Immunocompetent Patient With Primary Bone Marrow Hodgkin Lymphoma.

Journal of Medical Cases Pub Date : 2022-09-01 Epub Date: 2022-09-28 DOI:10.14740/jmc3973
Armaan Dhaliwal, Vanessa F Eller, Jeffrey J Pu
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Abstract

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.

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免疫功能正常的原发性骨髓霍奇金淋巴瘤患者。
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HL (cHL)。cHL的典型临床表现包括多区域淋巴结病变和影像学上发现的胸部肿块。然而,并不是所有的患者都有典型的cHL症状,这给诊断带来了挑战。结外HL,尤其是原发性骨髓HL (PBMHL),已被描述为人类免疫缺陷病毒(HIV)免疫功能低下患者。在这个病例报告中,我们提出了一个PBMHL病例在免疫功能正常的病人没有艾滋病毒暴露。我们讨论了一位51岁的免疫功能正常的女性,她表现为2 - 3个月的发烧,精神错乱,全身肌痛和疲劳。体格检查未见淋巴结病变。在进一步的检查中,患者的血液检查显示细胞减少,影像学证实无淋巴结病。最终,骨髓评估确定了她的PBMHL诊断。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可能伴有不典型的临床表现,这可能会延误诊断和治疗。PBMHL可发生在没有免疫功能低下或HIV阳性的正常人群中。在这种情况下,最好的诊断方法是全面的病史,体检,骨髓穿刺和活检。没有任何淋巴结病变或胸部肿块的体质症状应引起对非典型HL(如PBMHL)的关注。准确和及时地识别PBMHL可以及时开始适当的治疗。虽然cHL对化疗有反应,但需要进一步研究以改进PBMHL的治疗方法。
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