A 68 year old lady who has been experiencing fatigue for a couple of months comes to see you in the office. She has felt slightly depressed and mildly short of breath. She denies any diarrhea, vomiting, fever chills or rigors.

HENT exam reveals mild pallor, dental examination is normal, Chest is clear, cardiac exam reveals an ejection systolic murmur. Quality of pulse is normal.  Abdomen reveals fullness in the upper part and there is dullness in the peri-umbilical region and left upper quadrant.

Serum chemistry is normal, CBC shows Hb. 10.2, WBC 84,000/cumm, Predominantly neutrophils with altered nuclear morphology, Platelet count 104K/cumm.

Which of the following is NOT compatible with the patient's condition?

1. Chest X ray with indentation of the gastric bubble.

2. Philadelphia chromosome

3. Busulfan is the drug of choice

4. Gleevac (Imatinib) is commonly used to treat it.

5. Interferon is a helpful drug in such cases.

Answer is 3. This patient has CML and Hydroxyurea is the drug of choice not Busulfan. Although Busulfan is used to help, because of its cost advantage over hydroxyurea, Busulfan is not the DOC. Gleevac is contending to be the DOC on account of its effectivity and good side effect profile..

Management of CML ( Chronic myelocytic/granulocytic leukemia)

Explanation

CML/CGL is a leukemia with a relatively good prognosis and patients usually survive a long time without intervention. One should only intervene if patient has symptoms or a high white count (>50,000 and stop once count falls below 20,000).

The drug of choice is Hydroxyurea. Busulfan is an inexpensive alternative.

Bone marrow transplant in patients <50 is a chance for cure.

Interferon reduces the number of cells with the Philadelphia chromosome.

Beware of hyperviscosity which is due to sludging of cells. Treat it with leukapheresis/exchange transfusion.

Philadelphia chromosome is sometimes talked about and is a sign of good prognosis when present in CML.

On the other hand, if it is present in AML, it is a warning of bad outcome.

Many doctors have made me write about Gleevec (Imatinib) - a tyrosine kinase inhibitor that stops the formation of an abnormal tyrosine kinase that the Philadelphia chromosome produces. Thus these abnormal cells die. CML gets well controlled. I have not yet read about this being the drug of choice in CML.