Abstract：
BACKGROUND: Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of peripheral T-cell lymphoma. Patients usually present with splenomegaly and pancytopenia but without lymphadenopathy. Immunohistochemistry (IHC) staining of bone marrow biopsy shows intra-sinusoidal infiltration of CD3 and CD56 T-lymphocytes. Current treatment strategy of HSTCL includes a CHOP regimen (cyclophosphamide, adriamycine, vincristine, prednisone) followed by autologous transplantation.
METHODS: A 28-year-old male presented with abdominal fullness, weight loss, and massive splenomegaly. Laboratory findings revealed pancytopenia. A CT scan of the abdomen displayed hepatomegaly and massive splenomegaly. The bone marrow pathology examination showed monotonous medium-sized lymphocytes with some cluster of atypical lymphocytes with loosely condensed chromatin and pale cytoplasm. The intra-sinusoidal location was more prominent after using IHC staining of CD3 and CD56, which are characteristics of HSTCL. We administered CHOP-based regiment every 3 weeks for 3 cycles; however, the response was a stable disease. Since the splenomegaly was still massive and compromised the patient, the multidisciplinary team decided to perform splenectomy. Unfortunately, the patient did not survive the surgery.
CONCLUSIONS: Hepatosplenic T-cell lymphoma is a rare aggressive disease, which is part of peripheral T-cell lymphoma. CHOP-based chemotherapy appeared to be ineffective, and we need further studies to find the optimal treatment of HSTCL.
METHODS: A 28-year-old male presented with abdominal fullness, weight loss, and massive splenomegaly. Laboratory findings revealed pancytopenia. A CT scan of the abdomen displayed hepatomegaly and massive splenomegaly. The bone marrow pathology examination showed monotonous medium-sized lymphocytes with some cluster of atypical lymphocytes with loosely condensed chromatin and pale cytoplasm. The intra-sinusoidal location was more prominent after using IHC staining of CD3 and CD56, which are characteristics of HSTCL. We administered CHOP-based regiment every 3 weeks for 3 cycles; however, the response was a stable disease. Since the splenomegaly was still massive and compromised the patient, the multidisciplinary team decided to perform splenectomy. Unfortunately, the patient did not survive the surgery.
CONCLUSIONS: Hepatosplenic T-cell lymphoma is a rare aggressive disease, which is part of peripheral T-cell lymphoma. CHOP-based chemotherapy appeared to be ineffective, and we need further studies to find the optimal treatment of HSTCL.
摘要：
背景：肝脾T细胞淋巴瘤（HSTCL）是一种罕见的外周T细胞淋巴瘤亚型。患者通常表现为脾肿大和全血细胞减少，但无淋巴结肿大。骨髓活检的免疫组织化学（IHC）染色显示CD3和CD56T淋巴细胞的窦内浸润。HSTCL的当前治疗策略包括CHOP方案（环磷酰胺，阿霉素,长春新碱,泼尼松），然后进行自体移植。
方法：一名28岁男性表现为腹胀，减肥,和巨大的脾肿大。实验室发现显示全血细胞减少症。腹部CT扫描显示肝肿大和大量脾肿大。骨髓病理检查显示为单调的中型淋巴细胞，有一些非典型淋巴细胞簇，染色质松散凝聚，细胞质苍白。使用HSTCL的特征CD3和CD56的IHC染色后，窦内位置更加突出。我们每3周进行一次基于CHOP的方案，共3个周期；然而，反应是稳定的疾病。由于脾肿大仍然很大，并且损害了患者，多学科小组决定进行脾切除术.不幸的是,患者未能在手术中幸存下来。
结论：肝脾T细胞淋巴瘤是一种罕见的侵袭性疾病，是外周T细胞淋巴瘤的一部分.基于CHOP的化疗似乎无效，我们需要进一步的研究来寻找HSTCL的最佳治疗方法。
方法：一名28岁男性表现为腹胀，减肥,和巨大的脾肿大。实验室发现显示全血细胞减少症。腹部CT扫描显示肝肿大和大量脾肿大。骨髓病理检查显示为单调的中型淋巴细胞，有一些非典型淋巴细胞簇，染色质松散凝聚，细胞质苍白。使用HSTCL的特征CD3和CD56的IHC染色后，窦内位置更加突出。我们每3周进行一次基于CHOP的方案，共3个周期；然而，反应是稳定的疾病。由于脾肿大仍然很大，并且损害了患者，多学科小组决定进行脾切除术.不幸的是,患者未能在手术中幸存下来。
结论：肝脾T细胞淋巴瘤是一种罕见的侵袭性疾病，是外周T细胞淋巴瘤的一部分.基于CHOP的化疗似乎无效，我们需要进一步的研究来寻找HSTCL的最佳治疗方法。
