Kalac Matko, Ostojić Slobodanka, Gasparov Slavko, Planinc-Peraica Ana, Dominis Mara, Jaksić Branimir
Odjel za hematologiju, Interna klinika, Klinicka bolnica Merkur, Zagreb.
Lijec Vjesn. 2006 Mar-Apr;128(3-4):76-8.
Hepatosplenic T-cell lymphoma (HSTCL) is a rare form of extranodal non-Hodgkin lymphoma derived from cytotoxic T-cells, usually manifesting by sinusoidal infiltration of spleen, liver and bone marrow. In 1997 World Health Organization classified malignant lymphomas and placed HSTCL among peripheral T-cell neoplasms. The course of the diseases is usually very agressive with a median survival time of 8 to 16 moths despite multiagent chemotherapy. We present a case of a 48-year-old male patient whose initial symptoms were fatigue, weight loss and night sweats, which were followed by splenomegaly and pancytopenia. After clinical examination we suspected him to have HSTCL which was proved pathohistologically upon splenectomy and it is the first case of this lymphoma diagnosed in "Merkur" Clinical Hospital. As a first line of lymphoma therapy we decided to apply FED course (fludarabine, cyclophosphamide, prednisone), being aware of the published poor results the standard CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine, prednisolone) yields. As far as we know, the results of this chemotherapy course in the therapy of this tumor have never been published. The patient underwent 6 courses of FED therapy, which he tolerated well and was in good clinical condition. Upon the completion of the 6th course of therapy he was diagnosed with lung anaplastic microcellular carcinoma and was treated with 3 course of PE therapy (cisplatin, etoposide).
肝脾T细胞淋巴瘤（HSTCL）是一种罕见的结外非霍奇金淋巴瘤，起源于细胞毒性T细胞，通常表现为脾脏、肝脏和骨髓的窦状浸润。1997年世界卫生组织对恶性淋巴瘤进行了分类，并将HSTCL归为外周T细胞肿瘤。尽管采用了多药化疗，但该病的病程通常非常凶险，中位生存时间为8至16个月。我们报告一例48岁男性患者，其最初症状为疲劳、体重减轻和盗汗，随后出现脾肿大和全血细胞减少。临床检查后，我们怀疑他患有HSTCL，脾切除术后经病理组织学证实，这是“梅尔库”临床医院诊断的首例该型淋巴瘤。作为淋巴瘤治疗的一线方案，我们决定应用FED方案（氟达拉滨、环磷酰胺、泼尼松），因为我们知道已发表的标准CHOP化疗（环磷酰胺、阿霉素、长春新碱、泼尼松龙）效果不佳。据我们所知，这种化疗方案治疗该肿瘤的结果从未发表过。该患者接受了6个疗程的FED治疗，耐受性良好，临床状况良好。在完成第6个疗程的治疗后，他被诊断为肺间变性微细胞癌，并接受了3个疗程的PE治疗（顺铂、依托泊苷）。
