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{{WBRQuestion
{{WBRQuestion
|QuestionAuthor={{Rim}}
|QuestionAuthor={{Rim}}, {{AJL}} {{Alison}}
|ExamType=USMLE Step 1
|ExamType=USMLE Step 1
|MainCategory=Pathophysiology
|MainCategory=Pathophysiology
Line 20: Line 20:
|MainCategory=Pathophysiology
|MainCategory=Pathophysiology
|SubCategory=Oncology
|SubCategory=Oncology
|Prompt=A 36 year old woman presents to her primary care physician with complaints of fatigue, loss of energy and decreased exercise tolerance. She had noticed she wasn't feeling completely normal for the past 3 to 4 months but points out the past 3 weeks have been very unusual for her. Physical exam is mostly unremarkable except for minor splenomegaly. Lab ordered are shown below. Peripheral blood smear stains very low to absent for leukocyte alkaline phosphatase. Which of the following treatments is most likely to be considered in this patient?
|Prompt=A 36-year-old female presents to the physician's office with complaints of fatigue, loss of energy, and decreased exercise tolerance. She explains that over the past 3-4 months, these symptoms have became increasingly intolerable. Upon physical examination, you note minor splenomegaly. The results from the ordered lab tests are illustrated below. Peripheral blood smear stains demonstrate deficient leukocyte alkaline phosphatase levels. Which of the following treatments is most likely administered to this patient?


Hb = 9<br>
Hb = 9<br>
Line 29: Line 29:
%N = 96<br>
%N = 96<br>
%L = 2<br>
%L = 2<br>
|Explanation=Chronic myelogenous/myeloid leukemia is a myeloproliferative disorder accounting for 20% of adult leukemias that is characterized by the increased and unregulated growth of myeloid cells (neutrophils, eosinophils and basophils) without significant impairment of their differentiating ability. It is classically associated with a chromosomal translocation called the Philadelphia chromosome where a reciprocal translocation between chromosome 9 and 22 occurs, and is specifically designated as t(9;22). The result of the translocation is the bcr-abl gene fusion coding for an oncogenic tyrosine kinase. CML is usually an insidious disease that is often detected incidentally. It is characterized by very low to absent leukocyte alkaline phosphatase staining of peripheral blood and bone marrow aspirates. In 2001, the first tyrosine kinase inhibitor, imatinib, was introduced. It acted by blocking the effects of the bcr-abl gene product. The introduction of tyrosine kinase inhibitors has increased survival rate dramatically. Other drugs similar to imatinib include dasatinib, nilotinib, and bosutinib.


|Explanation=Chronic myelogenous/myeloid [[leukemia]] (CML), a myeloproliferative disorder accounting for 20% of adult [[leukemia]]s, is characterized by the increased and unregulated growth of myeloid cells (neutrophils, eosinophils, and basophils), without significant impairment of their differentiating ability. CML is frequently associated with a chromosomal translocation, which is referred to as the Philadelphia chromosome, where a reciprocal translocation between chromosome 9 and 22 occurs (specifically designated as t(9;22)). The translocation results in the bcr-abl genes fusion coding for an oncogenic tyrosine kinase. CML is usually an insidious disease, which is detected incidentally. CML is characterized by low leukocyte alkaline phosphatase staining of peripheral blood and bone marrow aspirates. The tyrosine kinase inhibitor, imatinib, acts by blocking the effects of the bcr-abl gene product. The introduction of tyrosine kinase inhibitors, such as imatinib, asatinib, nilotinib, and bosutinib, increased survival rates dramatically.


Educational objective: CML is characterized by very low to absent leukocyte alkaline phosphatase staining and treatment of choice is with tyrosine kinase inhibitors such as dastinib and imatinib.
|EducationalObjectives= CML, characterized by very low leukocyte alkaline phosphatase staining, is frequently treated with tyrosine kinase inhibitors, such as dastinib and imatinib.
|References= Kantarjian HM, Talpaz M. Chronic myelogenous leukemia. Hematol Oncol Clin N Am. Jun 2004;18(3):XV-XVI.


References:<br>
Kantarjian HM, Talpaz M. Chronic myelogenous leukemia. Hematol Oncol Clin N Am. Jun 2004;18(3):XV-XVI.
|AnswerA=Paclitaxel
|AnswerA=Paclitaxel
|AnswerAExp=Paclitaxel is a microtubule stabilizer. It is not usually used in the treatment of CML especially in the chronic-phase.
|AnswerAExp=Paclitaxel, not usually used in the treatment of CML,  is a microtubule stabilizer.
|AnswerB=Cytarabine
|AnswerB=Cytarabine
|AnswerBExp=Cytarabine is pyrimidine analog. It is not usually used in the treatment of CML especially in the chronic-phase.
|AnswerBExp=Cytarabine, not usually used in the treatment of CML, is pyrimidine analog.
|AnswerC=Methotrexate
|AnswerC=Methotrexate
|AnswerCExp=Methotrexate is a folic acid analogue. It is not usually used in the treatment of CML especially in the chronic-phase.
|AnswerCExp=Methotrexate, not usually used in the treatment of CML, is a folic acid analogue.
|AnswerD=Cisplatin
|AnswerD=Cisplatin
|AnswerDExp=Cisplatin is an agent that causes DNa cross-linking. It is not usually used in the treatment of CML especially in the chronic-phase.
|AnswerDExp=Cisplatin, not usually used in the treatment of CML, is an agent that causes DNa cross-linking.
|AnswerE=Dasatinib
|AnswerE=Dasatinib
|AnswerEExp=Dasatinib is a tyrosine kinase inhibitor used to treat CML.
|AnswerEExp=Dasatinib is a tyrosine kinase inhibitor frequently used to treat CML.
|RightAnswer=E
|RightAnswer=E
|WBRKeyword=CML, Chronic myelogenous leukemia, Imatinib, bcr-abl, Dasatinib
|WBRKeyword=CML, Chronic myelogenous leukemia, Imatinib, bcr-abl, Dasatinib, DOC, medicine, carcinoma, leukemia, cancer, incidental finding, enzymes, protien
|Approved=No
|Approved=Yes
}}
}}

Revision as of 15:28, 29 July 2014

 
Author [[PageAuthor::Rim Halaby, M.D. [1], Alison Leibowitz [2] (Reviewed by Alison Leibowitz)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Pathophysiology
Sub Category SubCategory::Oncology
Prompt [[Prompt::A 36-year-old female presents to the physician's office with complaints of fatigue, loss of energy, and decreased exercise tolerance. She explains that over the past 3-4 months, these symptoms have became increasingly intolerable. Upon physical examination, you note minor splenomegaly. The results from the ordered lab tests are illustrated below. Peripheral blood smear stains demonstrate deficient leukocyte alkaline phosphatase levels. Which of the following treatments is most likely administered to this patient?

Hb = 9
Hct = 26.8
MCV = 82
Plt = 120000
WBCs = 24000
%N = 96
%L = 2
]]

Answer A AnswerA::Paclitaxel
Answer A Explanation AnswerAExp::Paclitaxel, not usually used in the treatment of CML, is a microtubule stabilizer.
Answer B AnswerB::Cytarabine
Answer B Explanation AnswerBExp::Cytarabine, not usually used in the treatment of CML, is pyrimidine analog.
Answer C AnswerC::Methotrexate
Answer C Explanation AnswerCExp::Methotrexate, not usually used in the treatment of CML, is a folic acid analogue.
Answer D AnswerD::Cisplatin
Answer D Explanation AnswerDExp::Cisplatin, not usually used in the treatment of CML, is an agent that causes DNa cross-linking.
Answer E AnswerE::Dasatinib
Answer E Explanation AnswerEExp::Dasatinib is a tyrosine kinase inhibitor frequently used to treat CML.
Right Answer RightAnswer::E
Explanation [[Explanation::Chronic myelogenous/myeloid leukemia (CML), a myeloproliferative disorder accounting for 20% of adult leukemias, is characterized by the increased and unregulated growth of myeloid cells (neutrophils, eosinophils, and basophils), without significant impairment of their differentiating ability. CML is frequently associated with a chromosomal translocation, which is referred to as the Philadelphia chromosome, where a reciprocal translocation between chromosome 9 and 22 occurs (specifically designated as t(9;22)). The translocation results in the bcr-abl genes fusion coding for an oncogenic tyrosine kinase. CML is usually an insidious disease, which is detected incidentally. CML is characterized by low leukocyte alkaline phosphatase staining of peripheral blood and bone marrow aspirates. The tyrosine kinase inhibitor, imatinib, acts by blocking the effects of the bcr-abl gene product. The introduction of tyrosine kinase inhibitors, such as imatinib, asatinib, nilotinib, and bosutinib, increased survival rates dramatically.

Educational Objective: CML, characterized by very low leukocyte alkaline phosphatase staining, is frequently treated with tyrosine kinase inhibitors, such as dastinib and imatinib.
References: Kantarjian HM, Talpaz M. Chronic myelogenous leukemia. Hematol Oncol Clin N Am. Jun 2004;18(3):XV-XVI.]]

Approved Approved::Yes
Keyword WBRKeyword::CML, WBRKeyword::Chronic myelogenous leukemia, WBRKeyword::Imatinib, WBRKeyword::bcr-abl, WBRKeyword::Dasatinib, WBRKeyword::DOC, WBRKeyword::medicine, WBRKeyword::carcinoma, WBRKeyword::leukemia, WBRKeyword::cancer, WBRKeyword::incidental finding, WBRKeyword::enzymes, WBRKeyword::protien
Linked Question Linked::
Order in Linked Questions LinkedOrder::