Search

Background: Chronic lymphocytic leukemia (CLL) is an indolent B-lineage neoplasm responsible for 30% of all leukemias. The median age of onset is 67 years with a male predominance of 2:1. Localized infiltration in the oropharynx and paranasal sinuses is exceptionally rare. The aims of this study were (1) to add an additional case series of CLL with involvement of the oropharynx and paranasal sinuses to the literature and (2) to determine incidence and demographic data., Methods: Retrospective chart review from 1990 to 2014., Results: Five cases were found in our case series, representing 0.74% of the total number of cases analyzed (5/680). Sixteen additional cases were identified through literature review, resulting in a total of 21 cases of CLL (13 men, 8 women) with involvement of the oropharynx (n = 15) and paranasal sinuses (n = 6). The average age of patients with CLL in the oropharynx was 62 years whereas in the paranasal sinuses it was 52 years (p = 0.16). The average age of female cases was 62 years and the average age of male cases was 58 years (p = 0.63). Almost 85% (84.6%) of men had oropharynx invasion vs 50% of females (p = 0.15), which suggests a nonsignificant trend., Conclusion: The results of our study indicate that CLL infiltrates the oropharynx or paranasal sinuses in less than 1% of CLL cases. Although there seems to be no age bias between invasion in the oropharynx and the paranasal sinuses, there is a trend whereby women appear more likely to experience invasion of the paranasal sinuses., (© 2015 ARS-AAOA, LLC.)

Fucosidosis is an autosomal recessive lysosomal storage disorder caused by the deficiency of alpha-L-fucosidase. We present the case of an affected female in the second decade of life with chronic rhinosinusitis (CRS) including recalcitrant polypoid inflammation, which has not been previously reported in the literature. With the advancement of life-prolonging measures, children with lysosomal storage disorders may suffer increasingly from CRS due to the lymphohistiocytic and macrophage infiltrate of the paranasal sinus mucosa that resembles severe polypoid inflammation. [ABSTRACT FROM AUTHOR]