[Cutaneous localization of T-cell prolymphocytic leukemia]

Ann Dermatol Venereol. 1995;122(8):526-9.
[Article in French]

Abstract

Introduction: 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.

Case report: 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.

Discussion: 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:

Publication types

  • Case Reports
  • English Abstract
  • Review

MeSH terms

  • Aged
  • Aged, 80 and over
  • Humans
  • Leukemia, Prolymphocytic* / genetics
  • Leukemia, Prolymphocytic* / pathology
  • Leukemia, T-Cell* / genetics
  • Leukemia, T-Cell* / pathology
  • Male
  • Prognosis
  • Skin / pathology
  • Skin Neoplasms* / genetics
  • Skin Neoplasms* / pathology