Angioimmunoblastic T cell lymphoma with an unusual proliferation of Epstein-Barr virus-associated large B cells arising in a patient with progressive systemic sclerosis.
We report an unusual case of angioimmunoblastic T cell lymphoma arising in the setting of 5 years of immunosuppressive treatment for progressive systemic sclerosis. The lymph node lesion was accompanied by large blastic B cells with an association of Epstein-Barr virus. Southern blot study demonstrated the clonal rearrangement of T cell receptor beta-chain gene, but not of immunoglobulin heavy chain gene. Phenotypical examination of the lymph node also revealed the predominance of CD4+ T cells in addition to the proliferation of follicular dendritic cells, but no light chain restriction in large B cell components. In the clinical and laboratory aspects, neutrophilia (15.8 x 10(9)/l) and plasmacytosis (40%) in bone marrow were noted, which were considered to be closely related to elevated serum granulocyte colony-stimulating factor, interleukin (IL)-4 and IL-6. Based on the combined data described here, our preferred diagnosis was angioimmunoblastic T cell lymphoma with Epstein-Barr virus-associated B cell lymphoproliferative disorder, the pathogenesis of which was suggested to be closely associated with immunosuppressive treatment for progressive systemic sclerosis.
Yamamoto
Hidesuke
H
Division of Hematology, Department of Internal Medicine, Aichi Medical University, Nagakute, Japan. hidesuke@aichi-med-u.ac.jp
Miwa
Hiroshi
H
Kato
Yoshiro
Y
Nakamura
Shigeo
S
Hara
Kazuo
K
Nitta
Masakazu
M
D000368:Aged; D001402:B-Lymphocytes / Q000473:pathology / Q000821:virology; D015496:CD4-Positive T-Lymphocytes / Q000473:pathology / Q000821:virology; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000473:pathology* / Q000821:virology; D004854:Herpesvirus 4, Human; D006801:Humans; D007165:Immunosuppression Therapy / Q000009:adverse effects; D008198:Lymph Nodes / Q000473:pathology / Q000821:virology; D016393:Lymphoma, B-Cell / Q000150:complications / Q000473:pathology* / Q000821:virology; D016399:Lymphoma, T-Cell / Q000150:complications / Q000473:pathology* / Q000821:virology; D008297:Male; D045743:Scleroderma, Diffuse / Q000150:complications / Q000473:pathology* / Q000628:therapy; D016176:T-Lymphocyte Subsets / Q000473:pathology / Q000821:virology