Perfil epidemiológico, clínico-laboratorial e terapêutico de pacientes com a associação leishmaniose visceral/linfo-histiocitose hemofagocítica, admitidos em hospital de referência em área endêmica na região norte do Estado de Minas Gerais

Abstract

Visceral leishmaniasis (VL) is a neglected disease that is endemic in several regions of Brazil. It often presents with hemophagocytic lymphohistiocytosis (HLH), a potentially fatal disorder, as a complication, resulting from the excessive activation/proliferation of nonmalignant T lymphocytes and macrophages. The epidemiological, clinical, laboratorial, and therapeutic profile of this association in an endemic region, in the northern state of Minas Gerais, will be described. A total of 39 (15.1%) cases were analyzed from a retrospective cohort of 258 children from urban areas 31/39 (79.5%). The children had been hospitalized from January 2012 to December 2017, aged on average 2.86 ± 2.08 years, and 21/39 (53%) were male. Their average weight was 14.03 ± 5.96 kg. The main signs and symptoms were fever (100%), hepatosplenomegaly (100%), skin paleness (82.5%), edema (38.5%), bleeding (25%), and jaundice (7.5%). Blood transfusions were performed in 19/39 (48.71%) patients. Concentrated red blood cells were used in 18 (46%) patients, concentrated platelets in 7 (36.84%), and fresh frozen plasma in 3 patients (15.78%). Laboratory findings showed low levels of hemoglobin (6.82 ± 1.36 g/dL), leukocytes (3,722 ± 1,472/mm3), neutrophils (1,161 ± 671/mm3), platelets (58,384 ± 30,536/mm3), and prothrombin activity (73.65 ± 17.81%). Elevated levels of ferritin (1,497 ± 1,550 ng/dL); triglycerides (404.71 ± 230.77 mg/dL); C-reactive protein (64.06 ± 45.73 mg/L); glutamic-oxalacetic transaminase (245.58 ± 380.53 UI/L); glutamic-pyruvic transaminase (110.33 ± 177.79 UI/L); total (0.75 ± 0.72 mg/dL), direct (0.35 ± 0.46 mg/dL), and indirect (0.39 ± 0.33 mg/dL) bilirubin; total proteins (6.47 ± 1.33 g/dL); albumin (2.98 ± 0.47g/dL); globulins (3.59 ± 1.00 g/dL); creatinine (0.49 ± 0.14 mg/dL); urea (21.81 ± 7.09 mg/dL); amylase (42.10 ± 22.54 UI/L); and lipase (32.23 ± 25.68 UI/L) were found. Hemophagocytosis was identified in 16/37 (43.24%) patients. The direct test for VL was positive in 26/37 (70.27%), and the rapid test for VL was positive for 32/34 (94.11%). Fibrinogen, cluster of differentiation 25, or natural killer cell activity was not measured. Antibiotic therapy was administered in 31/39 (79.48%) patients, empirically in 22/39 (56.41%) and specifically in 9/39 (23.07%). Corticotherapy with dexamethasone was prescribed in 38.46% (15/39). Nine patients (60%; 9/15) received anti-inflammatory doses, and six (40%, 6/15) received immunosuppressive doses. Six (40%) patients were younger than 2 years of age. All patients tested negative for HIV and received their first treatment for VL. Meglumine antimonate was administered to 18 (46.1%) patients, of which 10 (25.64%) recovered and 8 (20.51%) interrupted the treatment. Five of the treatments (12.82%) were interrupted for no therapeutic response and three (7.69%) due to adverse effects. Liposomal amphotericin B was prescribed in 20 (51.28%) patients, of which 12 (30.76%) took 4 mg/kg/day for 5 consecutive days, and 8 (20.51%) took 3 mg/kg/day for 7 days. A total of 19 (95%) recovered. One patient (2.56%) took amphotericin B deoxycholate successfully. Recovery treatment was administered with liposomal amphotericin B. Six patients (15.38%) took 4 mg/kg/day for 5 consecutive days, and two took 3 mg/kg/day for 7 days. All patients recovered. The average time between the onset of the symptoms and admission was 23.72 ± 22.84 days. Fever was reported by all companions as an initial symptom of the disease. The hospitalization time was on average 17.74 ± 5.7 days. All patients underwent a 12-month follow-up and recovered from all symptoms. Therefore, HLH is not a rare complication in endemic areas, and the diagnosis of HLH should be considered due to overlapping clinical characteristics and pancytopenia. Liposomal amphotericin B proved to be the best therapeutic option.