Disseminated histoplasmosis induced hemophagocytic lymphohistiocytosis in an immunocompromised patient

Background

The search for the trigger of hemophagocytic lymphohistiocytosis (HLH) and its management can be challenging in immunocompromised patients. In HLH triggered by infection, immunosuppressive therapy is desired to suppress hyperinflammation, but may worsen the underlying infection.

Objectives

To review available literature on the management and prognosis of immunocompromised patients with disseminated histoplasmosis induced HLH.

Methods

We describe a case and review all previously reported cases of disseminated histoplasmosis induced HLH on PubMed until June 2023.

Results

The literature review yielded 30 cases. Antifungal therapy was administered in all except one patient. In 18 cases, HLH was treated with immunosuppressive therapy. The most common immunosuppressive regimens were corticosteroid monotherapy and corticosteroids combined with etoposide. This case report is the first to describe the use of anakinra. The overall mortality rate was 23.3 % (n = 7).

Conclusions

Disseminated histoplasmosis induced HLH is a severe disorder, requiring prompt recognition and immediate intervention. Currently, no specific treatment protocol exists for this disorder. Treatment should be tailored to the patient’s condition, symptoms and individual characteristics. Careful consideration regarding immunosuppressive therapy for HLH is needed to prevent worsening of the infection. Additionally, anticipating the progression of the disease is necessary to ensure timely and appropriate interventions.