Unraveling the Mystery of Gray Zone Lymphoma in Human Immunodeficiency Virus-Seropositive Patients: Two Cases

Abstract

Gray zone lymphoma (GZL) is an uncommon neoplasm with intermediate features of both classic Hodgkin lymphoma (cHL) and diffuse large B-cell lymphoma (DLBCL). It was identified in the World Health Organization (WHO) classification as its own neoplasm in 2008. Patients infected with human immunodeficiency virus (HIV) have been rarely diagnosed with this type of lymphoma and treatment strategies for this subset of patients is not well described. Here we present two cases of patients with HIV that were diagnosed with GZL in a single community-based institution. A 68-year-old male with HIV/acquired immunodeficiency syndrome (AIDS) on highly active antiretroviral therapy (HAART) presented with 6-month history of dyspnea and weight loss. Computed tomography (CT) of the chest revealed multiple lung and mediastinal lesions, the largest measuring 9.4 5.5 cm lesion in the right perihilar region. Lymph node biopsy revealed abnormal lymphocytes with immunohistochemistry (IHC) positive for cluster of differentiation 30 (CD30), CD20 and Epstein-Barr virus (EBV), consistent with a diagnosis of GZL. The patient received dose-adjusted etoposide, doxorubicin, vincristine, cyclophosphamide, prednisone, and rituximab (DA-EPOCH-R) and attained a complete response. He since completed maintenance rituximab therapy and remains disease-free at 33 months. A 40-year-old female with HIV/AIDS on HAART presented with high-grade fever, dyspnea, and weight loss. CT imaging revealed multiple lung lesions, hepatosplenomegaly and diffuse lymphadenopathy in the chest and abdomen. Lymph node and bone marrow biopsy revealed cells positive for CD20, CD30, and EBV within atypical lymphoid cells. With this, a diagnosis of GZL was made and she was treated with DA-EPOCH-R. She attained a complete response and was on maintenance rituximab therapy. At 9 months she relapsed, she has now received a bone marrow transplant. GZL is a rarely described neoplasm within the HIV population. Here we describe two HIV patients diagnosed with GZL that were successfully treated at our institution. DA-EPOCH-R was able to induce durable remission with limited side effects and it represents a viable strategy for treating patients in this population. Further studies need to be performed to better characterize this lymphoma, especially in HIV patients. Treatment strategies for this select group of patients also need to be better defined.

J Hematol. 2020;9(4):132-136
doi: https://doi.org/10.14740/jh554