malignant transformation of the stem cell, characterized by proliferation of the granulocytes which retain their capacity to differentiate.
therapeutical or accidental irradiated people(the median latent period seems to be 4 years in ankylosing spondylitis, 9 years in uterine cervical cancer and 11 years in Japanese atomic bombs survivors);
and CW4 has been found, suggesting thus a greater susceptibility to CGL. The disease is acquired, not inherited: children born from mothers with CGL are not affected, neither the identical twin of a patient with CGL ;
2. -the higher incidence appears in the fourth and fifth decade ; the CGL is uncommon in children and accounts for less than 5% of all childhood leukemias ;
the presence of the Ph-chromosome in the precursors of the erythropoietic, granulocytic and thrombocytic precursors, in the mono-macrophagic precursors well as (sometimes) in the lymphocytic ones;
studies on isoenzymes of the glucose-6-P-dehydrogenase and on the presence of Ph-chromosome in patients who are a mosaic for sex chromosomes, as in Turner or Klinefelter syndrome;
chromosome. It was recognized first as a small 22 chromosome and has been denominated thus after the city where it has been discovered. Later studies demonstrated that that the Philadelphia chromosome consists in a reciprocal translocationt(9,22)(q34;q11). The break- points appears on the long-arm of the 22-chromosome, named \u201cbreak point cluster region\u201d orbcr and in a region of the long arm of the chromosome 9, where thec- abl gene is located ( c-abl is the cellular homologue of the transforming oncogene of the Abelson murine leukemia virus). The remaining sequences of the bcr-region on the 22- chromosome act as an accepter for the c-abl gene and achimeric
chimeric RNA-messenger and consequently to a chimeric protein, namely a new 210 kD tyrosine-kinase, which has the capacity to activate cells. But the translocation phenomenon is reciprocal, so that a piece of chromosome 22 is translocated also to the chromosome 9.
-even in those patients where the Ph-chromosome could not been revealed by classical methods, the chimeric new gene bcr- abl can be detected by polymerase chain reaction
-it persists in all stages of the disease, but during the blast crisis, other chromosomal abnormalities associate (duble Ph, deletion of Y, trisomy 9, 19 or 21)
-the effects of bcr-abl on cell adhesion reveal the loss of blocking mitosis in clones Ph-positive chromosome, which is the hallmark of a proliferative advantage;
1. -the transformed hematopoietic stem cell generates an increasingly expanded pool of committed stem cells for granulocytic and, at least initially, megakaryocytic and erythroid cell lines;
peripheral blood , a continuously increasing number of neutrophils appears, as well as eosinophils and basophils; in earlier stages, because megakaryocytes are increased in bone marrow, a great number of platelets appears in peripheral blood;
in spleen, they proliferate profusely, growing out from the red pulp toward the white pulp, where progressively replace the normal lymphoid population ;
as the disease progresses, the leukemic cells proliferate also in lymph nodes and infiltrate diffusely or nodular other organs and systems, including the central nervous system (CNS);
no splenic enlargement (or a small and painless splenic enlargement only), neither liver or other organs or systems involvement ; the diagnosis could be suggested by fortuitous laboratory investigation and confirmed by specific tests (see later);
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