Aashi
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A 42-year-old man comes to your office complaining of feeling full after eating only salad at dinner for the past three months. He has felt fatigued recently while gardening and going to the shopping mall. Physical examination reveals a mild pallor, clear lungs, no murmurs, and no cervical or axillary adenopathy. There is massive splenomegaly, and the liver edge is felt one inch below the right costal margin. There are no ecchymoses or petechiae. Laboratory studies show: WBC 140,000/mm3, with 82% neutrophils, 10% basophils, and no blasts; hemoglobin 10 mg/dL, hematocrit 30%, platelets 320,000/mm3. The peripheral smear shows a left-shifted myeloid series and bands. What treatment would you recommend first?
(A) Leukapheresis
(B) Hydroxyurea
(C) Imitanib (Gleevec) STI 571
(D) Autologous bone-marrow transplant
(E) Interferon-alpha
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| doc_clotaire
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Seems like CML
D ) BMT
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| CocaCola
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c -gleevec (imitinab)
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| mjl1717
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Glleevac initially -answer-c......... BMT is 2nd if no response to Gleevac 
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| fox
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this is cml.....A??
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| Aashi
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Answer:
(C) Imitanib (Gleevec) STI 571
Explanation:
The best initial therapy for chronic myelogenous leukemia is the oral tyrosine kinase inhibitor, imitanib. Although this drug does not cure the disease, it offers an excellent hematologic response and often eliminates the Philadelphia chromosome. The only reliably curative treatment for CML is allogeneic bone-marrow transplantation, not an autologous transplantation. The crucial factor in the success of a transplant is the availability of HLA-matched siblings for donation. The cure rate is 70 to 80% if done within one year of diagnosis (if HLA-matched) but only 40 to 60% if an HLA match is found through a registry of nonfamilial donors.
The success of a drug used for CML is assessed by determining who becomes Philadelphia chromosome negative. If there is a complete cytogenetic response, survival rates are 90%. Hydroxyurea was formerly the treatment for CML patients awaiting transplantation. Hydroxyurea does not convert anyone to the Philadelphia chromosome-negative state and is only used to lower the cell count. Interferon-alpha was used to prolong the chronic phase of CML and after prolonged therapy could remove the Philadelphia chromosome in a minority of patients (<20-30%). Interferon has significant side effects, however, such as fatigue, myalgias, and anorexia, and it requires motivated patients. Imitanib or Gleevec (STI571) is a new oral drug that inhibits the tyrosine kinase activity of the bcr-abl gene and has shown excellent efficacy. There is a hematologic response in 80 to 90% of patients, and as many as three-quarters of these become Philadelphia chromosome negative. Although it is infinitely less dangerous than an allogeneic transplant, imitanib does not offer the same chance at a permanent cure.
Leukapheresis is performed if the patient shows signs of leukostasis or the sludging of the white cells in the vasculature, causing confusion, blurry vision, dyspnea, and stroke. This patient has a very high cell count but does not have any of these symptoms.
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