If there are symptoms in the early stages of CML, they develop gradually and are usually mild. They tend to be non-specific and can easily be confused with the symptoms of more common illnesses, such as flu. If you have any of these symptoms, it's important to see your doctor, but remember they are common to many illnesses other than CML. At The Christie, leukaemia s and other blood cancers are treated by the haematology and transplant unit. Chronic myeloid leukaemia CML. Taking out the spleen may improve red blood cell and platelet counts in some patients.
Most people have no problem living without a spleen, but the risk for certain bacterial infections is increased. This is why doctors often recommend certain vaccines be given before the spleen is removed. A splenectomy may help improve blood counts and relieve pressure and mild pain from an enlarged spleen that's pressing on nearby tissues and organs.
A splenectomy might be advised if:. Your spleen is so swollen that it's pushing on other organs, such as your stomach. Other treatments, such as chemotherapy or radiation therapy, are often used first to try to shrink the spleen. Your spleen is filtering out too many red blood cells and platelets from your blood. It's your spleen's job to remove worn-out blood cells.
But leukemia can make your spleen overactive. A splenectomy can help raise your red blood cell and platelet counts. You may need some vaccines before surgery to remove your spleen. What every physician needs to know. MPNs are stem cell-derived clonal disorders with proliferation of one or more of the components of myeloid lineage. CML patients carry a fusion gene called breakpoint cluster region-abelson BCR-ABL fusion gene derived from a balanced translocation between the long arms of chromosome 9 and 22; t 9;22 q34;q11 also known as Philadelphia Ph chromosome.
This fusion gene encodes a protein product with strong tyrosine kinase catalytic activity, and is leukemogenic. This deregulated tyrosine kinase is implicated in the pathogenesis of CML. Bone marrow and peripheral blood with Ph chromosome. Conventional cytogenetics miss approx. FISH can identify unusual variant rearrangements. White blood cell WBC count is usually controlled with medication. Recent studies suggest single measurements of BCR-ABL transcripts at 3 months is a good way to predict those patients who will do poorly.
Survival is months for older patients and slightly longer for younger patients. A typical patient with CML is a male in his fourth and fifth decade of life median age of 50 that usually presents in the chronic phase of the disease.
During the chronic phase, symptomatic patients complain of non-specific symptoms such as loss of energy, left upper quadrant pain, early satiety and decreased exercise tolerance. Most of these symptoms are related to an enlarged spleen and liver during this phase. Priapism is another frequently encountered symptom secondary to hyperviscosity due to increased circulating leukocytes. During the accelerated phase, myelofibrosis increases, anemia is worse and splenomegaly is not controllable by medication.
During the blast phase patients present with easy bruising, bleeding, massive splenomegaly and prominent constitutional symptoms. Skin and tissue infiltration is very common in this phase. Chronic neutrophilic leukemia — patients could present with hepatosplenomegaly due to granulocytic infiltration.
However, usually there is toxic granulation in neutrophils, high leukocyte alkaline phosphatase LAP score, and no Ph-chromosomes. Other myeloproliferative disease polycythemia vera, essential thrombocythemia or primary myelofibrosis — bone marrow evaluation is usually helpful. CML shows small megakaryocytes while others have large atypical megakaryocytes.
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