Chronic Myelogenous Leukemia: Progression

You might also like

Download as docx, pdf, or txt
Download as docx, pdf, or txt
You are on page 1of 2

CHRONIC MYELOGENOUS LEUKEMIA  Philadelphia chromosome is reciprocal

translocation the long arms of


 Is a MPN arising from a single genetic chromosome 9 and 22 which produces
translocation in a pluripotential the BRC/ABL1 chimeric gene(produces
hemapoetic stem cell producing a clonal a 210-kD BRC/ABL fussion protein that
overproduction of myeloid cell line in a enhanced tyrosine kinase activity).
cell line resulting in a preponderance of
immature cells in the neutrophilic line Peripheral blood and bone marrow
CLINICAL FEATURES  There are dramatic morphologic
‣Infection changes in peripheral blood and bone
‣Anemia marrow that reflect the expansion of
‣Bleeding the granulocyte pool, particularly in
‣splenomegaly maturational stages.

INCIDENCE Other laboratory findings


 It occurs predominantly in 46-53 y/o.  Hyperuricemia and uricosuria maybe
 Slightly more common in males than associated with secondary gout(due to
females cell increased turnover), urinary uric
acid stones, uric acid nephropathy.
 Carried also a mortality rate of 1,5 per
100,000yearly prior to the development
of imatinib meslate(tyrosine kinase PROGRESSION
inhibitor that is a treatment for CML).  Most cases of this disease would
 eventually transform into leukemia and
Symptoms associated with clinical onset some patient proceed through a
 Fatigue intermediate metamorphosis or
 Decreased tolerance of exertion accelerated phase.
 Anorexia  Disease progression is accompanied
 Abdominal discomfort by:
 Weight loss  Increased in number of clinical
 Symptomatic splenic enlargement
symptoms
Cytogenetics of Philadelphia chromosome
 Adverse changes I laboratory values
 A unique chromosome that is present in  Poorer response to therapy than in
proliferating hematopoietic stem cells the chronic phase
and their progany in hematopoetic
stem cell must be identified to confirm  Blast crisis involve peripheral blood,
diagnosis. bone marrow, extramedullary
 it appears that Philadelphia tissues
chromosome is more frequently in
population exposed to ionizing  Based on acute leukemia definitions,
radiation. blast constitute more than 20% of
 Appearance of the Philadelphia total bone marrow cellularity, and
chromosome in donor cells after
the peripheral blood exhibits
allogeneic bone marrow transplantation
indicates a possibility of a transmissable
increased blast.
agent.
 Blast crisis leukemia usually is AML
or ALL, but origins from other
hematopoietic clonal cells are
possible.

 Granulocytic sarcoma is an
extramedullary growth that may
occur as lymphocytic or
myelogenous cell proliferations.

Related disease
 Several disease exist that are clinically
similar to CML but do not exhibit the
Philadelphia chromosome and express
only a few pseudo-gaucher cells.
 Chronic neutrophilic leukemia
manifest bone marrow, and
extramedullary infiltrative
pattern similar to CML
 Juvenile myelomonocytic
leukemia observed in children
younger 4 y/o and accompanied
by expansion in number of
monocyte, granulocyte,
immature granulocyte and
manifestation of
dyserythropoiesis.
 adult myelomonocytic leukemia

You might also like