title: Follicular lymphoma in situ in the spleen of a patient with autoimmune hemolytic anemia and carrying HCV was associated with more clonal B-cells than t(14;18) positive B-cells.
abstract: Certain autoimmune conditions are associated with an increased risk of lymphoid malignancy. We report a 65-year old patient with autoimmune hemolytic anemia (AIHA) complicated by a follicular lymphoma (FL) in situ and other B-cell clones in the spleen. This diagnosis was made by immunohistochemistry, flow cytometry, and Southern blot analysis of the B-cell receptor. Chromosomal analysis revealed 46,XX,t(14;18)(q32;q21) 2/20, 46,XX,del(7)(q?),del(11)(q?) 2/20, and 46,XX 16/20. It has been speculated that these preneoplastic conditions do not progress to overt FL and other lymphomas without a second lymphomagenic insult. However, AIHA confers a 27.4-fold higher risk of such an insult leading to lymphoma compared with the normal healthy population. Without any therapy after splenectomy, our current study patient remained healthy with no lymphoma development for 28 months. Based on this case, we discuss the pathophysiology of lymphomagenesis in a spleen with AIHA and the roles of a splenectomy for preventing further lymphomagenesis in AIHA patients.
authors.affiliation: Department of Hematology, Shinmatsudo Central General Hospital, Chiba, Japan. Electronic address: kasimura@mvi.biglobe.ne.jp.
authors.forename: Makoto
authors.initials: M
authors.lastname: Kashimura
authors.affiliation: Department of Pathology, Dokkyo Medical University, Mibu, Japan.
authors.forename: Masaru
authors.initials: M
authors.lastname: Kojima
authors.affiliation: Department of Hematology, Shinmatsudo Central General Hospital, Chiba, Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Matsuyama
authors.affiliation: Department of Hematology, Shinmatsudo Central General Hospital, Chiba, Japan.
authors.forename: Jirou
authors.initials: J
authors.lastname: Tadokoro
mesh_terms: D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology / Q000821:virology; D001402:B-Lymphocytes / Q000473:pathology; D015139:Blotting, Southern; D005260:Female; D005434:Flow Cytometry; D006526:Hepatitis C / Q000150:complications* / Q000473:pathology / Q000503:physiopathology / Q000821:virology; D006801:Humans; D007150:Immunohistochemistry; D008224:Lymphoma, Follicular / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology / Q000821:virology; D013154:Spleen / Q000473:pathology; D013156:Splenectomy
keywords: Autoimmune hemolytic anemia; DAB.44; Follicular lymphoma in situ; Lymphomagenesis; Splenectomy