Kumagawa M, Suzumiya J, Ohshima K, Kanda M, Tamura K, Kikuchi M
First Department of Pathology; School of Medicine, Fukuoka University, 7-45-1, Nanakuma, Jonan-ku, Fukuoka, 814-0180, Japan.
Leuk Lymphoma. 2001 May;41(5-6):593-605. doi: 10.3109/10428190109060350.
Primary splenic involvement in lymphoid neoplasms is rare and the clinicopathologic features of splenic lymphoma are not well described compared to nodal non-Hodgkin's lymphoma (NHL). Here we characterized splenic lymphomas in an human T lymphotropic virus type-I (HTLV-I) endemic area of Japan. To assess the pattern of splenic involvement and evaluate prognosis, we reviewed 27 specimens consisting of 26 splenectomies and one necropsy, which were classified using REAL classification. Cases were divided into primary splenic lymphoma in 11 patients and secondary in 16 patients. The incidence of primary splenic lymphoma was 0.3% (11 of approximately 4,000 malignant lymphomas). Primary splenic lymphomas included 7 diffuse large B cell lymphoma (DLBL), 2 follicular lymphomas (FL), and 1 each of splenic marginal zone lymphoma (SMZL) and anaplastic large cell lymphoma (ALCL). Secondary splenic lymphomas included 6 DLBL, 4 mantle cell lymphoma (MCL), 2 FL, 2 Hodgkin's disease (HD), 1 each of hairy cell leukemia and ALCL. Gross examination showed two patterns of splenic involvement; solid type (formation of large nodular mass, n=16) and disseminated type (multiple nodules with diffuse infiltration but no large nodular formation, n=10). The type could not be determined in one case. Most solid types were DLBL or FL, while MCL was of the disseminated type. Immunohistochemistry showed all but each 2 cases of ALCL and HD were of B lineage. Follow-up of 26 patients indicated that all but one patient with primary lymphoma were still alive (range, 1-89 months) and 8 of 15 patients with secondary lymphomas died due to the progression of malignant lymphoma; the survival rate at 2 years was 50% in these patients. No elevation of anti-HTLV-I antibody was found. In situ hybridization for Epstein-Barr virus (EBV) showed no reactivity of lymphoma cells, although a few small lymphocytes were positive for EBV. Hepatitis C virus was observed in 6 of 20 (30%) patients examined and 4 of 11 (36%) cases of primary splenic lymphoma. Our findings indicate that patients with primary splenic lymphoma have a favorable prognosis after splenectomy.
脾脏在淋巴瘤中受累较为罕见，与淋巴结非霍奇金淋巴瘤（NHL）相比，脾淋巴瘤的临床病理特征尚未得到充分描述。在此，我们对日本人类嗜T淋巴细胞病毒I型（HTLV-I）流行地区的脾淋巴瘤进行了特征分析。为评估脾脏受累模式并评估预后，我们回顾了27份标本，包括26例脾切除术标本和1例尸检标本，这些标本采用REAL分类法进行分类。病例分为原发性脾淋巴瘤11例和继发性脾淋巴瘤16例。原发性脾淋巴瘤的发病率为0.3%（约4000例恶性淋巴瘤中的11例）。原发性脾淋巴瘤包括7例弥漫性大B细胞淋巴瘤（DLBL）、2例滤泡性淋巴瘤（FL）、1例脾边缘区淋巴瘤（SMZL）和1例间变性大细胞淋巴瘤（ALCL）。继发性脾淋巴瘤包括6例DLBL、4例套细胞淋巴瘤（MCL）、2例FL、2例霍奇金病（HD）、1例毛细胞白血病和1例ALCL。大体检查显示脾脏受累有两种模式；实体型（形成大的结节状肿块，n = 16）和弥漫型（多个结节伴弥漫性浸润但无大的结节形成，n = 10）。1例病例的类型无法确定。大多数实体型为DLBL或FL，而MCL为弥漫型。免疫组化显示，除2例ALCL和HD外，所有病例均为B细胞系。对26例患者的随访表明，除1例原发性淋巴瘤患者外，所有患者均存活（范围为1 - 89个月），15例继发性淋巴瘤患者中有8例因恶性淋巴瘤进展死亡；这些患者的2年生存率为50%。未发现抗HTLV-I抗体升高。爱泼斯坦-巴尔病毒（EBV）原位杂交显示淋巴瘤细胞无反应性，尽管少数小淋巴细胞EBV呈阳性。在20例接受检查的患者中有6例（30%）以及11例原发性脾淋巴瘤病例中有4例（36%）检测到丙型肝炎病毒。我们的研究结果表明，原发性脾淋巴瘤患者脾切除术后预后良好。
