A 15-year-old female from the Macanao municipality on the Margarita's Island in Venezuela presented to the local health center with a 6-month history of intermittent fever predominantly occurring at night, malaise, 6 kg weight loss, arthralgias, loss of appetite, dizziness, and amenorrhea.
The patient lives in a rural area close to a landfill in a house built with cement ground, brick walls, and a metal roof without running water. She has one dog (unknown Leishmania infection status) and various turtles as pets. She has no history of traveling outside Venezuela. On physical examination, she was pale, with increased respiratory and heart rate, and fever of 39 C. Her weight was 49 kg (percentile 36% according to age and gender), height of 1.57 m (percentile 22.3% according to age and gender), and an abdominal circumference of 76 cm. She had cervical, axillary, and inguinal adenopathy. Cardiac and lung examination were normal. Abdominal examination revealed right upper quadrant tenderness to palpation, and hepatosplenomegaly (liver was palpable at right flank 9.5 cm from the midclavicular line, and spleen with Hackett's system grade V) (Figure 1). Laboratory values on admission demonstrated pancytopenia (Table 1). Abdominal ultrasound and computed tomography confirmed the presence of hepatosplenomegaly. The initial clinical suspicion was that of a lymphoproliferative or myeloproliferative disorder. From an infectious disease perspective, the possibility of VL, a systemic mycosis, or tuberculosis were entertained.
We performed a bone marrow biopsy to rule out a hematologic malignancy but also to assess the possibility of histoplasmosis or VL by Giemsa staining. Cultures were negative, but the K-39 ELISA Leishmania titers were positive at 1:100. Immuno-phenotyping of bone marrow cells demonstrated a heterogeneous population of leukocytes with arrest and anomalies in the myeloid line's antigenic maturation, and 1% of blasts compatible with a secondary myelodysplastic syndrome. Bone marrow biopsy showed hypocellularity, discrete collagen fibrosis, and increased macrophages with severe intracellular colonization of Leishmania donovani (Figure 2). Tuberculin skin testing and serologies for human immunodeficiency virus (HIV), syphilis, cytomegalovirus, and Epstein-Barr virus were all negative.
The patient received treatment with meglumine antimoniate at 20 mg/kg/day for 30 days and tolerated this therapy without any significant side effects. The patient experienced significant improvement after completion of the treatment, becoming afebrile, and with increased appetite and gradual regression of her hepatosplenomegaly, and was discharged on stable clinical condition. Follow up laboratories after completion of antiparasitic therapy showed a hemoglobin of 10 g/dl, hematocrit of 29%, leukocytes of 5800 U/l, and platelets of 231,000 U/l.