Ponzoni Maurilio, Fumagalli Luca, Rossi Giuseppe, Freschi Massimo, Re Alessandro, Viganò Maria Grazia, Guidoboni Massimo, Dolcetti Riccardo, McKenna Robert W, Facchetti Fabio
Department of Pathology, S. Raffaele H Scientific Institute, Milan, Italy.
Mod Pathol. 2002 Dec;15(12):1273-8. doi: 10.1097/01.MP.0000037311.56159.13.
Human immunodeficiency virus-associated Hodgkin lymphoma frequently involves the bone marrow and is usually recognized at staging after Hodgkin lymphoma diagnosis on a lymph node or other tissue biopsies, but occasionally the marrow involvement is the only apparent manifestation of disease. In the latter setting, diagnosis can be problematic. From a total of 42 patients with newly diagnosed human immunodeficiency virus-associated Hodgkin lymphoma, 22 subjects had positive marrow involvement at diagnosis; 16 of them had additional substantial histological and/or clinical extramedullary Hodgkin lymphoma. In the remaining 6 patients the bone marrow was the only site of disease at diagnosis. In all six cases, bone marrow biopsy revealed obvious lymphomatous involvement. Reed-Sternberg cells were identified both morphologically and immunophenotypically in all cases. Spared marrow tissue consistently showed fibrosis. All patients were males with a median age of 35 years (range, 31-58 years). All presented with fever, blood cytopenias, and severe CD4+ lymphocyte depletion (median, 70 cells/mm(3)). After diagnosis, all staging procedures were negative, and all patients were treated with chemotherapy. Median survival was 4 months (range, 2-118 mo). Longer survival was achieved in the patients who completed chemotherapy regimens; three subjects, however, died shortly before the full completion of chemotherapy, two of them from Hodgkin lymphoma. Isolated bone marrow HIV-associated Hodgkin lymphoma may be an underestimated condition in HIV-infected patients; in those individuals with unexplained fever and blood cytopenias, bone marrow biopsy should be performed with the aim of assessing for Hodgkin lymphoma, even in the absence of nodal and visceral lymphomatous involvement. A rapid diagnosis of isolated bone marrow HIV-associated Hodgkin lymphoma could expedite therapy.
人类免疫缺陷病毒相关性霍奇金淋巴瘤常累及骨髓,通常在霍奇金淋巴瘤经淋巴结或其他组织活检确诊后的分期检查中被发现,但偶尔骨髓受累是疾病的唯一明显表现。在后一种情况下,诊断可能存在问题。在总共42例新诊断的人类免疫缺陷病毒相关性霍奇金淋巴瘤患者中,22例在诊断时骨髓受累为阳性;其中16例还有其他明显的组织学和/或临床髓外霍奇金淋巴瘤。其余6例患者在诊断时骨髓是疾病的唯一部位。在所有6例病例中,骨髓活检均显示明显的淋巴瘤累及。所有病例均在形态学和免疫表型上鉴定出里德-施特恩伯格细胞。剩余的骨髓组织持续显示纤维化。所有患者均为男性,中位年龄35岁(范围31 - 58岁)。所有患者均有发热、血细胞减少和严重的CD4 +淋巴细胞减少(中位值70个细胞/mm³)。诊断后,所有分期检查均为阴性,所有患者均接受化疗。中位生存期为4个月(范围2 - 118个月)。完成化疗方案的患者生存期较长;然而,有3例患者在化疗即将结束前不久死亡,其中2例死于霍奇金淋巴瘤。孤立性骨髓人类免疫缺陷病毒相关性霍奇金淋巴瘤在感染人类免疫缺陷病毒的患者中可能是一种被低估的疾病;对于那些有不明原因发热和血细胞减少的个体,即使没有淋巴结和内脏淋巴瘤累及,也应进行骨髓活检以评估是否患有霍奇金淋巴瘤。快速诊断孤立性骨髓人类免疫缺陷病毒相关性霍奇金淋巴瘤可加快治疗。