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.
Subject(s)
Cryoglobulinemia/pathology , Intestinal Neoplasms/pathology , Intestine, Small/pathology , Lymphoma, B-Cell, Marginal Zone/pathology , Aged , Chromosome Aberrations , Cryoglobulinemia/complications , Cryoglobulinemia/metabolism , Cryoglobulins/metabolism , Cytogenetics , Fatal Outcome , Female , Humans , Immunoglobulin M/metabolism , Immunoglobulin kappa-Chains/metabolism , Intestinal Neoplasms/genetics , Intestinal Neoplasms/metabolism , Karyotyping , Lymphoma, B-Cell, Marginal Zone/genetics , Lymphoma, B-Cell, Marginal Zone/metabolism , Oncogene Proteins, Fusion/genetics , Oncogene Proteins, Fusion/metabolism , RNA, Messenger/metabolism , RNA, Neoplasm , Reverse Transcriptase Polymerase Chain ReactionABSTRACT
BACKGROUND: Anaplastic large cell lymphoma (ALCL) is a subtype of non-Hodgkin's lymphoma characterized by CD30 antigen-positive, large neoplastic cells. We describe a case of ALCL suggested by cytologic examination of the tumor cells obtained from bronchial scratch preparations. CASE: A 26-year-old woman had had a dry cough since November 1996. Chest radiography in May 1997 revealed an abnormal shadow in the mediastinum extending to the pulmonary hilar region. The patient was hospitalized in June 1997. Computed tomography revealed a neoplastic lesion in the anterior mediastinum invading the right lung. Transbronchial scratch cytology revealed large, atypical lymphoid cells expressing CD30 and CD3 on immunocytochemical examination. A transcutaneous mediastinal biopsy was performed and a diagnosis of ALCL made. CONCLUSION: Differentiation from Hodgkin's disease was the most difficult point in this case. Detailed cytologic observation and CD3-positive immunocytology led to the correct diagnosis. The cell transfer technique of Sherman et al was very useful for immunocytologic staining. Thus, transbronchial scratch cytology was an especially valuable and effective procedure in this case.
