MEDICAL HISTORY AND CLINICAL COURSE: A 42-year-old patient with hairy cell 
leukemia had been treated for 3 years by a hematologist in private practice. 
Initially the patient received 1 course of cladribine upon which the disease 
went into complete remission. 6 weeks ago a relapse was diagnosed and 
combination therapy with cladibrin and rituximab was initiated. Now the patient 
presented to the emergency room with shortness of breath and pain when 
breathing.
INVESTIGATIONS, TREATMENT AND COURSE: In the chest x-ray, patchy infiltrates and 
pleural effusions were found on both sides. The subsequently performed computed 
tomography showed bilateral compactions with an Halo suspicious for fungal 
infiltrates. Upon admission to the hospital, an empirical antibiotic therapy 
with clarithromycin and piperacillin/tazobactam was initiated, which was later 
escalated to meropenem and linezolid. Additionally, an antifungal therapy with 
voriconazole was started and later switched to liposomal amphotericin B. At his 
admission, a positive aspergillus antigen could be detected in the 
microbiological laboratory. Under antimycotic treatment the aspergillus antigen 
was repeatedly negative. The patient presented with pronounced cytopenias and 
after a switch of therapy to vemurafenib and filgrastim, the hematopoiesis could 
only be stimulated insufficiently. The patient was transferred to the intensive 
care unit three days after admission with severe respiratory failure. He died on 
day 8 after admission. AUTOPSY AND DIAGNOSIS: Diagnosis was consistent with 
relapse of hairy cell leukemia with positive BRAF mutation and a bone marrow 
infiltration > 80 %. Autopsy revealed a significant hepato-splenomegaly, a lack 
of erythro-, granulo- and thrombopoiesis. Clots interspersed with fungal hyphae 
were found in both lungs and an infarction of the spleen with evidence of fungal 
hyphae was detected. The cultural findings post mortem on yeast or mold were 
negative.
CONCLUSION: Patients with refractory hairy cell leukemia and prolonged 
neutropenia are at increased risk for systemic fungal infections. Therefore, 
prohylactic antimycotic therapy should be considered early in this group of 
patients. The therapeutic approach of vemurafenib in treatment-refractory hairy 
cell leukemia is promising and offers an additional treatment option. In the 
present case, the patient could unfortunately not be stabilized due to the 
septic complications.