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T-CELL LARGE GRANULAR LYMPHOCYTIC LEUKEMIA – CASE REPORT

机译:T细胞大颗粒性淋巴细胞白血病–病例报告

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摘要

SUMMARY – T-cell large granular lymphocytic leukemia (T-LGLL) is an uncommon but probably underdiagnosed disease caused by clonal proliferation of large granular lymphocytes. Diagnosis is typically based on the high number of morphologically characteristic lymphoid cells and finding of an abnormal immunophenotype by flow cytometry. Because of its relatively indolent clinical behavior, observation is often an appropriate therapy. Here we present a case of a 53-year-old male admitted to the hospital because of abdominal pain. Blood examination revealed mild mycrocitic anemia and multiplied lactate dehydrogenase level. Abdominal ultrasound showed splenomegaly of 16 cm, with no lymphadenopathy. Fine needle aspiration of bone marrow revealed hypocellular marrow with 50% of atypical lymphoid cells. There were 81% of atypical medium sized granular lymphocytes with irregularly shaped nuclei in peripheral blood, so the cytologic diagnosis was lymphoproliferative process. Bone marrow biopsy showed nodular and interstitial proliferation of small, partially atypical T lymphocytic cells positive for CD2, CD3, CD5, CD8, granzyme and TIA, and negative for hairy cell markers, CD10, MUM 1, bcl 1, CD4 and CD56. The finding was consistent with T-LGLL. Due to splenomegaly, the patient was treated with cyclosporine and gradually reduced dose of corticosteroids, leading to regression of splenomegaly and normalization of lactate dehydrogenase level.
机译:总结– T细胞大颗粒淋巴细胞性白血病(T-LGLL)是一种罕见的疾病,但可能是由大颗粒淋巴细胞的克隆性增殖引起的未被诊断的疾病。诊断通常基于大量的形态特征性淋巴样细胞和通过流式细胞术发现异常的免疫表型。由于其相对柔和的临床行为,观察通常是一种适当的疗法。在这里,我们介绍了一例因腹痛入院的53岁男性。血液检查发现轻度霉菌性贫血和乳酸脱氢酶水平成倍增加。腹部超声显示脾肿大为16 cm,无淋巴结肿大。骨髓的细针穿刺检查发现骨髓下丘细胞中含有50%的非典型淋巴样细胞。外周血中81%的非典型中型粒状淋巴细胞核形状不规则,因此细胞学诊断为淋巴增生过程。骨髓活检显示小而部分非典型的T淋巴细胞的结节和间质增生,对CD2,CD3,CD5,CD8,粒酶和TIA呈阳性,而对毛细胞标记CD10,MUM 1,bcl 1,CD4和CD56呈阴性。该发现与T-LGLL一致。由于脾肿大,患者接受了环孢霉素治疗并逐渐减少了皮质类固醇的剂量,导致脾肿大消退和乳酸脱氢酶水平正常化。

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