Severe mycotic pansinusitis in a patient with hairy cell leukemia, ending lethally

Abstract

Hairy cell leukemia, HCL is the result of a mutation of B-memory lymphocytes from the germinal center with a low degree of malignancy, with an indolent course, slow progression and a very good therapeutic effect. Clinically, HCL is characterized by a tendency toward peripheral pancytopenia, discrete lymphoadenomegaly, and significant splenomegaly. The main reason for shortened survival, even in contemporary, is various infectious complications, especially opportunistic infections, mycotic, etc. They not only limit or compromise the therapeutic response, but also often lead to a fatal outcome.
We present a 42-year-old patient diagnosed with HCL in 2014 who underwent a significant number of specific courses of treatment, including splenectomy. In a subsequent recurrence, marked enlargement and thickening of the mucosa of the left maxillary, sphenoidal, frontal and ethmoidal sinuses, polypoidal mucosal hypertrophy with partial obstruction of the nasal passages, massive necrotic involvement of the soft tissues and bilateral thrombosis of the facial arteries were found. Histological examination confirmed mycotic hyphae. Aspergillus Flavus was verified microbiologically. Despite comprehensive conservative and surgical treatment, the disease ended fatally. Infectious complications (especially mycotic ones) are the result of severe quantitative and qualitative disorders in the main effector cell lines with pronounced granulocytopenia, monocytopenia, reduced activity of natural killers, T-helpers, poor antibody response even with normal immunoglobulins and reduced antibody-dependent cellular cytotoxicity. When such complications occur, patients require immediate action by an interdisciplinary team.