切除和骨髓移植,确诊10个月后病情缓解。据我们所知,这是中东地区报道的第一例γδ HSGDTL病例,也是第二例记录的妊娠期HSGDTL病例。
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