A Review of Hemophagocytic Lymphohistiocytosis in Patients With HIV

Abstract

We provide an elaborate review of cases published between January 2005 and April 2021 on hemophagocytic lymphohistiocytosis (HLH) in HIV patients. Seventy articles describing 81 adult patients (age ≥19 years) were included. Median age was 40 years, and 78% were males. Only 65% were known to have HIV prior to presentation. CD4 count was ≥200 cells/mm3 in 23% and HIV viral load <200 copies/mL in 41%. The lack of meticulous reporting of ≥5 of 8 criteria for HLH diagnosis was evident in a third of cases. At least one infectious agent - other than HIV- was believed to trigger HLH in 78% of patients. The most common were EBV (26%), HHV-8 (21%) and Histoplasma capsulatum (17%). Sixty percent survived. Among those, 93% received treatment for identified secondary trigger(s) while 51% received HLH directed therapy. There was significant heterogeneity in the treatment regimens used for HLH.

Clinical Characteristics of Retroviral Rebound Syndrome: A Case Report and Literature Review

“…Non-infectious triggers included pregnancy and malignancy. Those cases without obvious triggers (42%) were attributed to acute retroviral syndrome (ARS); however, none have reportedly been triggered by RRS ( 6 ). A meticulous search for possible triggers in the present case, including contrast enhanced computed tomography, serologic tests for EB virus and toxoplasmosis, hepatitis screening, and multiple blood cultures, failed to identify triggers other than HIV alone, and the patient recovered without treatment for other conditions.…”

Presumptive hemophagocytic syndrome associated with co‐infections with FIV, Toxoplasma gondii, and Candidatus mycoplasma haemominutum in an adult cat

“…HS has previously been described in a cat with presumptive calicivirus infection, 9 a cat with multiple myeloma 10 and a cat with cardiac abnormalities and detection of antibodies against FIV (Table 2). 11 Human immunodeficiency virus (HIV) has been associated with HS in humans, 17,18 triggered by HIV itself or by HIV and other infections and malignancies, which are higher in this patient population. 18 In a review article describing 81 HIV patients with HS, 26 of 63 tested for HIV viral loads had low viral loads, 18 as the cat in this case.…”

“…11 Human immunodeficiency virus (HIV) has been associated with HS in humans, 17,18 triggered by HIV itself or by HIV and other infections and malignancies, which are higher in this patient population. 18 In a review article describing 81 HIV patients with HS, 26 of 63 tested for HIV viral loads had low viral loads, 18 as the cat in this case. Six months after the first PCR was done, the PCR to quantify FIV proviral DNA, performed in a different external laboratory, was undetectable.…”

The value of bone marrow examinations performed in the investigation of HIV infected patients with cytopenias

“…HIV may infect bone marrow (BM) stromal cells, T‐lymphocytes and macrophages resulting in the dysregulation of BM cytokines and chemokines which in turn disturbs haematopoiesis 3 . Additionally, drug therapy and a predisposition to opportunistic infections, autoimmune diseases, haemophagocytic lymphohistiocytois (HLH) and certain malignancies are indirect ways in which HIV can affect haematopoiesis or cause peripheral destruction of blood cells 3–5 . The proposal that HIV can directly infect haematopoietic stem and progenitor cells is contested 6–9 …”

“…3 Additionally, drug therapy and a predisposition to opportunistic infections, autoimmune diseases, haemophagocytic lymphohistiocytois (HLH) and certain malignancies are indirect ways in which HIV can affect haematopoiesis or cause peripheral destruction of blood cells. [3][4][5] The proposal that HIV can directly infect haematopoietic stem and progenitor cells is contested. [6][7][8][9] Bone marrow (BM) aspirate and trephine biopsy together with adjunctive investigations, herein referred to as BM examination (BME), may be utilised to investigate HIV-infected patients with unexplained cytopenia.…”