Abstract INTRODUCTION Visceral leishmaniasis (VL) is an ill-studied disease that is endemic to several regions of Brazil. It is often complicated by hemophagocytic lymphohistiocytosis (HLH), a potentially fatal disorder resulting from excessive non-malignant activation/proliferation of T lymphocytes and macrophages. Considering the overlapping clinical and laboratory characteristics of these diseases, diagnosing HLH is a challenge. Therefore, tracking the association between VL and HLH is necessary in endemic areas. Although HLH can be inapparent and resolve with antileishmanicides, this may not always occur. HLH causes high lethality; therefore, immunosuppressive therapy should be instituted immediately in order to avoid a fatal outcome. METHODS: We described the epidemiological, clinical, laboratory, and therapeutic profile of this association in a region of Brazil endemic for VL. RESULTS We presented 39 patients with this association in a retrospective cohort of 258 children who were admitted from January 2012 to June 2017. Of the 39 patients, 31 were from urban areas (79.5%), and 21 (53%) were males. The mean age and weight were 2.86 (2.08) years and 14.03 (5.96) kg, respectively. The main symptoms were fever (100%), hepatosplenomegaly (100%), pallor of the skin and mucosa (82.5%), edema (38.5%), bleeding (25%), and jaundice (7.5%). Hemophagocytosis was identified in 16/37 (43.24%) patients, and direct examination revealed that 26/37 (70.27%) patients were positive for VL. The patients were treated as recommended by the Ministry of Health. CONCLUSIONS It was observed that HLH is a common complication in endemic areas, and its diagnosis must consider the overlapping of clinical characteristics and pancytopenia.

Costela intratorácica é definida como uma anomalia congênita rara, possivelmente causada por uma fusão incompleta dos processos cefálico e caudal dos esclerótomos durante a embriogênese. Frequentemente é achado incidental, porém, pode estar associada a malformações vertebrais e a alguns sintomas como dor torácica. O quadro apresentado é de costela intratorácica à esquerda, associada a malformação vertebral, com quadro clínico inespecífico.

Intrathoracic rib is defined as a rare congenital anomaly, possibly caused by an incomplete fusion of cephalic and caudal processes of sclerotomes during embryogenesis. Frequently, such abnormality is incidentally found, but may be associated with vertebral malformation and some symptoms such as chest pain. The authors report the case of a patient with left-sided intrathoracic rib in association with vertebral malformation, with a non-specific clinical picture.