Profound Hypereosinophilia Secondary to Sezary Syndrome

Background: Sezary syndrome is a subtype of cutaneous non-Hodgkin's lymphoma that is characterized by erythroderma, the presence of atypical lymphocytes in the blood, and lymphadenopathy. The clonal cell in Sezary syndrome is thought to be derived from mature Th-2 cells that are able to express Il-4, Il-5, and Il-13 which can stimulate the replication of eosinophils. It is has been shown that higher levels of eosinophilia correlate with a worse prognosis. One explanation is that the degree of eosinophilia represents the level of imbalance between Th-1 and Th-2 cells. Here we report the first case of end organ damage caused by hypereosinophilia secondary to the Sezary syndrome. In rare cases Sezary syndrome can cause hypereosinophilia but has never been reported to cause hypereosionphilia that results in end-organ damage. To our knowledge this is the first case that reports end-organ damage resultant from secondary hypereosinophilia from the Sezary syndrome.

Case presentation: A 75-year-old man was admitted for severe rash and pruritis, massive lymphadenopathy, and extremely high eosinophil count. He had recurrent skin rash with pruritis for five years with previous skin biopsies without evidence of malignancy. He had never had hypereosinophilia until he presented to our institution. Peripheral smear showed marked eosinophilia as well as the presence of Sezary cells. A skin biopsy and lymph node biopsy revealed cutaneous T cell lymphoma. Bone marrow biopsy showed atypical large T-cells comprising 29% of the total population with loss of CD5 and CD7 and 22% eosinophils. The patient ultimately sustained multiorgan failure and expired from respiratory failure. Tissue eosinophilia was later demonstrated on autopsy.

Conclusion: Our report indicates that the Sezary syndrome can cause extreme hypereosinophilia that can result in end-organ damage.