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恶性淋巴瘤及相关疾病的原发性脾脏表现。49例研究。

Primary splenic presentation of malignant lymphoma and related disorders. A study of 49 cases.

作者信息

Kraemer B B, Osborne B M, Butler J J

出版信息

Cancer. 1984 Oct 15;54(8):1606-19. doi: 10.1002/1097-0142(19841015)54:8<1606::aid-cncr2820540823>3.0.co;2-5.

DOI:10.1002/1097-0142(19841015)54:8<1606::aid-cncr2820540823>3.0.co;2-5
PMID:6548171
Abstract

The diagnosis of malignant lymphoma presenting as an initial splenic manifestation may go unrecognized as such when peripheral lymph nodes are not enlarged and when results of bone marrow biopsies are negative. Tissues from 49 patients, ranging in age from 15 to 78 years, in whom the original diagnosis of malignant lymphoma and related conditions was made at splenectomy, were classified as: diffuse small lymphocytic (20), diffuse large cell (11), diffuse small cleaved (5), diffuse large cell, immunoblastic (5), follicular small cleaved cell (3), and follicular mixed small cell and large cell (2). Two additional spleens, diagnosed as acute blastic leukemia, were initially confused with malignant non-Hodgkin's lymphoma by light microscopy. One patient presented with Hodgkin's disease confined to the spleen. For the non-Hodgkin's lymphoma group, parameters of age, sex, splenic weight (range, 226-4000 g), lymph node, bone marrow, or liver involvement did not adversely influence prognosis. Abdominal lymph nodes were positive in 31 of 37 patients having splenic hilar and/or abdominal lymph nodes available for review. Of 29 patients with adequate follow-up, 7 died of disease, 5 were free of disease at 3 years, 2 were free of disease at 5 years, 2 were alive with disease at 3 years, 4 were alive with disease at 5 years, and 9 died from second malignancies, unknown, or unrelated causes. Six of the 7 patients who died of lymphoma were classified as large cell (four diffuse large cell and two diffuse large cell, immunoblastic), with a mean 2-year survival. One patient died of leukemia. Those lymphomas classified as low grade behaved in an indolent fashion. The morphologic diversity of these cases emphasizes the need for the initial recognition and correct classification of lymphomas which present in the spleen, since survival is best determined according to histologic type.

摘要

当外周淋巴结未肿大且骨髓活检结果为阴性时,以脾脏为首发表现的恶性淋巴瘤可能无法得到正确诊断。对49例年龄在15至78岁之间、最初在脾切除时被诊断为恶性淋巴瘤及相关疾病的患者的组织进行分类,结果如下:弥漫性小淋巴细胞型(20例)、弥漫性大细胞型(11例)、弥漫性小裂细胞型(5例)、弥漫性大细胞免疫母细胞型(5例)、滤泡性小裂细胞型(3例)以及滤泡性小细胞和大细胞混合型(2例)。另外两个脾脏最初被诊断为急性原始细胞白血病,通过光学显微镜检查最初被误诊为恶性非霍奇金淋巴瘤。1例患者表现为局限于脾脏的霍奇金病。对于非霍奇金淋巴瘤组,年龄、性别、脾脏重量(范围为226至4000克)、淋巴结、骨髓或肝脏受累情况等参数对预后没有不利影响。在37例有脾门和/或腹部淋巴结可供检查的患者中,31例的腹部淋巴结呈阳性。在29例有充分随访的患者中,7例死于疾病,5例在3年时无病生存,2例在5年时无病生存,2例在3年时带瘤生存,4例在5年时带瘤生存,9例死于第二原发恶性肿瘤、不明原因或无关原因。7例死于淋巴瘤的患者中有6例被分类为大细胞型(4例弥漫性大细胞型和2例弥漫性大细胞免疫母细胞型),平均生存2年。1例死于白血病。那些被分类为低级别淋巴瘤的病情发展较为缓慢。这些病例的形态学多样性强调了对脾脏中出现的淋巴瘤进行早期识别和正确分类的必要性,因为生存情况最好根据组织学类型来确定。

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