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.
Division of Hematology, Vanderbilt University Medical Center, Nashville, TN 37232-2288.
J P
JP
Greer
K E
KE
Salhany
J B
JB
Cousar
J P
JP
Fields
L E
LE
King
S E
SE
Graber
J M
JM
Flexner
R S
RS
Stein
R D
RD
Collins
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