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Disseminated mucormycosis-induced perforated intestine in a late presenting AIDS patient with steroid-dependent secondary hemophagocytic lymphohistiocytosis

2015; Lippincott Williams & Wilkins; Volume: 29; Issue: 16 Linguagem: Inglês

10.1097/qad.0000000000000849

1473-5571

Danit Dayan, Subhi Abu‐Abeid, Joseph M. Klausner, Boaz Sagie,

Immunodeficiency and Autoimmune Disorders

HIV infection has been associated with secondary hemophagocytic lymphohistiocytosis (HLH) – a rare condition causing severe neutropenia with subsequent infection and death. The combination of HIV and HLH-induced neutropenia with steroid treatment for the latter could lead to mucormycosis – a fatal fungal infection. A 33-year-old man was recently diagnosed with late presenting AIDS and concomitant secondary HLH. Presenting symptoms included fever, night sweats, diarrhea, anorexia and weight loss for 1 month. Investigation revealed HIV-positive CD4+ cell count 24 cells/μl with a viral load of 900 000 copies/ml. Highly active antiretroviral therapy was initiated (emtricitabine/tenofovir/raltegravir), Nystatin for oral Candida albicans, PCP and MAC prophylaxis. During hospitalization, the following HLH criteria were fulfilled: fever, splenomegaly, pancytopenia, hypertriglyceridemia, elevated ferritin 17 000 μg/l and soluble CD25− 20 300 U/ml; bone marrow biopsy – without hemophagocytosis. Other infectious HLH causes were excluded. Total body computed tomography (CT) depicted splenomegaly and mildly dilated jejunum. Dexamethasone 18 mg/day was initiated with improvement. However, tapering down caused HLH exacerbation, hence resumed. Four months later he suffered new-onset right drop foot and headache. Brain CT was normal. Later on, he developed septic shock, secondary to severe pneumonia inflicted by resistant Acinetobacter baumannii. Also, perioral herpetiform-like lesions and oral candidiasis were treated with Zovirax and Fluconazole, respectively. He temporarily recovered in ICU, only to redeteriorate, complaining of abdominal pain. He became septic with abdominal tenderness. CT depicted pneumoperitoneum, multiple liver abscesses and lung abscess. Broad-spectrum antibiotics, Caspofungin, steroid stress dose and hydration were given. Urgent laparotomy revealed multiple liver abscesses, necrotic lesions at the distal stomach (Fig. 1), transverse colon and three necrotic widely open segments of small intestine located 20, 40 and 120 cm distal to the Treitz, with enteric spillage. Small bowel resection was performed. The stumps were left closed in the abdomen, planned for second-look operation. Pathology discovered many broad hyphae, some invaded vessel walls. Invasive mucormycosis was concluded and ambisome treatment was initiated. Second-look laparotomy on post operative day 1 (POD 1) revealed a large amount of turbid peritoneal fluid, peritoneal lesions, liver abscesses appeared more solid and one new lesion on the stomach. Esophagogastroduodenoscopy revealed lesions throughout the stomach. An enteroenteric anastomosis was completed, and multiple samples were sent for microbiology and pathology including fluid, liver and peritoneal lesions. All samples were infected with mucormucosis. Hence, further debridement did not seem justified in light of the dismal prognosis. Soon, left periorbital swelling developed, and CT depicted new infarct-like hypodense lesions in capsula interna and basal ganglia, marked infiltration of left facial soft tissues and sinuses. Transnasal endoscopy showed necrotic lesions in sinuses, biopsy invaded by mucormycosis. He died on POD 4.Fig. 1: Gastric lesion.HIV infection has been associated with secondary HLH, a rare fatal condition, in which activated macrophages engulf hematopoietic cells because of uncontrolled hyperinflammatory response [1]. Left untreated, severe neutropenia ensues, with subsequent bacterial or fungal infections and death. Revised diagnostic criteria include five of the following: fever, splenomegaly, cytopenias (>2 lineages), hypertriglyceridemia, hypofibrinogenemia, hemophagocytosis (bone marrow/spleen/lymph nodes), low/absent NK-cell activity, elevated ferritin and elevated soluble CD25 [2]. Treatment includes immunosuppression, immunomodulation and of the inciting trigger. HLH-induced neutropenia and corticosteroid treatment probably predisposed our patient to mucormycosis, a life-threatening infection caused by Mucorales fungi, with Rhizopus being amongst the most common species. It is characterized by extensive angioinvasion, thrombosis and tissue necrosis. It affects immunocompromised hosts. However, it is very rare in patients with HIV/AIDS, as it does not inherently predispose to mucormycosis, unless associated with predisposing factors [3]. Mucormycosis is classified into rhinocerebral, pulmonary, cutaneous, gastrointestinal, disseminated and miscellaneous [4]. Treatment strategies include early diagnosis, reversal of predisposing factors, surgical debridement and antifungal medications (lipid-based formulations of amphotericin B) [5,6]. Disseminated mucormycosis is a rapidly progressive and deadly disease, mandating a high index of suspicion and prompt medical and surgical treatment in an effort of salvage. Acknowledgements Conflicts of interest There are no conflicts of interest.