Human Herpesvirus 8-Associated Hemophagocytic Lymphohistiocytosis in Human Immunodeficiency Virus-Infected Patients

Abstract

We retrospectively reviewed 5 cases of hemophagocytic lymphohistiocytosis (HL) associated with human herpesvirus 8 (HHV-8) reactivation in human immunodeficiency virus (HIV)-infected patients. All patients had clinical and biological features characteristic of HL. Pulmonary symptoms were present in all patients and were frequently life threatening. The mean number of HL episodes was 6. Four patients had HL-associated Kaposi sarcoma, and 3 had multicentric Castleman disease. The mean CD4 cell count was 200 cells/mm(3). HIV loads were stable in all patients. All patients had high levels of HHV-8 in peripheral blood mononuclear cells during attacks, and a significant increase in this parameter before the attacks was seen in 3 patients. Although 2 patients died of HL, 3 are still alive and receiving etoposide therapy (mean follow-up, 3 years). HHV-8-related HL is associated with life-threatening symptoms and biological HHV-8 reactivation, and it may be controlled in the long term by etoposide therapy combined with highly active antiretroviral therapy.

Development of hemophagocytic lymphohistiocytosis in triplets infected with HHV-8

“…The source of the HHV-8 infection is difficult to discern; however, since a small percentage of healthy American adults are HHV-8 seropositive, one possibility is transmission via a blood product while in the nursery. 15 Although HLH has been previously associated with HHV-8 infection, all of these reports have been in HIV-infected individuals with associated Kaposi sarcoma, [16][17][18] or patients who received a renal transplant. 19,20 Genetic analysis demonstrated that triplets A and B had the same compound heterozygous mutations in the perforin gene.…”

Haemophagocytic lymphohistiocytosis: A fulminant syndrome associated with multiorgan failure and high mortality that frequently masquerades as sepsis and shock

“…However, the median CD4 + counts of the patients was significantly lower (200 cells/µl). [20] Case 1 was HIV-negative on ELISA and p24 antigen-negative at admission. Recent repeat HIV testing revealed that he is HIV-positive with a CD4 + count of 371 cells/µl and a VL of 104 copies/ml.…”

“…Members of the herpesvirus family coincidentally are the most frequent triggers of viral-associated HLH. [5,6,20] These include Epstein-Barr virus (EBV), the reactivation of which has been implicated the most in viral-associated HLH, [21] CMV and human herpesvirus 8 (HHV-8). EBV was detected by in situ hybridisation of the trephine in case 2.…”

Haemophagocytic lymphohistiocytosis in Adult- A Case Report and Literature Review

“…26 Fardet et al reported Human Herpes virus 8 associated HLH among HIV-infected patients. 27 Malignancy associated acquired HLH (MAHS), with lymphoma being the commonest trigger, is well known entity in adults but rare in children. In a recent review of patients with lymphoma associated haemophagocytic syndrome (LAHS) in Japan showed that EBV genome was detected from more than 80% of T/NK cell lymphoma but rarely from B cell lymphoma.…”