Hemophagocytic lymphohistiocytosis in a child with human immunodeficiency virus infection – a case report

Abstract

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a rare condition and furthermore human immunodeficiency virus (HIV)-associated HLH is rarely reported in the literature. The most frequent presentation of secondary HLH is in association with infections, malignancies or drugs. In HIV-positive patients, the viral infection itself or the antiretroviral therapy (ART) could trigger HLH. Case presentation: A 14-year-old boy was admitted for persistent diarrhea, severe weight loss and chest burns. Laboratory tests revealed important neutropenia, which led to HIV infection diagnosis. ART in combination was started associated with granulocyte-colony stimulating factor. Viral copies declined after six weeks of treatment, but the cluster of differentiation 4 (CD4) T-lymphocytes and neutrophil counts remained very low. Infections and malignancies were ruled out. The bone marrow aspirate revealed hemophagocytosis which in association with fever, bicytopenia, hypofibrinogenemia and hypertriglyceridemia established HLH diagnosis. Cortisone therapy and intravenous immunoglobulins were added. Due to lack of response, HLH-2004 protocol was initiated in collaboration with pediatric hematologist. In the first six months of treatment, CD4 T-lymphocytes and neutrophil count remained low and then they showed significant increase simultaneously. During treatment, the patient developed spontaneous severe back pain. Magnetic resonance imaging showed vertebral compression, osteomalacia and a thoracic vertebral fracture. Conclusions: Having ruled out the usual associated infections and malignancies, this is a case of HIV-associated with HLH. In this case, only the ART and immunomodulatory therapy were not enough and failed to work. Systemic immunosuppression also worked only after a prolonged course which led to an unfortunate complication: a vertebral fracture.

COVID-19 infection: from stress-related cortisol levels to adrenal glands infarction

“…Another retrospective study on nine adult UK subjects (with mean age of 73 years) with SARS (due to coronavirus as cause of death was published in 2020 [81]. Post-mortem analysis showed that 33% of studied patients had an adrenal microinfarction, while thrombotic events were confirmed in 89% of cases, mostly at pulmonary level, in association with other three major HP elements including diffuse alveolar damage (which was confirmed in all the studied individuals), immune anomalies like lymphocyte depletion [mostly cluster of differentiation (CD)-positive T-cells], as well as hemophagocytes [as otherwise seen in other severe infections like human immunodeficiency virus (HIV) infection] [81,82]. If recognized, AI-related adrenal insufficiency requires intravenous Hydrocortisone, followed by lifetime oral Hydrocortisone and Fludrocortisone [83].…”

Survival of Hemophagocytic Syndrome Secondary to Fungal and Bacterial Infection in a Pediatric Patient with HIV: A Case Report

“…Primary HLH is associated with genetic mutations that alter the cytotoxic activity of NK cells and CD8 lymphocytes. These cells accumulate in organs such as the spleen and bone marrow, where they attack erythrocytes, leukocytes, and platelets [ 2 ]. Therefore, the affliction occurs in the first years of life.…”

“…Therefore, the affliction occurs in the first years of life. Conversely, secondary HLH is triggered by infections caused by the Epstein–Barr virus (EBV), cytomegalovirus (CMV), human herpesvirus-8 (HHV-8), Histoplasma capsulatum , and, less frequently, the human immunodeficiency virus (HIV), as well as malignant neoplasms (lymphomas in greater frequency), autoimmune or autoinflammatory diseases, and drugs such as antiretroviral therapy (ART) [ 1 , 2 , 3 , 4 ].…”

“…The incidence of HIV and HLH in pediatric and non-pediatric patients is rare [ 1 , 2 , 3 , 4 , 5 , 6 , 7 ]. According to reports, the most frequently isolated infectious agents triggering HLH in HIV patients are EBV, HHV-8, Histoplasma capsulatum , Mycobacterium sp., CMV, and Cryptococcus neoformans [ 1 , 2 , 3 , 4 , 7 , 8 ].…”

“…The incidence of HIV and HLH in pediatric and non-pediatric patients is rare [ 1 , 2 , 3 , 4 , 5 , 6 , 7 ]. According to reports, the most frequently isolated infectious agents triggering HLH in HIV patients are EBV, HHV-8, Histoplasma capsulatum , Mycobacterium sp., CMV, and Cryptococcus neoformans [ 1 , 2 , 3 , 4 , 7 , 8 ]. On the other hand, some reports of ehrlichiosis caused by Ehrlichia chaffeensis and E. erwingii in non-pediatric HIV patients or non-immunosuppressed pediatric patients have been described less frequently [ 9 ].…”