Clinical features associated with transformation of cerebriform T-cell lymphoma to a large cell process.
Some patients with cerebriform T-cell lymphoma (CTCL) undergo morphologic transformation to a large cell lymphoma. From a series of 113 patients with CTCL, 22 patients were identified with transformed CTCL. Stages of involvement at diagnosis were: I (seven), II (four), III (four), IV (seven). Nine patients had transformation at the initial diagnosis while the median time from diagnosis to transformation in the other 13 patients was 16 months (range: 3 months-6 years). Thirteen had transformation extracutaneously: lymph nodes (eight), central nervous system (two), and other extranodal sites (three). T cell markers were identified in all cases; of 15 cases with complete phenotypes, there were eight T-helper, three T-suppressor, and four aberrant T phenotypes. Serology for human T-leukemia virus-I (HTLV-I) was negative in eight patients tested. Median survival from diagnosis was 27 months compared to 53 months in 53 patients without transformation (p = 0.003). Despite combination chemotherapy in 12 patients following transformation, median survival after transformation was 12 months and only 7 months with extracutaneous disease. The likelihood of transformation could not be predicted by the initial histology, immunophenotype, or stage of disease.
Greer
J P
JP
Division of Hematology, Vanderbilt University Medical Center, Nashville, TN 37232-2288.
Salhany
K E
KE
Cousar
J B
JB
Fields
J P
JP
King
L E
LE
Graber
S E
SE
Flexner
J M
JM
Stein
R S
RS
Collins
R D
RD
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D002471:Cell Transformation, Neoplastic / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D006678:HIV / Q000302:isolation & purification; D015368:Human T-lymphotropic virus 1 / Q000302:isolation & purification; D006801:Humans; D007223:Infant; D008198:Lymph Nodes / Q000473:pathology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000276:immunology / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D016399:Lymphoma, T-Cell / Q000276:immunology / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D016410:Lymphoma, T-Cell, Cutaneous / Q000473:pathology; D008875:Middle Aged