Hara T, Tsurumi H, Yoshimura K, Takenaka K, Goto H, Tajika M, Sawada M, Murakami N, Moriwaki H
First Department of Internal Medicine, Gifu University School of Medicine.
Rinsho Ketsueki. 2000 Jan;41(1):48-53.
We report 2 cases of orbital non-Hodgkin's lymphoma (NHL) with hemophagocytic syndrome (HPS). Patient 1 was a 64-year-old man with a diagnosis of peripheral T-cell lymphoma originating in the right orbita (clinical stage: IV B). Epstein-Barr virus DNA was demonstrated in tissue specimens by polymerase chain reaction. Laboratory findings on admission were WBC: 4,700/microliter, Hb: 12.1 g/dl, Plt: 14.6 x 10(4)/microliter, LDH: 951 IU/l, sIL-2R: 2,553 IU/ml, and ferritin: 5998.1 ng/ml. Patient 2 was a 73-year-old man with a diagnosis of diffuse large B-cell lymphoma originating in the right orbita (Clinical stage: IV B). Laboratory findings on admission were WBC: 9,100/microliter, Hb: 7.7 g/dl, Plt: 15.4 x 10(4)/microliter, LDH: 1,043 IU/l, sIL-2R: 10,090 IU/ml, and ferritin: 2079.3 ng/ml. Both patients had high-grade fever and extremely high serum cytokine levels. Bone marrow aspiration disclosed many histiocytes with hemophagocytosis. In both cases, combined chemotherapy was transiently effective, but patient 1 died of relapse of HPS and patient 2 of cerebral bleeding. Orbital non-Hodgkin's lymphoma with HPS is rare. These cases were interesting in terms of the relationship between HPS and the primary site of lymphoma.
我们报告2例伴有噬血细胞综合征（HPS）的眼眶非霍奇金淋巴瘤（NHL）。病例1为一名64岁男性，诊断为起源于右眼眶的外周T细胞淋巴瘤（临床分期：IV B期）。通过聚合酶链反应在组织标本中检测到爱泼斯坦-巴尔病毒DNA。入院时实验室检查结果为：白细胞计数（WBC）：4700/微升，血红蛋白（Hb）：12.1克/分升，血小板计数（Plt）：14.6×10⁴/微升，乳酸脱氢酶（LDH）：951国际单位/升，可溶性白细胞介素-2受体（sIL-2R）：2553国际单位/毫升，铁蛋白：5998.1纳克/毫升。病例2为一名73岁男性，诊断为起源于右眼眶的弥漫性大B细胞淋巴瘤（临床分期：IV B期）。入院时实验室检查结果为：WBC：9100/微升，Hb：7.7克/分升，Plt：15.4×10⁴/微升，LDH：1043国际单位/升，sIL-2R：10090国际单位/毫升，铁蛋白：2079.3纳克/毫升。两名患者均有高热和极高的血清细胞因子水平。骨髓穿刺发现许多具有噬血细胞现象的组织细胞。在这两个病例中，联合化疗有短暂疗效，但病例1死于HPS复发，病例2死于脑出血。伴有HPS的眼眶非霍奇金淋巴瘤很罕见。就HPS与淋巴瘤原发部位之间的关系而言，这些病例很有意思。
