Spontaneous loss of ph chromosome with maintenance of clonal hemopoiesis in an untreated patient with myeloproliferative disease and a long survival
✍ Scribed by Mario Luppi; Monica Morselli; Giovanni Emilia; Paola Temperani; Roberto Marasca; Patrizia Barozzi; Licia Selleri; Giuseppe Torelli
- Book ID
- 102221939
- Publisher
- John Wiley and Sons
- Year
- 1995
- Tongue
- English
- Weight
- 375 KB
- Volume
- 12
- Category
- Article
- ISSN
- 1045-2257
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✦ Synopsis
The unusual case of myeloproliferative disease described here is characterized by the following features: ( I) a clinically completely silent course for I I years without splenomegaly. marrow fibrosis, or cellular morphologic alterations; (2) the presence, at the onset, of a Philadelphia (Ph) chromosome without DNA breakpoints in the M-bcr region; (3) the spontaneous loss of detectable Ph-positive cells, 5 years after the first finding of leukocytosis, in the absence of any therapy; (4) the maintenance of the clonal nature of hematopoiesis, as revealed by the PGK X-linked inactivation pattern, in the absence of the Ph chromosome; arid (5) a biphasic trend in the levels of leukocytes, red cells, and platelets during the years of observation.