title: Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) arising in the small intestine with monoclonal cryoglobulinemia.
abstract: A case of small intestinal extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) with monoclonal cryoglobulinemia is described. The patient was a woman in her mid-sixties with purpura of the bilateral lower legs and abdominal pain. An immunoserological investigation showed expression of IgM-kappa type monoclonal cryoglobulin. A renal biopsy specimen revealed proliferative glomerulonephritis with cryoglobulin deposition. Physical examination disclosed a stenosis, edematous changes and ascariasis in the small intestine. In aspiration cytology of the ascites, proliferation of the atypical lymphoid cells with plasmacytoid differentiation was observed. These cells were positive for B-lineage antigens in immunocytochemistry, and showed an immunoglobulin heavy-chain gene rearrangement in Southern blotting and chromosomal alteration in G-banded karyotype analysis. Although medicinal treatment was used, the patient died of general prostration. The diagnosis of intestinal MALT lymphoma was made at autopsy. Expression of API2-MALT1 fusion transcripts was detected by reverse transcription-polymerase chain reaction analysis using formalin-fixed, paraffin-embedded tissue. Intestinal MALT lymphomas with API2-MALT1 expression have distinctive forms of infiltration compared with those without translocation. Therefore, detection of API2-MALT1 fusion transcripts is useful for evaluating the prognosis and clinical behavior of the disease.
authors.affiliation: Department of Pathology, Narita Red Cross Hospital, Chiba, Japan. saito.chu923@j-wave.net
authors.forename: Tadashi
authors.initials: T
authors.lastname: Saito
authors.forename: Jun-ichi
authors.initials: J
authors.lastname: Tamaru
authors.forename: Hirohisa
authors.initials: H
authors.lastname: Kishi
authors.forename: Junko
authors.initials: J
authors.lastname: Kayao
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Kuzuu
authors.forename: Hisashi
authors.initials: H
authors.lastname: Wakita
authors.forename: Akiko
authors.initials: A
authors.lastname: Adachi
authors.forename: Shinji
authors.initials: S
authors.lastname: Itoyama
authors.forename: Ittaku
authors.initials: I
authors.lastname: Mikata
mesh_terms: D000368:Aged; D002869:Chromosome Aberrations; D003449:Cryoglobulinemia / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D003450:Cryoglobulins / Q000378:metabolism; D003582:Cytogenetics; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000378:metabolism; D007145:Immunoglobulin kappa-Chains / Q000378:metabolism; D007414:Intestinal Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D007421:Intestine, Small / Q000473:pathology*; D007621:Karyotyping; D018442:Lymphoma, B-Cell, Marginal Zone / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D015514:Oncogene Proteins, Fusion / Q000235:genetics / Q000378:metabolism; D012333:RNA, Messenger / Q000378:metabolism; D012334:RNA, Neoplasm; D020133:Reverse Transcriptase Polymerase Chain Reaction
chemical_list: C415943:API2-MALT1 fusion protein, human; D003450:Cryoglobulins; D007075:Immunoglobulin M; D007145:Immunoglobulin kappa-Chains; D015514:Oncogene Proteins, Fusion; D012333:RNA, Messenger; D012334:RNA, Neoplasm