Case 3-2011: Invasive mucormycosis (zygomycosis) after bone marrow transplantation in a 26-year-old man with relapsing acute myeloid leukaemia.

Patient J.S., 26-year-old man, was first seen by his general practitioner because of chest pain, dyspnoea, weakness, vomiting, headache, dizzines and recent fever on January 29th 2010. Blood samples were taken and revealed leukocytosis 149 × 109/L. His previous medical history was unremarkable. He was immediately referred to our University Hospital with suspicion of acute leukaemia. On admission in the evening of the same day, his leukocyte blood count was already 248 × 109/L and mild anaemia and thrombocytopenia was present. The diagnosis of acute myeloid leukaemia (myelomonocytic according to WHO classification) was made. There also was a severe syndrome of leukostasis on admission, and the patient developed acute myocardial infarction and respiratory insufficiency. Immediate leukoreduction with leukapheresis and hydroxyurea was started. When leukocyte count was reduced below 50 × 109/L chemotherapy with intermediate dose cytarabine (total dose 24 g) and idarubicin (total dose 70 mg) was started. Despite drastic leukoreduction our patient developed systemic inflammatory response syndrome (SIRS) with multiorgan failure reflecting tumor lysis syndrome following chemotherapy. He received artificial ventilation for respiratory failure caused by diffuse alveolar haemorrhage, and continuous renal replacement therapy for renal failure was started. The course of his disease was further complicated by shock, disseminated intravascular coagulation and liver failure. With full supportive care and corticosteroids patient was stabilised and eventually, after 5 days, he was successfully extubated and his renal and liver function recovered. Unfortunately, the induction chemotherapy did not lead to remission of the leukaemia. Patient received reinduction chemotherapy with FLAG IDA regimen (fludarabine, cytarabine, idarubicin, G-CSF) on March 6th. The remission was not achieved once again and another reinduction with HAM chemotherapy (cytarabine, mitoxantrone) was instituted on April 18th. Finally, there was a complete remission on bone marrow examination on May 27th. Patient obtained consolidation treatment (again HAM chemotherapy) and was scheduled for allogeneic stem cell transplantation. On August 18th he was admitted for allogeneic peripheral blood stem cell transplantation. His donor was unrelated woman with 3 mismatches (7/10). Unfortunately, before transplantation his bone marrow examination revealed a relapse of acute leukemia, and we decided to proceed to fully ablative approach enforced with cytarabine and mitoxantrone. The remission with 96% donor chimerism was achieved on September 29th. After transplantation he developed grade 3 skin acute graft versus host disease and BK virus hemorrhagic cystitis. Graft versus host disease resolved with corticosteroids treatment and hemorrhagic cystitis with reduced immunosupression. On November 11th, second relapse of the disease was diagnosed. The patient received chemotherapy and again developed multiorgan failure with respiratory failure and was artificially ventilated. He recovered and still cytopenic he refused further hospital stay and was discharged. Voriconazol was used as a prophylaxis during neutropenia. He was admitted shortly afterwards with the clinical signs of sepsis. Physical examination and X-ray revealed left side pneumonia and pericarditis. On pulmonary high resolution CT scan (HRCT), there was bilateral pneumonia with atypical pattern (Fig. 1). He also complained of vision disturbance, and an ischemic lesion in the occipital area on brain CT scan was found (Fig. 2). Lumbar puncture did not reveal any pathogen. Immediate treatment with antibiotics (meropenem and amikacin) and amphotericin B in combination was started. The patient died within 2 days (November