Juvenile Myelomonocytic Leukemia

Question 1

Distinguish JMML from CML.

Answer 1

Absence of the BCR-BAL gene fusion.

Question 2

Leukocyte Adhesion Deficiency (LAD) is characterized by?

Answer 2

Absence of CD18 and subunits of CD11a, CD11b, and CD11c.

Question 3

GM-CSF was normal in the patient with JMML. how does this help rationalize the patient’s leukocytosis?

Answer 3

WBCs are hypersensitive to the stimulus GM-CSF

Question 4

Defined as lysis of cell releasing potassium, phosphate, and nucleic acids; causing increased amount of uric acid in the blood; and having the potential to occur spontaneously.

Answer 4

Tumor lysis syndrome (ALL of the above)

Question 5

Multiple treatment therapies exist for managing JMML, Which is identified as being most effective for patients who are not clinically stable?

Answer 5

hematopoietic stem cell transplant.

Question 6

Justify the diagnosis of uncompensated respiratory acidosis: pH, pCO2, and HCO3-

Answer 6

pH is acidic (Increased), pCO2 is increased, HCO3- no change (not corrected). (1, 2, and 4)