Reconstitution Inflammatory Syndrome Like Reactive Hemophagocytic Syndrome Associated with Disseminated Histoplasmosis in a HIV Patient

Abstract

We describe an unusual clinical association of disseminated histoplasmosis with reactive hemophagocytic syndrome. We report the case of a new HIV-positive patient with reconstitution inflammatory syndrome like reactive hemophagocytic syndrome associated with disseminated histoplasmosis. We describe the clinical case, the procedures performed, the treatment provided and the patient's evolution. A figure of liver biopsy Grocott's silver methenamine stain that shows lots of uniform ovoid yeasts in portal spaces' macrophages that supports the diagnosis of disseminated histoplasmosis in our case.

Histoplasmosis Diagnosed in Europe and Israel: A Case Report and Systematic Review of the Literature from 2005 to 2020

“…Progressive disseminated histoplasmosis (PDH) was diagnosed in 102 of the 114 cases (89.5%); the remaining 12 patients presented with colonic or small bowel involvement (6 cases, 5.3%) [17,18,21,31,34,68], lymph node involvement (2 cases, 1.8%) [24,28], and there was one case each of primary cutaneous [39], cerebral [64], pulmonary [36] or vaginal histoplasmosis [35]. Eight cases of immune reconstitution inflammatory syndrome (IRIS) were diagnosed following antiretroviral treatment [26,29,48,57,59], three patients had haemophagocytic syndrome [27,30,59], and one presented with posterior reversible encephalopathy syndrome (PRESS) [54]. Concomitant opportunistic infections and/or neoplasia were observed in 28.3% of the patients at the time of the diagnosis of histoplasmosis (Table 2 [23,50,51,69], two disseminated cryptococcosis [18,52], and one pulmonary coccidioidomycosis [22].…”

“…)[18,19,22,23,25,28,30,[32][33][34]37,39,41,44,45,[49][50][51]53,56,58,59,65,69]: ten patients had candidiasis[19,22,25,41,44,45,51,56,58,59], five Pneumocystis jirovecii pneumonia…”

Hemophagocytic Lymphohistiocytosis Secondary to Disseminated Histoplasmosis

“…5,6 Most reported cases of histoplasmosis-associated HLH are in the setting of HIV with depressed CD4 cell count. [4][5][6]10,11 In our series, 2 of the patients were on TNF-α inhibitors for autoimmune disease, which would presumably blunt cytokine release, and the third was broadly immunosuppressed. Tumor necrosis factor α inhibitors are a well-described risk factor for histoplasmosis.…”

“…The American Society of Hematology encourages use of the full HLH-2004 chemotherapy protocol in the setting of viral infections, specifically Epstein-Barr virus and HIV, but acknowledge that HLH induced by intracellular infections may respond to directed antimicrobial therapy. 4,11,15 This alludes to the importance of a rigorous infectious workup when managing adults with HLH. This includes not only testing for viral infections but also workup for histoplasmosis in the appropriate geographical location.…”

Histoplasmosis: Epidemiology, Diagnosis, and Clinical Manifestations

“…Hemophagocytic lymphohistiocytosis (HLH) has been reported in association with disseminated histoplasmosis, particularly in patients with AIDS who have profoundly suppressed CD4 counts [80,81]. This life-threatening complication has been reported to resolve in some patients treated with antifungal therapy alone and without the need for further cytotoxic chemotherapy [80,81].…”