INTRAVASCULAR LYMPHOMATOSIS - A CLINICOPATHOLOGICAL STUDY OF 10 CASES AND ASSESSMENT OF RESPONSE TO CHEMOTHERAPY
INTRAVASCULAR LYMPHOMATOSIS - A CLINICOPATHOLOGICAL STUDY OF 10 CASES AND ASSESSMENT OF RESPONSE TO CHEMOTHERAPY
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DOI：
10.1200/jco.1994.12.12.2573
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发表时间：
1994-12-01
影响因子：
45.3
通讯作者：
MANN, RB
中科院分区：
文献类型：
--
作者：
DIGIUSEPPE, JA;NELSON, WG;MANN, RB
目的：报告10例血管内淋巴瘤病(IVL)的临床病理研究，并对这些患者的化疗反应和文献复习中的病例进行评估。每个病例的随访信息都是通过咨询治疗医生获得的。结果：本组10例患者中，最常见的临床表现为不明原因发热、精神状态改变和皮疹。诊断标本来自各种来源，包括大脑、皮肤、前列腺、肝脏、肾脏和胆汁。接受联合化疗的4例患者全部存活，其中2例获得长期生存(分别为48个月和45个月)；其余2例存活，经6个月的短期随访后完全缓解(CR)。在文献中报道的35名接受化疗的患者中(包括本系列的4名)，43%的患者获得了CR，并且在发表时没有疾病。在我们的系列中，接受局部治疗(手术加或不加放射治疗)的三名患者无一存活(平均生存时间为9个月)。3例尸检确诊的患者，发病至死亡的平均间隔时间为3个月，且疾病广泛。结论：IVL是一种高度恶性的非霍奇金淋巴瘤(NHL)，有全身扩散的倾向，CR和长期生存可能导致患者接受积极的联合化疗。(C)1994年，由美国临床肿瘤学会主办。
Purpose: We report a clinicopathologic study of 10 cases of intravascular lymphomatosis (IVL) seen at a single institution, and assess the response to chemotherapy in these patients, as well as those collected from a literature review.Patients and Methods: The clinical, pathologic, and immunophenotypic features of 10 cases of IVL diagnosed at the Johns Hopkins Hospital since 1977 were studied. Follow-up information was obtained in each case by consultation with the treating physician. In addition, cases of IVL reported previously in which patients were treated with chemotherapy and for which follow-up data were available at the time of publication were reviewed.Results: In the present series of 10 cases, the most common clinical features were fever of unknown origin (FUO), mental status changes, and rash. Diagnostic specimens were obtained from a variety of sources, including brain, skin, prostate, liver, kidney, and gall bladder. All of the four patients treated with combination chemotherapy are alive and two have achieved long-term survival (48 and 45 months, respectively); the remaining two are alive and in complete remission (CR) after short follow-up duration of 6 months. Among 35 patients reported in the literature who received chemotherapy (including four from this series), 43% attained a CR and were free of disease at the time of publication. None of the three patients in our series who received localized therapy (surgery with or without radiation therapy) is alive (mean survival duration, 9 months). For the three patients diagnosed at postmortem examination, the mean interval between onset of symptoms and death was 3 months, and disease was widespread.Conclusion: These findings suggest that IVL represents a high-grade non-Hodgkin's lymphoma (NHL) with a propensity for systemic dissemination, and that CR and long-term survival may result in patients treated with aggressive combination chemotherapy. (C) 1994 by American Society of Clinical Oncology.