A 12-year-old African boy from Guinea-Bissau was referred to our hospital with a presumptive diagnosis of lymphoma. Upon review, he had a 2-year history of multiple nodular formations in cervical, axillary, and inguinal regions, which were enlarging with time, and were associated with cutaneous fistulas. He had no fever, weight loss or respiratory symptoms. He had been treated in his home country with large-spectrum antibiotics for several weeks without any clinical improvement.
On physical examination he appeared pale, but was fully conscious, and had normal vital signs. Cervical, axillary, and inguinal nodular formations were discharging a yellowish pus. There were no abdominal palpable masses. The other physical examination was unremarkable.
Initial workup revealed anemia (hemoglobin = 7.2 g/dL), leucocytosis (15,860/muL) with neutrophilia, thrombocytosis (967,000/muL), and elevated C-reactive protein (98.3 mg/L). Liver and kidney function tests were normal. Evaluation for immunologic profile did not show any evidence of immunosuppression and human immunodeficiency virus testing was negative. Computerized tomography (CT) of the chest revealed a condensation in the left lower lobe with peripheral micronodules and hilar calcifications. Abdominal ultrasound was normal.
On day 3, he started a 6-month course of anti-tuberculous therapy due to a positive polymerase chain reaction (PCR) sample of Mycobacterium tuberculosis (Mt) in a gastric aspirate. This infection was later confirmed by a positive sputum culture. Meanwhile, a cervical nodular biopsy revealed necrotic areas along with others containing numerous histiocytes with multinucleated giant cells, polymorphic inflammatory infiltrate, and numerous uninucleate thick-walled large yeasts, budding from a narrow base (Fig. 1, arrow). Based on this morphology, a diagnosis of Hcd infection was made and he began therapy with liposomal amphotericin B (5 mg/kg/day). Histoplasmosis was later confirmed by multiple positive swab cultures of the exudates. After 4 weeks of amphotericin B therapy, he was switched to oral itraconazole (10 mg/kg/day). At this stage, a small clinical improvement was observed, with only minor reduction in the size of lymphadenopathies. In the following weeks, the patient clinical status was rapidly deteriorating, with enlargement of the previously noted adenopathies, along with appearance of new cutaneous fistulas. A painful supra-pubic abdominal mass appeared, and abdominal CT identified numerous new retroperitoneal lymphadenopathies and left hydronephrosis due to direct compression of the ureter (Fig. 2). Amphotericin B was restarted, with no apparent clinical benefit.
After approval by our hospital's ethics committee, posaconazole therapy was started, at a dosage of 400 mg twice daily. A dramatic and lasting improvement, both clinical (Fig. 3A-D) and biochemical, was observed. Imagiologic control 2 months after introduction of posaconazole demonstrated resolution of hydronephrosis and only minor (1 cm) lymphadenopathies. At day 127 of posaconazole therapy, complete cicatrization of all skin lesions was achieved (Fig. 3). Our patient completed 12 months of therapy with posaconazole. No relapse was noted during or 3 months after treatment.