Successful Treatment of Severe Aplastic Anemia with Hematopoietic Stem Cell Transplantation in the Setting of Active Mucormycosis.

ABSTRACT

Severe aplastic anemia (SAA) is a life-threatening hematological disease characterized by the suppression of the bone marrow. Patients with SAA are predisposed to recurrent bacterial infections and invasive fungal infections (IFI) due to profound and persistent neutropenia. Mucorales are the second most common cause of IFI encountered in SAA. Here we present a pediatric case of SAA with active mucormycosis infection of the paranasal sinuses. In the first step, surgical debridement was performed and combined antifungal therapy (liposomal amphotericin B, posaconazole, caspofungin) was started. Due to severe neutropenia, daily granulocyte transfusion was added to therapy. Hyperbaric oxygen therapy was applied for wound healing. After all this the patient went under flap surgery. One week after the successful flap procedure, HSCT was performed and he had no complications related to HSCT. The patient was followed in the outpatient clinic for 6 months with posaconazole. Now, he is out of drugs and followed without problems for 15 months after HSCT. Our case confirms that urgent HSCT with multiple therapies (surgical debridement, granulocyte support, combined antifungal therapy, hyperbaric O2) is crucial for saving life in SAA patients with active mucormycosis.