chronic myeloid leukemia (CML)

Cml is acquired disease of hemopoietic stem cell which is characterized by

 1. Leukocytosis with granulocytic immaturities.( Increase no of leukocyte

 2. Splenomegaly

 3. Basophilia( many of them are basophils)

 4. Distinct chromosomal abnormalities (Philadelphia chromosome) 

•  Age. It occur after 50 years in about all cases 

• Pathogenesis. 

 In a normal person there are 23 pair of chromosome are present. In the pair No 9 and pair no 22 there are a translocation occur from which new gene form causing cancer. From pair no 9 ABL cut and attached with pair no 22 chromosome BCR and fused. Thus a new gene form BCR ABL . These new gene produce abnormal production of protein called tyrosine which is responsible for hemopoietic division. This tyrosine increase the production of abnormal no of leukocytes. BCRABL are the hallmark of cml

cml pathology

•  Phases. There are three phases 

1. Chronic phase.

 2. Accelerated phase

 3. Blast phase

 Chronic phase

             Less then 10 percent blast come to blood.

  Accelerated phase.

          10 to 19 percent blast come to blood.

 Blast phase.

        More then 20 percent blast come to the blood.

 What is blast

              Blast is the immature form of white blood cell which are not full matured and come to blood.

 Clinical features.

                 Due to bone marrow failure anemia occur which cause lethargy , dysphonia.

 Bleeding manifestation.

          Due to thrombocytopenia causing spontaneous bruised petechiae, bleeding from gums and other bleeding tendencies.

         Infections are more common.

 Symptoms due to hypermetabolism.

        Due to high metabolism such as weight loss ,anorexia and night sweating occur. 

 Splenomegaly occur due to which some time acute pain also occur due to spleen infarction.

 Diagnostic.

      CBC Blood picture( peripheral blood smear)

 Bone marrow examination

 Cytochemistry

 Blood picture or blood smear Anemia are normocytic or normochromic

 Thrombocytopenia less platelets 

Wbc More leukocyte approximately 2 lakh or more at the time of presentation Immature white blood cell Basophile Eosinophilia 

 In p smear garden party appearance will be occurs due immature blast.

garden party apearence

blood test cml

 Bone marrow examination.

 Hypercellurity

            There will be hypercellurity with total or partial replacement of fat spaces by proliferation of myeloid cells.

 Myeloid cell.

         The myeloid cell are predominant in the bone marrow with increase myeloid erythroid ratio. Erythropoiesis.

       Reduction in erythropoietic cells. Megakaryocytes. Megakaryocytes are normal but usually in smaller size then normal.

 Other investigation. Elevation in serum uric acid. 

 Treatment.

 Treatment for anemia is transfusion of blood and platelets.

 1. Imatinib therapy.

treatment cml

 

 2. Allogenic bone marrow transplant.

      But the most effective therapy is imatinib therapy which can increase survival rate to 90 percent.