Bone Marrow Disorders

Question 1

Pure red cell aplasia (PRCA)

Answer 1

Severe reduction in the number of reticulocytes in the peripheral blood and the virtual absence of erythroid absence.
The bone marrow ceases to produce RBC’s

Question 2

PRCA causes

Answer 2

Autoimmune disease
Thymoma
Viral infxns
Large granular lymphocyte leukemia
Idiopathic
congenital

Question 3

PRCA dx

Answer 3

Very low retic count and anemia when the following are present:

• normocytic, normochromic anemia
• Absolute retic < 10,000
• Normal WBC and platelet count
• Normal marrow, except no erythroid precursors

Question 4

PRCA Tx

Answer 4

Red cell transfusions
Removal of cause
Usually relapses

Question 5

Myelophthisic Anemia (MA)

Answer 5

Severe anemia caused by the displacement of hematopoetic bone marrow into peripheral blood.

Question 6

MA Causes

Answer 6

Chronic myeloproliferative disease
Leukemia
Lymphoma
Metastatic carcinoma lymphopma

Question 7

MA Dx

Answer 7

Tear drop RBC’s
Immature granulocytes
Treat underlying cause/cancer

Question 8

Myelodysplastic Syndrome (MDS)

Answer 8

Heterogenous group of stem cell disorders characterized by dysplastic and ineffective blood cell production.
Dysplasia of one or more cell lines
AKA pre-leukemia

Question 9

MDS patients have a reduction in which blood cells?

Answer 9

Normal RBC’s
Platelets
Granulocytes

Question 10

MDS leads to?

Answer 10

Anemia
Bleeding
Increased risk for infxn

Question 11

Who is affected by MDS?

Answer 11

Elderly (60 - 80)

Double the incidence of AML

Question 12

Transformation of MDS to acute leukemia occurs in ___% of patients.

Answer 12

40%

Question 13

Manifestations of MDS

Answer 13

Anemia (macro, normo)
Oval macrocytes
Neutropenia
Thrombocytopenia
Dysplastic features

Question 14

Blasts account for ___ than 20% in MDS.

Answer 14

Less than 20%. If greater, consider Acute Myeloid Leukemia

Question 15

MDS Tx

Answer 15

Bone marrow transplant. It is risky the older you get.

Question 16

Aplastic anemia (AA)

Answer 16

Severe anemia characterized by pancytopenia accompanied by hypocellular bone marow

Question 17

Aplastic causes

Answer 17

Direct stem cell injury (meds)
Autoimmune
SLE (rare)

Question 18

How many cases of aplastic anemia are idiopathic?

Answer 18

70%

Question 19

AA symptoms

Answer 19

Pallor
Mucousal bleeding
ecchymoses
infxns
gingivitis

Question 20

AA lab findings

Answer 20

Pancytopenia
normo, normo anemia
Low retic
Empty fatty bone marrow
Few hematopoetic cells

Question 21

AA Tx

Answer 21

Bone marrow transplant
Immunosuppression
Transfusions if needed
Withdraw etiological agent if found

Question 22

Fanconi Anemia (FA)

Answer 22

Genetic disease, 1 in 350,000 births.
Bone marrow failure by 40
Development of AML
Usually diagnoses by 8 yrs

Question 23

Is FA the most common cause of inherited bone marrow failure?

Answer 23

Yes.

Question 24

FA Dx

Answer 24

Chromosome fragility test (MMC, DEB)

Question 25

FA Tx

Answer 25

Transfusions
Androgens
Growth factors
Bone marrow transplant

Question 26

Allogenic

Answer 26

Comes from another person

30 - 50% will die

Question 27

Syngenic

Answer 27

Comes from an identical twin

Question 28

Autologous

Answer 28

Comes from the patient

<5% will die

Question 29

MUD

Answer 29

Matched unrelated donors

Question 30

MRD

Answer 30

Matched related donors

25% chance of being a match

Question 31

CD34+

Answer 31

Identification for stem cells