COVID-19, HISTOPLASMOSIS, AND HIV/AIDS AS A CAUSE OF SECONDARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS

TOPIC: Pulmonary Manifestations of Systemic Disease TYPE: Fellow Case Reports INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a rare, fatal syndrome of immune hyper-activation from unregulated macrophage activity resulting in cytokine storm and hemophagocytosis. Usually affects adults and is triggered by autoimmune disease, infection or malignancy. In this report we are describing COVID and histoplasmosis co-infection as a very rare cause of HLH in persons live with human immune deficiency virus (HIV). CASE PRESENTATION: A 26-year-old male with no PMHx presented to ER complaining of shortness of breath, fever and night sweats for few weeks. He reported an unintentional weight loss of 30 pounds over 3 months period. Initial vitals showed a fever of 101F˚, HR 120/m, RR 22/m O2 saturation of 92% on room air. Physical exam was unremarkable. He was found to be SARS-CoV-2 and HIV positive chest X-ray and CT chest showed diffuse bilateral micro-nodules with mediastinal lymphadenopathy. Ferritin, C-RP, D-dimer and LDH were elevated. He was admitted for further evaluation. Initially, he was started on Remdesivir, Dexamethasone and therapeutic anticoagulation for COVID-19. Further workup revealed an indetectable CD4 count with HIV viral load of 511,000 copies, Histoplasma urine Ag was positive and serum fungetill>500. Flow cytometry was negative for lymphoma. He refused bone marrow or lymph node biopsy, but he met the criteria for the diagnosis of HLH. He was started on amphotericin B, the steroids dose was increased, and highly active antiretroviral therapy. In a few days, the patient significantly improved. DISCUSSION: Secondary HLH (s-HLH) happens in adulthood secondary to infections like HIV, histoplasmosis, malignancy like lymphomas, or autoimmune disease(1). COVID-19 was reported to cause HLH syndrome in few case reports during this pandemic. This syndrome is characterized by hyperinflammation with abnormal activation of T lymphocytes and NK cells that result in activation of macrophages and phagocytosis of hematopoietic cells. Patients may present with un-remitting fevers, lymphadenopathy, splenomegaly, pancytopenia, multiorgan failure, markedly elevated ferritin and pro-inflammatory cytokines. Usually diagnosed with the HLH 2004 diagnostic criteria and H-score. Treatment of sHLH usually require treatment or removal of the offending trigger, high-dose steroids, and possibly IVIG and IL-1 blocker. Treatment targeting COVID-19, histoplasmosis, and HIV/ADIS with steroids was tried in this case with improvement in clinical condition(2). CONCLUSIONS: sHLH is a rare life-threatening disease that can be triggered by HIV/AIDS, COVID 19, and histoplasmosis. Early diagnosis and treatment are crucial to prevent end-organ damage and decrease mortality. REFERENCE #1: Soy M, Atagündüz P, Atagündüz I, Sucak GT. Hemophagocytic lymphohistiocytosis: a review inspired by the COVID-19 pandemic. Rheumatol Int. 2021;41(1):7-18. doi:10.1007/s00296-020-04636-y REFERENCE #2: Carter SJ, Tattersall RS, Ramanan AV. Macrophage activation syndrome in adults: recent advances in pathophysiology, diagnosis, and treatment. Rheumatology. 2019;58:5–17. doi: 10.1093/rheumatology/key006 DISCLOSURES: No relevant relationships by Hossam Abed, source=Web Response No relevant relationships by Iyad Farouji, source=Web Response No relevant relationships by mohammed halabiya, source=Web Response No relevant relationships by William Meng, source=Web Response No relevant relationships by Richard Miller, source=Web Response No relevant relationships by Shawn Pate, source=Web Response No relevant relationships by Rutwik Patel, source=Web Response