[Clinicomorphological characteristics of Sezary's disease and mycosis fungoides].
OBJECTIVE
To try a combined approach to the study of clinicomorphological and immunophenotypical characteristics of primary cutaneous T-cell lymphomas.


METHODS
Clinical, histological, genotypic and immunophenotypical parameters were studied in 7 patients (4 male and 3 female, mean age 53.1 +/- 7.8%) with Sezary's disease (SD) and 10 patients (6 male, 4 female, mean age 54.0 +/- 4.0 years) with mycosis fungoides (MF) treated in Hematological Research Center in 1998-2004.


RESULTS
Six of seven SD patients had SD stage IV with leukemization, Sezary's cells were found in peripheral blood. Bone marrow and lymph nodes involvement was observed in 5 patients. Morphological signs of transformation into lymphosarcoma were detected in three patients. Skin samples of all the patients showed epidermotropism with lymphoid infiltration of the derma and skin appendages. All the patients had clonal rearrangement of T-cell receptor by gamma-chain. Immunophenotyping (IPT) detected T-cell markers CD45RO, CD43, CD3, CD4 on lymphoid cells. IPT of peripheral blood lymphoid cells was typical for SD in 3 patients. Low density of CD4 and CD2, CD4 and CD5, the presence of CD7 were registered in 1 patient each. The disease history was 3.4 +/- 0.7 years. A lethal outcome was related with septic complications after polychemotherapy. MF history in 10 patients was 10.9 +/- 2.1 years. Stages III and IV were diagnosed in 2 of 10 patients. All the patients had typical pathohistological changes. Polymerase chain reaction test detected clone by rearrangement of gamma-chain of T-cell receptor. In 2 patients IPT detected CD4 absence in the presence of CD8 and CD7. The aberrant clone typical for NK-cells was detected in one case. Two patients died of the disease progression after 7 and 20 years of MF.


CONCLUSIONS
Multiple tests help early diagnosis and conduction of optimal therapy for cutaneous T-cell lymphomas.
Vinogradova
Iu E
IuE
Giliazitdinova
E A
EA
Zamulaeva
I A
IA
Zybunova
E E
EE
Kaplanskaia
I B
IB
Kravchenko
S K
SK
Kremenetskaia
A M
AM
Lutsenko
I N
IN
Samoĭlova
R S
RS
Selivanova
E N
EN
Sidorova
Iu V
IuV
Shklovskiĭ-Kord
N E
NE
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005500:Follow-Up Studies; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / Q000276:immunology; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000150:complications* / Q000276:immunology / Q000473:pathology*; D009367:Neoplasm Staging; D012189:Retrospective Studies; D012751:Sezary Syndrome / Q000097:blood / Q000175:diagnosis / Q000235:genetics / Q000276:immunology / Q000401:mortality / Q000473:pathology*