A Case of Parvovirus-Related Haemophagocytic Lymphohistiocytosis in a Patient with HbH Disease

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is rare and life-threatening medical emergency. Parvovirus infection is rarely associated with HLH. We report a case of parvovirus-related HLH in a patient with alpha thalassaemia (HbH disease). The patient responded well to a course of dexamethasone without the need of etoposide. Based on our literature search, this is the first case of parvovirus related HLH in a patient with HbH disease in the medical literature.

Hemophagocytic Lymphohistiocytosis Associated With Parvovirus B19 in a Patient With Acquired Immunodeficiency Syndrome

“…While IVIG has been used successfully in the treatment of parvovirus-associated HLH, there are some cases that have been treated with high-dose corticosteroids alone. 8,12 We believe that in our patient, IVIG alone was insufficient in treating HLH, and etoposide and dexamethasone were required as the patient only began showing clinical improvement with the initiation for the latter 2 agents. Regardless, the underlying cause must be sought after and treated if possible, similar to our patient's worsening immune suppression, which was corrected with initiation of antiretroviral agents.…”

The Dilemma of Diagnosing Hemophagocytic Lymphohistiocytosis in Sickle Cell Disease

“…Many reported cases of parvovirus B19 and HLH improved spontaneously or with high dose steroids, as in our case. HLH has been reported previously with sickle cell crisis [ 5 ] and in an adult patient with HbH and parvovirus infection [ 6 ]. HLH has been rarely reported with other infections, also like Mycobacterium avium, histoplasmosis in patients with sickle cell anemia, and both the reported cases succumbed to their infection [ 7 - 8 ].…”

Cytomegalovirus and disseminated histoplasmosis-related hemophagocytic lymphohistiocytosis syndrome in an HIV-patient late presenter with IRIS: a case report

“…Also, the release of pro-inflammatory cytokines promotes the hemophagocytosis, a hallmark of HLS [7]. Previous reports indicate that the presence of hemophagocytosis has a sensitivity of 80-83% and a specificity of 60% for HLS; nevertheless, hemophagocytosis can be absent in some cases [10].…”

“…Reactive HLS carries a high mortality, ranging from 52 to 74% at 1 year, depending of variables like patients older than 30 years, the presence of disseminated intravascular coagulation, elevated ferritin level, and anemia with accompanying thrombocytopenia [28]. HLS treatment consists of dexamethasone and etoposide [10]. In this paper, we described a patient with acquired immunodeficiency syndrome who presented HLS related to an unmasked IRIS, secondary to disseminated histoplasmosis and CMV infection.…”