HIV-Associated Primary Bone Marrow Hodgkin's Lymphoma: A Distinct Entity?

Primary bone marrow Hodgkin's lymphoma (PBMHL) in patients with HIV is rare with few patient case reports. We describe a patient with HIV-associated PBMHL who presented with fever and cytopenias. We reviewed the literature and present clinical characteristics of HIV-associated PBMHL. A 43-year-old African American man with history of HIV/AIDS was admitted with a recurrent fever, night sweats, and malaise of several weeks in duration. The patient denied any change in appetite or weight loss. He denied any history of smoking, alcohol abuse, or illicit drug use. His home medications were antiretroviral medication and trimethoprim-sulfamethoxazole for Pneumocystis carinii pneumonia prophylaxis. There was no family history of blood dyscrasias or malignancies. Physical examination did not reveal any lymphadenopathy or hepatosplenomegaly. Laboratory results revealed pancytopenia with WBC of 2,100/μL, hemoglobin of 9 g/dL, and platelet count of 115,000/μL. The absolute neutrophil count was 1,700/μL. An infection work-up was negative. The patient underwent bone marrow aspiration and biopsy for evaluation of cytopenias. Bone marrow biopsy showed hypercellular marrow with present but decreased hematopoiesis as a result of a dense infiltrate of large atypical cells with bilobed nuclei, prominent eosinophilic nucleoli, and abundant cytoplasm typical of Reed-Sternberg cells (Fig 1) as well as many variants. The background consisted of abundant lymphocytes, plasma cells, histiocytes, and eosinophils. Immunohistochemical stains showed that the large, atypical Reed-Sternberg cells were positive for CD15 and CD30 and negative for CD2, CD3, and CD20 antigens. Diagnosis of Hodgkin's lymphoma (HL) was made based on the characteristic morphology and immunophenotype. Staging computed tomography scans of the neck, chest, abdomen, and pelvis were negative for lymphadenopathy or hepatosplenomegaly. The patient had a normal ejection fraction on multiple-gated acquisition scan and normal pulmonary function tests. The lymphoma was determined to be stage IVB per Ann Arbor staging system, based on the findings of bone marrow involvement and “B” symptoms. A decision was made to treat the patient with doxorubicin, bleomycin, vinblastine, and dacarbazine for six to eight cycles. The patient has completed two cycles of therapy and is doing well.

HL is five to 10 times more common in patients with HIV infection. HIV-associated HL is usually widespread at presentation and has an aggressive natural history. It involves unusual extranodal sites and may spread to extranodal sites without first involving the spleen, a phenomenon almost never observed in classical HL. Most patients present with B symptoms. Bone marrow involvement is common in HIV-associated HL, and it has a negative impact on treatment tolerance and prognosis. Isolated bone marrow disease is uncommon and is known to occur in non-Hodgkin's lymphoma. HIV-associated PBMHL is rare with few patient case reports. Our literature search yielded three publications^1–3 reporting eight additional patients. Patient characteristics and clinical courses, including the current patient, are listed in Table 1. All patients with HIV-associated PBMHL were young men age 29 years to 58 years. Fever and cytopenias were the presenting symptoms in all patients. In contrast, cytopenias tend to occur less frequently in HIV-associated HL with bone marrow involvement.⁴ The viral load was reported in three patients and was undetectable in all of those patients. At the time of publication, only four of nine patients were alive. Ponzoni et al³ reported a median survival time in patients with HIV-associated PBMHL of 4 months compared with 15 months for patients with HIV-associated HL. It should be noted that the duration of onset of symptoms to the diagnosis of HIV-associated PBMHL lymphoma was 1 to 5 months in the literature. These features suggest that HIV-associated PBMHL is a clinically distinct entity with a more aggressive clinical course than HIV-associated HL. We recommend that physicians should consider PBMHL as a differential diagnosis in an HIV-positive male patient with persistent fever and cytopenias and perform bone marrow biopsy for the diagnosis. The awareness of the existence of such a rare disease may facilitate early diagnosis and therapeutic intervention.