TY - JOUR
T1 - Immunoglobulin and T-cell receptor gene-gene rearrangement in pleural cavity-based T-cell rich B-cell lymphoma in an immunocompetent patient
AU - Potti, Anil
AU - Malik, Azhar Ali
AU - Ganti, Apar Kishor
AU - Koch, Michael
AU - Leitch, John
PY - 2002
Y1 - 2002
N2 - Body cavity-based lymphomas are fluid-based lymphomas that are not associated with a tumor mass or adenopathy which could explain the origin of the lymphomatous effusion. A distinct lymphoma that grows in the body cavity as a lymphomatous effusion in the absence of a tumor mass has been identified as a primary effusion lymphoma. This almost exclusively occurs in patients with acquired immunodeficiency syndrome (AIDS), who invariably have a history of Kaposi sarcoma. We report a rare case of a recurrent pleural effusion in an immunocompetent patient. There was no evidence of lymphadenopathy or an associated mass on computerized tomography of the chest, abdomen and pelvis. Serology for HIV, HHS-8, EBV and HTLV-1 were negative. Cytologic examination of the pleural fluid showed an elevated white cell count with 97% lymphocytes, mostly with T-cell markers. Bone marrow aspirate and biopsy were negative and bronchoscopy was unrevealing. Pleural biopsy was significant for >70% T-lymphocytes and some large atypical cells. Which had CD19, CD20 and weak bcl-2 positivity. Kappa and lambda light chains did not show distinct clonality. A preliminary diagnosis of T-cell rich B-cell lymphoma (TCRBCL) of the pleural cavity was made. The diagnosis was confirmed with DNA studies done on the pleural biopsy specimen using PCR and southern blot. Dual rearrangement of Ig heavy chain region and TCR-beta genes were identified. The patient responded to combination chemotherapy with cyclophosphamide, adriamycin, vincristine and prednisone. Our case is the first known case of pleural cavity-based TCRBCL and illustrates the role of gene rearrangement studies in such patients.
AB - Body cavity-based lymphomas are fluid-based lymphomas that are not associated with a tumor mass or adenopathy which could explain the origin of the lymphomatous effusion. A distinct lymphoma that grows in the body cavity as a lymphomatous effusion in the absence of a tumor mass has been identified as a primary effusion lymphoma. This almost exclusively occurs in patients with acquired immunodeficiency syndrome (AIDS), who invariably have a history of Kaposi sarcoma. We report a rare case of a recurrent pleural effusion in an immunocompetent patient. There was no evidence of lymphadenopathy or an associated mass on computerized tomography of the chest, abdomen and pelvis. Serology for HIV, HHS-8, EBV and HTLV-1 were negative. Cytologic examination of the pleural fluid showed an elevated white cell count with 97% lymphocytes, mostly with T-cell markers. Bone marrow aspirate and biopsy were negative and bronchoscopy was unrevealing. Pleural biopsy was significant for >70% T-lymphocytes and some large atypical cells. Which had CD19, CD20 and weak bcl-2 positivity. Kappa and lambda light chains did not show distinct clonality. A preliminary diagnosis of T-cell rich B-cell lymphoma (TCRBCL) of the pleural cavity was made. The diagnosis was confirmed with DNA studies done on the pleural biopsy specimen using PCR and southern blot. Dual rearrangement of Ig heavy chain region and TCR-beta genes were identified. The patient responded to combination chemotherapy with cyclophosphamide, adriamycin, vincristine and prednisone. Our case is the first known case of pleural cavity-based TCRBCL and illustrates the role of gene rearrangement studies in such patients.
KW - Ig rearrangement
KW - Pleural cavity lymphoma
KW - T-cell rich B-cell lymphoma
KW - TCR rearrangement
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U2 - 10.1080/10428190210172
DO - 10.1080/10428190210172
M3 - Article
C2 - 11908729
AN - SCOPUS:0036119628
SN - 1042-8194
VL - 43
SP - 195
EP - 198
JO - Leukemia and Lymphoma
JF - Leukemia and Lymphoma
IS - 1
ER -