Myeloproliferative Disorder

Question 1

High WBC count and absolute granulocytosis- YES
Blasts in peripheral blood- NO

Answer 1

non-malignant granulocytic abnormality

Question 2

High WBC count and absolute granulocytosis- YES
Blasts in peripheral blood- YES
Blasts <10% in blood and bone marrow- YES

Answer 2

Chronic Myeloproliferative disorder

Question 3

Chronic Myeloproliferative disorder

Answer 3

Chronic myelogenous leukemia
Idiopathic myelofibrosis
Polycythemia vera
Essential thrombocythemia

Question 4

High WBC count and absolute granulocytosis- YES
Blasts in peripheral blood- YES
Blasts >30% in bone marrow- YES

Answer 4

Acute Myeloproliferative disorder

Question 5

Acute Myeloproliferative disorder

Answer 5

Acute Myelogenous Leukemia

Question 6

Acute Myeloproliferative disorder

Answer 6

Acute Myelogenous Leukemia
AML-M1
AML-M2
AML-M3
AML-M4
AML-M5
AML-M6
AML-M7

Question 7

Polycythemica Vera

Answer 7

Defect in myeloid cell phase
Signs/Symptoms
-splenomegaly (big spleen) due to increased breakdown of RBCs
-hepatomegaly (big liver)
-gout= increase in uric acid
- blockage of vessels (santa claus face)
-histamine release due to breakdown of mast cells=puritis on bathing(itching)

Diagnosis
-increase in RBC, HCT, Baso, Neutro, PLTS
-bone marrow aspirate

Treatment
-phlebotomy (bloodletting)
-hydroxyurea- gout- decreases uric acid by preventing proerythroblast production
-interferon alpha- can cause gout due to proerythroblast destruction

Question 8

Essential Thrombocythemia

Answer 8

Defect before production of megakaryoblast (thrombocytes)
Signs/Symptoms
-Thromboses (clots)
plt plugs everywhere, leads to headaches, dizziness, weakness, strokes. hands and feet get no oxygenated blood, leads to numbness and burning.
-Bleeding- use up ingredients for clotting
nosebleeds and bruises
-Mega spleen (splenomegaly)

Diagnosis (of exclusion)
JAK2 Kinase
increase PLT (PLT >450,000 for months)
bone marrow aspirate
exclude other MPNS

Treatment
low risk-anti-coagulate (aspirin) common patients
high risk- hydroxyurea leads to decrease in production of megakaryoblasts.
emergencies- PLT pheresis

Question 9

Chronic Myelogenous Leukemia (CML)

Answer 9

Defect in the Myeloblast phase
type of tyrosine kinase that constantly tells cells to keep dividing- Philadelphia Chromosome
Signs/Symptoms
Chronic Phase
-abdominal fullness- big spleen/liver
-fever (increased wbc)- increased basal metabolism
Accelerated Phase
-increase in defected cell production
-bleeding (Petechiae=bleeding of vessels)
-bruises
-fever (opportunistic infections)
Blast Phase/Crisis
-rapid immature cell production
-bone pain, fever

Diagnosis
-increased WBC 50-200K (peripheral leukocytosis)
-FISH test %95 case of CML lights up to show Philadelphia Chromosome
-Bone marrow aspiration (increased myeloblasts)

Treatment
-Imatinib- stem cells transplant that blocks the BCR/ABL gene from communicating

Question 10

Primary Myelofibrosis

Answer 10

Defect in bone marrow
Bone marrow is replaced with connective tissue, which then cannot make stem cells for cell production.
Signs/Symptoms
-abdominal fullness
-bone pain
-bleeding due to decrease in PLTS (nosebleeds, bruises)
-pallor, dizziness
-opportunistic infections due to decrease in wbcs

Treatment
-replace fibrosis with stem cells
-transfusion to increase rbcs and plts
-splenectomy-surgical removal of the spleen, usually after injury, infection or tumor.

Diagnosis
- decrease in wbc and plts
-increase in immature wbc- tear drop rbc due to passing through fibrotic tissue
-BM biopsy=dry tap, shows fibrosis