[Cutaneous localization of T-cell prolymphocytic leukemia].
BACKGROUND
T cell prolymphocytic leukemia (T PLL) is a rare variant of mature lympho-proliferative disorder. The main physical sign is a gross splenic enlargement contrasting with no enlargement of lymph nodes. Skin involvement is found in 30 p. 100 cases. Twenty-one cases of cutaneous lesions of PLL have been reported, mainly with T PLL, only 2 cases of B PLL: Clinical lesions are polymorphous; histology shows a dermal prolymphocytic infiltrate. The main cytogenetic abnormalities are: translocation (14; 14) (q11; q32), inversion of chromosome 14 (q11; q32), isochromosome 8q.


METHODS
We report a case of an 87-year-old patient presenting a T cell prolymphocytic leukemia CD4+ with specific papular lesions of the back. Electron microscopy showed typical prolymphocytes and cytogenetic studies showed a tendency to polyploidy, with the lost of chromosome 14, translocation 8-22 and inversion of chromosome 13. After 12 months of treatment with a combination of chloraminophen and prednisone the patient was in partial remission and the cutaneous lesions disappeared.


CONCLUSIONS
This case is rare and the patient has an unusual long survival (mean survival is 7 months). Contrary to the other hematologic disorders, cutaneous involvement does not change the prognosis of T PLL:
Thomas
A
A
Service de Dermatologie, CHU, Caen.
Dompmartin
A
A
Troussard
X
X
Moreau
A
A
Manard
C
C
Leroy
D
D
D000368:Aged; D000369:Aged, 80 and over; D006801:Humans; D015463:Leukemia, Prolymphocytic / Q000235:genetics / Q000473:pathology; D015458:Leukemia, T-Cell / Q000235:genetics / Q000473:pathology; D008297:Male; D011379:Prognosis; D012867:Skin / Q000473:pathology; D012878:Skin Neoplasms / Q000235:genetics / Q000473:pathology
Localisation cutanée de la leucémie prolymphocytaire T.