Bone marrow disorders

Question 1

2 classes of bone marrow disorders

Answer 1

1. bone marrow failure

2. myleoproliferative neoplasms

Question 2

2 types of failure

Answer 2

1. aplastic anemia

2. myelodysplastic syndrome

Question 3

3 types of myleoproliferative disorders

Answer 3

1. polycythemia vera
2. essential thrombocythemia
3. primary myelofibrosis

Question 4

what is aplastic anemia

Answer 4

• loss of stem cells
• pancytopenia
• mostly children and young adults

Question 5

causes of aplastic anemia

Answer 5

• autoimmune most common

- toxic (chemo, rads)

Question 6

presentation of aplastic anemia (5)

Answer 6

1. pancytopeina
2. progressive over weeks to months
3. anemia - fatigue
4. leukopenia - infections
5. thromobpenia - bruising

Question 7

how to treat aplastic anemia (2)

Answer 7

1. regen. stem cells - immunosuprese: ATG

2. replace stem cells - transplant

Question 8

what is myleodysplastic syndrome (MDS)

Answer 8

• clonal stem cell disorder
• takes over marrow
• progeny are defective
• cytopenia

Question 9

presentation of MDS

Answer 9

• cytoenias - slow and progressive

- anemia most common

Question 10

2 tests for MDS

Answer 10

1. blood film
2. bone marrow aspirate
   - blast count
   - cytogenetics
   - iron stain

Question 11

what are 2 risk stratifications of MDS

Answer 11

some stay stable and chronic, some rapid to AML

Question 12

what is treatment for high and low risk MDS

Answer 12

low - fix cytopenia to ammeliorte Sx
high - delay or prevent AML
- azitothiprizine
- transplant

Question 13

what are features of myeloproliferative neoplasms

Answer 13

• clonal stem cell disorders
• overproduce on lineage
• may enter fibrotic phase
• older patients

Question 14

what is polycythemia vera

Answer 14

• too much RBC

Question 15

Sx of poly vera

Answer 15

think blood leads to

• H/A
• visual dist.
• bleeding
• pruritis
• red
• splenomegaly
• thrombosis

Question 16

what is mech of most vera

Answer 16

Jak2 self activates without epo and get unrestrained erethropoisis
- see mech

Question 17

4 treats of vera

Answer 17

1. reduce RBCs
2. phlebotomy
3. oral chemo
4. Jak2 inhib

Question 18

what is essential thrombocytopenia

Answer 18

too much platelelts

Question 19

Sx of ET

Answer 19

• usually asymtomiatic
• may bleed
• thrombosis
• may spelnomegaly

Question 20

what is ET mech

Answer 20

similar to Vera, but instead of EPO, JAK2 without TPO

Question 21

treatment of ET

Answer 21

asymptomiatic - observe
if thrombosis 
- ASA
- hydroxyurea
if bleeding
- hydroxurea

Question 22

what is primary myelofibrosis

Answer 22

• marrow replaced by fibrosis
• extramedularry hematopoesis
• early: increaed bloods
• late: decreased

Question 23

3 diagnositic tests

Answer 23

1. marrow and blood - dry tap!
2. JAK2 in 75%
3. CALR in 25%

Question 24

4 treatments

Answer 24

1. transfusions
2. splenectomy - risky
3. stem cell transplant - can be cure
4. JAK2 inhib.

Question 25

see cases

Answer 25

if you will