Abstract
Anaplastic lymphoma kinase-positive large B-cell lymphoma (ALK+ LBCL) is a very rare and aggressive subtype of diffuse large B-cell lymphoma characterized by ALK rearrangement. Immunophenotypically, the tumor cells are typically negative for common B-cell markers, T-cell markers, and CD30; however, they express markers of terminally differentiated B cells/plasma cells such as CD38, CD138, and MUM-1/IRF4. The diagnosis of ALK+ LBCL can be challenging, and often a large panel of immunostains is required to exclude other hematopoietic and nonhematopoietic neoplasms. To date, approximately 130–140 cases have been reported, but here we report the first known case of ALK+ LBCL with unusual CD33 expression.
Highlights
Anaplastic lymphoma kinase-positive large B-cell lymphoma (ALK+ LBCL) is a rare hematopoietic neoplasm accounting for less than 1% of diffuse large B-cell lymphomas (DLBCL) [1]. e entity was first described in 1997, and to date, approximately 130–140 cases have been reported in the literature [2]
To the best of our knowledge and based on literature review, we describe the first case of ALK+ LBCL with CD33 expression
ALK+ LBCL is a rare type of large cell lymphoma recognized by the World Health Organization’s classification of tumors of hematopoietic and lymphoid tissue [1]
Summary
Anaplastic lymphoma kinase-positive large B-cell lymphoma (ALK+ LBCL) is a rare hematopoietic neoplasm accounting for less than 1% of diffuse large B-cell lymphomas (DLBCL) [1]. e entity was first described in 1997, and to date, approximately 130–140 cases have been reported in the literature [2]. Anaplastic lymphoma kinase-positive large B-cell lymphoma (ALK+ LBCL) is a rare hematopoietic neoplasm accounting for less than 1% of diffuse large B-cell lymphomas (DLBCL) [1]. Rare cases are associated with t(2;5)(p23; q35), as described in ALK-positive anaplastic large cell lymphoma [1]. Myeloid lineage-associated markers such as CD13 and CD33 that are commonly expressed by myeloid neoplasms are not routinely performed as a part of lymphoma workup and have not been reported in ALK+ LBCL. CD33 has been reported in a high percentage of ALK+ anaplastic large cell lymphoma, rare cases of CLL/SLL, and Burkitt lymphoma [9,10,11]. To the best of our knowledge and based on literature review, we describe the first case of ALK+ LBCL with CD33 expression
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