Splenic marginal zone lymphoma with t(8;14)(q24.1;q32)/MYC rearrangement

Excerpt

Splenic marginal zone lymphoma (SMZL) is a distinctive low-grade B-cell lymphoma that was first termed by Schmid and colleagues [1] in 1992. The 2008 World Health Organization (WHO) classification of tumors of haematopoietic and lymphoid tissues defines SMZL as a “B-cell neoplasm comprising of small lymphocytes that surround and replace the splenic white pulp germinal centers, efface the follicle mantle, and merge with a peripheral zone of larger cells, including scattered transformed blasts; both small and larger cells infiltrate the red pulp” [2]. According to the WHO, this rare low-grade B-cell lymphoma is considered to be a distinct clinical and pathological entity. SMZL is characterized by massive splenomegaly with very frequent involvement of the bone marrow and peripheral blood [2]. The splenic hilar lymph nodes and bone marrow are often involved, and the lymphoma cells are often found in the peripheral blood as villous lymphocytes [2]. Therefore, the terminology “splenic lymphoma with circulating villous lymphocytes” has been used in the past literature [3]. …