Hemophagocytic lymphohistiocytosis: a rare disease unveiling the diagnosis of EBV-related large B cell lymphoma in a patient with HIV

Abstract

Background Hemophagocytic lymphohistiocytosis (HLH) is a rare disease resulting from the overactivation of the immune system due to under regulation of cytotoxic lymphocytes, macrophages and natural killer (NK) cells. HLH is associated with malignancies, infections, autoimmune disorders and rarely AIDS and is rapidly fatal. Case presentation This case report identified a 53 year old male with acquired immunodeficiency syndrome (AIDS) who presented with neutropenic fever of unknown origin. He had two previous hospitalizations prior to the hospitalization diagnosing HLH. The first led to a diagnosis of drug fevers in the setting of treatment for thrombotic thrombocytopenic purpura and subsequent hospitalization led to empiric treatment of hospital acquired pneumonia after workup for intermittent fevers was negative. He was discharged but readmitted 10 days after for recurrence of neutropenic fevers. During this final hospitalization, he was found to have elevated liver enzymes, ferritin, triglycerides and soluble IL-2 receptor with persistent fevers, new splenomegaly and bicytopenia meeting the 2004 HLH criteria. Bone marrow biopsy confirmed the diagnosis of HLH as well as EBV associated large B-cell lymphoma. The patient improved on treatment with steroids, rituximab, tocilizumab, and chemotherapy but ultimately passed away due to refractory septic shock from multi-drug resistant Klebsiella pneumoniae. Conclusion This novel case highlights a patient diagnosed with HLH in the setting of several risk factors for the disease, including AIDS, B-cell lymphoma and EBV. Additionally, this case highlights the importance of early consideration of HLH in the setting of neutropenic fever without clear infectious etiology and search for malignancy associated reasons for HLH especially in immunocompromised patients.

Primary Bone Marrow HIV-Associated Hodgkin Lymphoma Complicated by Hemophagocytic Lymphohistiocytosis

“…Our case demonstrates the importance of the early consideration of HLH and possible underlying HL in a cytopenic patient with a fever of unknown origin in the setting of HIV. The early detection of HLH can lead to a quick treatment, but also a thorough work-up for secondary causes [ 15 ]. Furthermore, although the role of a bone marrow biopsy has been challenged in the work-up of HL in favor of imaging, such as PET-CT, our case demonstrates that it may still have a role in the work-up of HIV patients for primary bone marrow HL [ 3 , 4 ].…”

Diagnosis and Management of Adult Malignancy-Associated Hemophagocytic Lymphohistiocytosis

“…The use of anti-IL6 therapy for adult HLH (with tocilizumab; no published data using siltuximab) has been extrapolated from its use in cytokine release syndrome (CRS) and COVID-19. Only a few publications have reported the efficacy of tocilizumab treatment in mHLH, with a recent retrospective review suggesting that the use of tocilizumab could increase infectious complications compared to conventional therapy [ 69 , 70 ].…”