Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder of uncontrolled immune activation which is usually divided into two main types. Primary, which is associated with genetic mutation and familial predisposition and secondary, which is usually associated with infections, malignancies and autoimmune conditions. More often multiple risk factors are present at the time of initial presentation. We report a case where HLH was the presenting manifestation of relapsed Classic Hodgkin's Lymphoma in the presence of multiple risk factors of secondary HLH such as human immunodeficiency virus (HIV), active genital herpes, Epstein-Barr virus (EBV) viremia, Mycobacterium avium complex (MAC) infection and prior chemotherapy. A 38-year-old male to female transgender woman presented with one-week history of fever, nausea, vomiting and generalized weakness. The past medical history was significant for HIV and previously treated and positron emission tomography (PET) scan confirmed complete remission of Classic Hodgkin's Lymphoma. Physical examination showed BP 92/40 mmHg, fever of 102.6 F, heart rate of 114 beats per minutes, diffuse abdominal tenderness and male genitalia with multiple small ulcerative lesions. Labs showed pancytopenia, hyponatremia, mildly elevated total and direct bilirubin, transaminitis, CD-4 count 96/mcL, HIV viral load undetectable and COVID-19 polymerase chain reaction (PCR) negative. Imaging showed right middle lung lobe consolidation and hepatosplenomegaly with multiple hypodense lesions. Lymphadenopathy was reported in mediastinum and retroperitoneum. The patient was initially treated with broad spectrum antibiotics, IV fluids, vasopressors and stress dose steroids. After initial improvement, vasopressors and steroids were stopped. The patient again started spiking fever on day 9 despite being on antibiotics. Workup showed EBV viremia, genital herpes and evidence of MAC infection on sputum culture. No improvement noted despite appropriate treatment for genital herpes and MAC. Additional lab work showed hyperferritinemia and elevated soluble Interleukin-2 receptors. The patient was diagnosed with HLH as per HLH-2004 criteria and treated with dexamethasone and etoposide. Bone marrow biopsy confirmed hemophagocytosis and immunoperoxidase staining established the diagnosis of relapsed Classic Hodgkin's Lymphoma. We can conclude that in patients with a history of hematological malignancy presenting with HLH, a high degree of suspicion for relapse should be maintained even in the presence of other risk factors.