Kohla Samah, Ibrahim Feryal, Bilal Ilham, Al Kuwari Einas, Al-Sabbagh Ahmad
Department of Laboratory Medicine and Pathology, Hematology Section, NCCCR, Hamad Medical Corporation, Doha, Qatar.
Department of Clinical Pathology, Hematology Division, Faculty of Medicine, Al-Azhar University, Cairo, Egypt.
Case Rep Oncol. 2020 Dec 18;13(3):1520-1529. doi: 10.1159/000512188. eCollection 2020 Sep-Dec.
Gamma-delta (γδ) T-cell lymphomas are very rare and aggressive neoplasms. We describe here a challenging case of γδ T-cell neoplasm composed of γδ mature T-cells and γδ precursor T-cells with marked eosinophilia that is inapplicable to the current 2016 World Health Organization (WHO) classification. A 3-year-old female child who was presented with fever and marked leukocytosis. Peripheral blood smear showed marked lymphocytosis, marked eosinophilia, neutrophilia, monocytosis, and 5% circulating blasts. CT scan showed anterior mediastinal mass, lymphadenopathy, and hepatosplenomegaly. The patient underwent a bone marrow examination and a biopsy taken from the mediastinal mass. Peripheral blood and bone marrow findings were consistent with a γδ T-cell neoplasm with increased blasts and eosinophilia. The patient was sequentially treated with imatinib (tyrosine kinase inhibitor), acute lymphoblastic leukemia protocol (BFM 2009) then shifted to lymphoma protocol (LMP 96). In conclusion, we report a unique rare case of γδ T-cell neoplasm with a combination of mature and immature γδ T-cells and eosinophilia that is inapplicable to the current 2016 WHO classifications. This case raises a challenging concept of a mature T-cell lymphoma arising in an immature T-cell neoplasm. It also highlights the need to target all neoplastic components to eradicate the disease.
γδ T细胞淋巴瘤是非常罕见且侵袭性强的肿瘤。我们在此描述一例具有挑战性的γδ T细胞肿瘤病例，其由γδ成熟T细胞和γδ前体T细胞组成，并伴有明显嗜酸性粒细胞增多，这一病例并不适用于当前的2016年世界卫生组织（WHO）分类。一名3岁女童，出现发热和明显的白细胞增多。外周血涂片显示明显的淋巴细胞增多、明显的嗜酸性粒细胞增多、中性粒细胞增多、单核细胞增多以及5%的循环原始细胞。CT扫描显示前纵隔肿块、淋巴结病和肝脾肿大。该患者接受了骨髓检查以及从纵隔肿块处获取的活检。外周血和骨髓检查结果与伴有原始细胞增多和嗜酸性粒细胞增多的γδ T细胞肿瘤一致。该患者先后接受了伊马替尼（酪氨酸激酶抑制剂）治疗、急性淋巴细胞白血病方案（BFM 2009），随后转向淋巴瘤方案（LMP 96）。总之，我们报告了一例独特的罕见γδ T细胞肿瘤病例，其兼具成熟和未成熟γδ T细胞以及嗜酸性粒细胞增多，并不适用于当前的2016年WHO分类。该病例提出了一个在未成熟T细胞肿瘤中出现成熟T细胞淋巴瘤的具有挑战性的概念。它还强调了针对所有肿瘤成分以根除疾病的必要性。
