Primary cutaneous T-cell lymphoma of unspecified type with cytotoxic phenotype: clinicopathological analysis of 27 patients.
The objective of our study was to investigate the clinicopathological features of the currently ill-defined subtype of primary cutaneous T-cell lymphoma of unspecified type (CTCLU) with a cytotoxic phenotype and no Epstein-Barr virus (EBV) association. A series of 27 patients with CTCLU (median age 49 years; range 25-87 years; 18 men) was reviewed. Performance status scores above 1 (7%), clinical stages above 2 (15%), B symptoms (26%), extracutaneous involvement (30%), and a fatal course within 1 year of diagnosis (19%) were observed infrequently. The International Prognostic Index was high or high to intermediate in 11%, and the Prognostic Index for Peripheral T-cell Lymphoma unspecified was above group 2 in 22%. Notably, the rates of spontaneous regression and T-cell receptor gene rearrangements by polymerase chain reaction analysis were seen in 26 and 17% of our cases, respectively. Histologically, 22 patients had subcutaneous involvement of whom eight showed a lethal clinical course, and five patients without subcutaneous involvement were all survivors. Immunophenotypical and morphological features allowed us to subclassify our cases according to the following four categories: (1) epidermotropic CD8+ T-cell lymphoma (n=5); (2) cutaneous gamma/delta T-cell lymphoma (n=8); (3) cutaneous alpha/beta pleomorphic T-cell lymphoma (n=8); and (4) cutaneous medium/large pleomorphic T-cell lymphoma, not otherwise specified (n=6). All four of these groups of lymphomas exhibited a relatively favorable clinical course compared to previous reports. However, epidermotropic CD8+ T-cell lymphoma appeared to be unique with a higher ratio (80%) of spontaneous regression, a lower ratio (40%) of subcutaneous involvement, and a more favorable clinical course than the other three subcategories.
Hagiwara
Masahiro
M
Department of Dermatology, Nagoya Graduate School of Medicine, and Department of Pathology and Clinical Laboratories, Nagoya University Hospital, Showa-ku, Nagoya 466-8550, Japan. hgwrmshr@yahoo.co.jp
Takata
Katsuyoshi
K
Shimoyama
Yoshie
Y
Yamamoto
Kazuhito
K
Takahashi
Emiko
E
Asano
Naoko
N
Iwase
Yuko
Y
Okazaki
Yoshiko
Y
Tamada
Yasuhiko
Y
Yoshino
Tadashi
T
Tomita
Yasushi
Y
Nakamura
Shigeo
S
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006801:Humans; D016410:Lymphoma, T-Cell, Cutaneous / Q000188:drug therapy / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012878:Skin Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000473:pathology*; D015996:Survival Rate