Isolated B-cell lymphopenia and autoimmune hemolytic anemia as a curious combination of findings at the time of advanced Hodgkin lymphoma diagnosis: a pediatric case report

Lymphopenia is associated with poor outcome in Hodgkin lymphoma (HL), but the impact of specific cytopenias is unexplored. We report a case of isolated B-cell lymphopenia with HL, EBV infection, and autoimmune hemolytic anemia (AIHA). Our patient is a 19-year-old male without any significant past medical history who presented with two weeks of epigastric abdominal pain, subjective fevers, night sweats, fatigue and a five-pound weight loss. At presentation, he had a white blood cell count of 10.3 k/uL and positive Coombs with a panagglutinin. Infectious testing was negative for HIV but positive for EBV. Peripheral lymphocyte flow cytomctry identified 2% CD19+ cells with an absolute count of 43 cells/uL. This profound B-cell lymphopenia persisted despite the EBV viral load diminishing to barely detectable levels of less than 28 copies/mL. Computed tomography (CT) scan of the chest, abdomen and pelvis identified diffuse mediastinal and abdominal lymphadenopathy, as well as hepatosplenomegaly with focal lesions in the liver and spleen. A periaortic lymph node biopsy was morphologically consistent with Classical Hodgkin Lymphoma, Mixed Cellularity subtype (CHL, MC). Diagnosed with CHL, stage IVB, he was treated with the standard combination therapy of cyclophosphamide, doxorubicin, vincristine, bleomycin, prednisone, and etoposide, and he achieved a complete remission. This case highlights the unique presentation of isolated B cell lymphopenia and autoimmune hemolytic anemia in a young patient with HL.