CMPD - Polycythemia Vera

Question 1

CMPD classifications

Same disease but with different manifestations

Answer 1

Polycythemia vera (PV)
Chronic myelogenous leukemia (CML)
Agnogenic myeloid metaplasia (AMM)
Essential thrombocythemia (ET)
CHRONIC NEUTROPHILIC LEUKEMIA

Question 2

CMPD Cells involved include

Answer 2

mature and immature granulocytes, erythrocytes, and platelets

Question 3

Common manifestations

Answer 3

Splenomegaly
Cellular cytosis
Degrees of marrow fibrosis

Question 4

Characterized by PANMYELOSIS specifically red cells

Answer 4

POLYCYTHEMIA VERA

Question 5

POLYCYTHEMIA VERA

Answer 5

primary polycythemia

Question 6

POLYCYTHEMIA VERA

RBC count are usually

Answer 6

> 5.9 X 1012/L (women) and

6.6 X 1012/L (males) associated with an increase in plasma volume

Question 7

PV CLINICAL PRESENTATION

Mucous membranes have a

Answer 7

50-60 years old

headaches, vertigo, ringing of the ears, blurred vision, upper GI pain (peptic ulcers)
splenomegaly, pruritus

ruddy cyanotic (reddish purple) color

Hypertension
Hepatomegaly
Gout

Question 8

PV

LAB FINDINGS
PERIPHERAL BLOOD

Answer 8

Basophils are frequently elevated

Abnormal platelet forms may be present

No signs of marrow fibrosis

Decreased PF-3

Blood viscosity

Question 9

PV

LABORATORY FINDINGS
BONE MARROW

Answer 9

Hypercellular marrow with fat spaces filled with RBC

Increased megakaryocytes and normoblast

Abnormal and large megakaryocytes

Increased basophils = Increased histamine (itching)

Decreased to absent iron

Question 10

PV

LABORATORY FINDINGS
BONE MARROW

Minimal to absent fibrosis but tends to worsen with disease progression

Answer 10

Due to Megakaryocytic alpha granules releasing platelet derived growth factors

Question 11

PV

LABORATORY FINDINGS
CHEMISTRY

Answer 11

Normal to elevated values of serum B12
Increased B12 binding capacity
Decreased serum iron
Decreased ferritin
Decreased to normal serum erythropoietin

Question 12

CATEGORY A

Answer 12

Increased red cell mass
    Male >36mL/kg
    Female >32mL/kg
Normal arterial O2 saturation
     >92%
****Splenomegaly

Question 13

CATEGORY B

Answer 13

Thrombocytosis
     Platelets >400 X 109/L
Leukocytosis
     WBC > 12 X 109/L
      \+/- fever or infection
Increased LAP (>100) with no fever or infection
Increased serum B12
     >900ng/mL
Increased unbound B12 binding capacity
     >2200 ng/mL
**no splenomegaly

Question 14

increased erythropoietin

Answer 14

SECONDARY POLYCYTHEMIAS

Question 15

associated with tissue hypoxia

Answer 15

COMPENSATORY ERYTHROCYTOSES

Question 16

High altitudes, CVD or pulmonary diseases, abnormal hemoglobins with increased oxygen affinity, and heavy smoking

Answer 16

INCREASED ERYTROPOIETIN

Question 17

kidney diseases and tumors, liver CA, ovarian tumors, uterine fibroids, cerebellar hemangioblastomas

Answer 17

INAPPROPRIATE ERYTHROPOIESIS

Question 18

not associated with true increases in RCM and is

measured by the microhematocrit

Answer 18

RELATIVE POLYCYTHEMIA

Question 19

RELATIVE POLYCYTHEMIA elevation

Answer 19

Elevation is due to decrease in plasma volume

Dehydration and burns

Question 20

drug of choice

Answer 20

HYDROXYUREA

Question 21

TX Reduce platelets and rbcs

Answer 21

Radioactive phosphorus (32P)

Question 22

TX Myelosupprerssive drugs

Answer 22

(Busulfan and chlorambucil)