A Rare Case of Hemophagocytic Lymphohistiocytosis Associated with Parvovirus B19 Infection

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening syndrome resulting from excessive immune activation. Secondarily, HLH is often associated with autoimmune disease, infection, and malignancy. The most common infectious trigger is Epstein-Barr virus (EBV) infection. HLH is rarely triggered by parvovirus B19. We discuss a case of a 62-year-old male who presented with multi-organ failure with presumed septic shock who eventually was diagnosed with HLH, with positive parvovirus B19 deoxyribonucleic acid (DNA) polymerase chain reaction (PCR). Prompt treatment with dexamethasone resulted in significant clinical resolution.

Parvovirus B19‐induced hemophagocytic lymphohistiocytosis: Case report and review of the literature

“…Similarly, 2 cases of PVB19‐HLH in pregnancy describe females who recovered and delivered healthy children while 7 cases of PVB19‐HLH in patients with underlying hematologic aberration exhibited recovery either with corticosteroids or no specific intervention . Eleven cases of PVB19‐HLH in reportedly healthy individuals were found, where eight patients recovered either with steroids or no treatment, while the other three suffered from multiorgan dysfunction before passing away despite steroids, IVIG, and/or etoposide . Only 9 cases actually described meeting five or more diagnostic criteria: Six of these patients recovered with either no intervention, steroids, and/or IVIG while the other three succumbed to their disease …”

“…Eleven cases of PVB19‐HLH in reportedly healthy individuals were found, where eight patients recovered either with steroids or no treatment, while the other three suffered from multiorgan dysfunction before passing away despite steroids, IVIG, and/or etoposide . Only 9 cases actually described meeting five or more diagnostic criteria: Six of these patients recovered with either no intervention, steroids, and/or IVIG while the other three succumbed to their disease …”

Tratamiento exitoso de síndrome hemofagocítico secundario a infección por virus de Epstein Barr. Reporte de caso

“…Nuestro país reúne todas las condiciones para que más de estos casos puedan presentarse, pues contamos con tasas elevadas de enfermedades infecciosas y condiciones sanitarias que las propician [21] . Se han descrito casos de SHF secundarios a diferentes infecciones virales y bacterianas; por tanto, es de vital importancia enfocar nuestra atención en la población pediátrica pues la inmadurez de su sistema inmune puede hacerlos más susceptibles al desarrollo del SHF [6,12,23] .…”

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

“…HLH is a syndrome caused by increased immune response, excessive macrophage activation, and cytokine release that leads to multiorgan dysfunction [1, 2]. It can be primary (familial) or secondary (reactive) [1]. Primary HLH is due to genetic defect and is mainly seen in children [1].…”

“…Hemophagocytic lymphohistiocytosis (HLH) is rare and life-threatening medical emergency [1]. Our patient is a middle-aged Chinese lady with alpha thalassaemia (HbH disease), presented to us with persistent fever and rapidly progressive pancytopaenia.…”