Dhaliwal Armaan, Eller Vanessa F, Pu Jeffrey J
Department of Medicine, University of Arizona College of Medicine, Tucson, AZ, USA.
University of Arizona NCI Designated Comprehensive Cancer Center, Tucson, AZ, USA.
J Med Cases. 2022 Sep;13(9):427-431. doi: 10.14740/jmc3973. Epub 2022 Sep 28.
Hodgkin lymphoma (HL) is a hematologic malignancy that comprises about 10% of all lymphomas with the most common type being classical HL (cHL). The typical clinical presentation of cHL involves multiple region lymphadenopathy and a chest mass found on imaging. However, not all patients present with the typical symptomology of cHL which poses a diagnostic challenge. Extranodal HL, especially primary bone marrow HL (PBMHL), has been described in immunocompromised patients with human immunodeficiency virus (HIV). In this case report, we present a PBMHL case in an immunocompetent patient with no HIV exposure. We discuss a 51-year-old immunocompetent female who presented with 2 - 3 months of fever, confusion, generalized myalgias, and fatigue. She had no lymphadenopathy on physical exam. On further testing, the patient's blood work demonstrated cytopenia and imaging confirmed no lymphadenopathy. Eventually, a bone marrow evaluation established her diagnosis of PBMHL. The patient expired after receiving one cycle of a modified chemotherapy regimen. This case illustrates that HL can be associated with an atypical clinical presentation which may delay diagnosis and treatment. PBMHL can occur in the normal population who is not immunocompromised nor HIV positive. In this situation, the best diagnostic approach is a thorough medical history, physical exam, and bone marrow aspiration and biopsy. Presence of constitutional symptoms without any lymphadenopathy or chest mass should raise the concern for possible atypical HL such as PBMHL. Accurate and timely identification of PBMHL allows for timely initiation of appropriate therapy. While cHL is responsive to chemotherapy, further research is required to improve the therapy for PBMHL.
霍奇金淋巴瘤(HL)是一种血液系统恶性肿瘤,约占所有淋巴瘤的10%,最常见的类型是经典型HL(cHL)。cHL的典型临床表现包括多区域淋巴结肿大以及影像学检查发现的胸部肿块。然而,并非所有患者都表现出cHL的典型症状,这给诊断带来了挑战。在免疫功能低下的人类免疫缺陷病毒(HIV)患者中已描述了结外HL,尤其是原发性骨髓HL(PBMHL)。在本病例报告中,我们呈现了一例无HIV暴露史的免疫功能正常患者的PBMHL病例。我们讨论了一名51岁免疫功能正常的女性,她出现了2至3个月的发热、意识模糊、全身肌痛和疲劳症状。体格检查未发现淋巴结肿大。进一步检查发现,患者的血液检查显示血细胞减少,影像学检查证实无淋巴结肿大。最终,骨髓评估确诊她为PBMHL。该患者在接受一个周期的改良化疗方案后死亡。本病例表明,HL可能与非典型临床表现相关,这可能会延迟诊断和治疗。PBMHL可发生在既无免疫功能低下也无HIV阳性的正常人群中。在这种情况下,最佳的诊断方法是详细的病史、体格检查以及骨髓穿刺和活检。出现全身症状但无任何淋巴结肿大或胸部肿块时,应考虑可能存在非典型HL,如PBMHL。准确及时地识别PBMHL能够及时启动适当的治疗。虽然cHL对化疗有反应,但仍需要进一步研究以改善PBMHL的治疗。