Listed below is the best description of my disease that I have found to date.
I have increased the white cell production, my blasts were above 6% and I was tx dependent for 4 months( 2 units weekly).
I began VIDAZA over 18 months ago with injections on 4 week cycles. After about 6 months I went to 6 week cycles and am currently on my 3rd 8 week cycle.
I have both myeloproliferative and myelodysplastic diseases.
Currently my blasts are less that 1%, I have been tx independent for 15 months and have survived 33 months which is well past the 24 month median mentioned below.
I was told by my NP at Moffitt Cancer Center that VIDAZA has been found to prevent reticulin fibrosis, which is an unexpected plus.
I played 4 rounds of golf last week, not very good rounds, but I played.
If your symptons match mine and your DR. has not mentioned VIDAZA, ask him about it.
Good Luck to all,
JulesH
Clinics and Pathology
Disease The defining features of CMML are an absolute monocytosis of >1X109/l, increased numbers of monocytes in bone marrow, and a variable degree of dyplasia in all three lineages. Myeloblasts and promonocytes comprise less than 5% of nucleated cells in peripheral blood and less than 20% of cells in bone marrow. Roughly half of patients present with an elevated white cell count that is commonly associated with hepatomegaly and splenomegaly, the so-called myeloproliferative form of the disease. Patients lacking these features are generally considered to have the myelodysplastic form of the disease.
Phenotype / cell stem origin Presumably a multipotential stem cell.
Epidemiology CMML is usually a disease of older adults althought the disease also occurs in children, where distinction from juvenile chronic myelogenous leukemia (JCML, also known as juvenile myelomonocytic leuekmia) is difficult, and at times impossible. In one series the median age for the myelodysplastic type was 70 and for the myeloproliferative form 72 years. The disease shows a distinct male predominance of 1.6-2.1:1.
Clinics CMML patients may present with hepatomegaly or splenomegaly, serous effusions or other sites of extramedullary involvement such as skin or, less commonly, gums.
Cytology Blood: The peripheral blood count may be increased or decreased. By definition monocytes and promonocytes exceed 1X109 /l. Dyplastic monocytes or neutrophils are usually evident. Normocytic or macrocytic anemia is common as is thrombocytopenia, which is usually mild. Serum lysozyme is usually elevated and immunoglobulin elevations occur in about a third of patients, with monoclonal gammopathy in about 5-10% of patients.
Bone marrow: The bone marrow is almost always hypercellular, often with trilineage dysplasia. Myeloblasts and monoblasts are less than 20% of the total cellularity. About 30% of cases show significant reticulin fibrosis. Cases associated with the t(5;12) (see below) are commonly associated with eosinophilia.
Treatment Although CMML often initially respond to conventional chemotherapy, complete responses are rare. Allogeneic bone marrow transplantation is potentially curative for those patients who are eligible.
Prognosis The median survival for patients with CMML is about 24 months. There does not appear to be a difference in survival between patients with the myeloproliferative versus the myelodysplastic forms of the disease nor are the number of blasts of prognostic value. Of adult patients who underwent allogeneic bone marrow transplantation the disease-free survival was 39% at 3 years.
.
Hope you continue to do well.
