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Author7 Posts
  #1

37 yr old male software enginner presents for diagnosis of elevated white cell count. He went to the unrgent care 15 days ago with complain of back pain, band like in nature and aggrevated by breathing. Pain was constant and dull in nature. X-ray, CT scan, Urine examination, CBC and CMP failed to reveal any infection, factrure, organomegaly but elevated white count were consistently seen. His count were in range of 27000 to 33000.

At present, his back pain has improved but still there. There is some fullness and tenderness in left upper abdominal region. A tip of spleen is felt on inspiration. Hepatomegaly is not appreciated. He does not have fever, weight loss or loss of appetite.He is healthy and athletic otherwise. His medical, social and drug history has no significant finding.

Q-1 What will be the best next step in management

A CBC with peripheral smear
B Leucocyte esterase test
C PET scan
D Bone marrow biopsy
E RT-PCR for bcr-able fusion gene
F FISH for philadelphia gene

Q-2 What will be the most specific diagnostic test

A CBC with peripheral smear
B Leucocyte esterase test
C PET scan
D Bone marrow biopsy
E RT-PCR for bcr-able fusion gene
F FISH for philadelphia gene

Q-3 What will be the drug of choice

A Gleevac
B Imatinib
C Hydroxyurea
D Watchful waiting
E Bone marrow transplant

___________________
Past is a history. Tomorrow is a mystery. What you have today is gift of God- that is why it is called present. Enjoy it...

  #2

I wll go with 1.A.....
2.with F
3.Watchful waiitng

I think,CBC will reveal a differential count--If there is increased neutrophils with a left shift suggetsing of CML,Then we can detect the presence of ph chromosome.Moreover,the patient has no symptoms and hence we can wait.

If the DC reveals increased neurophils and if there is a presence of ph chromosome,then we can do a BMT

  #3

1- A

2- F

3- E

  #4

D
F
B

  #5

1 - A
2 - E/F (I don't know what is more specific)
3 - A/B (the same drug) - untreated CML progresses to blast phase and earlier the treatment is started response is better. BMT, although curative in >90% cases (if appropriate donor is available) still carries high risk of TRM (transplant-related mortality) and is not the treatment of choice for newly diagnosed CML. It is loosing its importance even with the resistance to Gleevec (since new TKI have been discovered).

However, the vagueness/duality of answers make me wonder what the problem actually is.
It looks more like leukemoid reaction, but there are no features in his history that would favor it (hemorrhage, drugs, infections...).
I dunno!


  #6

50% of the patients suffering from CML presents without any symproms in routine check up and high white cell count should raise the flag. Abscence of fever and other related signs/symptoms, presence of splenomegaly should definately make one suspect leukemia highly.
So the best initial test would be CBC with PS- it will allows to look at the morphology of abnormal cells (promyelocytes/auer rods/lymphocyte/band cell/smudge cell/blasts) and help next step in the management of patient.

Most sensitive and specific test would RT-PCR for bcr-abl fusion. 15% of CML patient have gene rearrangement other then classic philadelphia gene. These patient group is best detected with RT-PCR

Best initial therapy would be Imatinib. BMT will be most curative and the patient of young age with otherwise best health is best candidate. But it is best to have patients in complete remission (cytologic and molecular) for best outcome. Therefore, imatinib is best initial therapy. Also finding, matching and harvesting the marrow/pbsc takes time.

___________________
Past is a history. Tomorrow is a mystery. What you have today is gift of God- that is why it is called present. Enjoy it...

  #7

Thanks!
The only q is is in 3rd q Gleevec misspelled on purpose (as Gleevac) to point us to Imatinib?
I considered it to be mistakenly misspelled and therefore didn't know what to choose.
If it is Gleevac in quiz, then I'd go with Imatinib.









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