DiGiuseppe J A, Nelson W G, Seifter E J, Boitnott J K, Mann R B
Department of Pathology, Johns Hopkins Hospital, Baltimore, MD 21287.
J Clin Oncol. 1994 Dec;12(12):2573-9. doi: 10.1200/JCO.1994.12.12.2573.
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.
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.
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 gallbladder. 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.
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.
我们报告了在单一机构中观察到的10例血管内淋巴瘤（IVL）的临床病理研究，并评估了这些患者对化疗的反应，以及从文献综述中收集的相关患者的反应。
研究了自1977年以来在约翰霍普金斯医院诊断的10例IVL患者的临床、病理和免疫表型特征。通过与主治医生协商获取每个病例的随访信息。此外，还回顾了先前报道的接受化疗且在发表时可获得随访数据的IVL病例。
在本系列的10例病例中，最常见的临床特征是不明原因发热（FUO）、精神状态改变和皮疹。诊断标本取自多种来源，包括脑、皮肤、前列腺、肝、肾和胆囊。接受联合化疗的4例患者均存活，其中2例已实现长期生存（分别为48个月和45个月）；其余2例存活，在6个月的短期随访后处于完全缓解（CR）状态。在文献报道的接受化疗的35例患者（包括本系列中的4例）中，43%达到CR，在发表时无疾病。本系列中接受局部治疗（手术联合或不联合放疗）的3例患者均无存活（平均生存时间为9个月）。对于3例尸检确诊的患者，症状出现至死亡的平均间隔为3个月，且疾病广泛播散。
这些发现表明，IVL是一种具有全身播散倾向的高级别非霍奇金淋巴瘤（NHL），积极的联合化疗可能使患者获得CR和长期生存。
