3.24.14* Goorha - Bone Marrow Failure

Question 1

Symptoms of anemia including

Answer 1

difficulty breathing, chest pain and fatigue.

Question 2

Symptoms of leukopenia/neutropenia include

Answer 2

fever, infection and mouth sores.

Question 3

causes of pancytopenia by increased destruction

Answer 3

immune destruction
sepsis
hyperpsplenism

Question 4

Causes of pancytopenia by decreased production

Answer 4

myelodysplasia
marrow infiltrate
B12 deficiency
aplastic anemia
drugs
viruses
radiation

Question 5

causes of hypercellular marrow

Answer 5

bone marrow infiltration
hematologic malignancies (leukemia, myeloma, lymphoma)
storage disorders
myelodysplastic syndromes
B12 or folate deficiency

Question 6

causes of hypercellular marrow

Answer 6

aplastic anemia

a. congenital (fanconi’s anemia)
b. aquired (idiopathic, myelodysplastic syndrome, drugs/chemicals, radiation, viruses)

Question 7

Aplastic anemia

Answer 7

characterized by peripheral pancytopenia and hypocellular bone marrow.

• This may be due to quantitative or qualitative damage to the pluripotential stem cell
• or the result of a defective bone marrow microenvironment
• or from cellular or humoral immunosuppression of hematopoiesis.

Question 8

What is a primary mechanism of idiopathic aplastic anemia?

a. Infiltration of bone marrow by abnormal protein
b. Immune mediated destruction of hematopoietic stem cells
c. Immune mediated destruction of peripheral blood cells
d. Vitamin deficiency

Answer 8

b

Question 9

Fanconi’s anemia

Answer 9

the disorder usually becomes symptomatic ~5 years of age and is associated with progressive bone marrow hypoplasia. Congenital defects such as skin hyperpigmentation and small stature are also seen in affected individuals.

Question 10

Infections that can lead to aplastic anemia

Answer 10

viral and bacterial infections such as infectious mononucleosis, infectious hepatitis, parvovirus and cytomegalovirus infections, and miliary tuberculosis occasionally lead to aplastic anemia

Question 11

Lab findings in aplastic anemia

Answer 11

a. Severe pancytopenia with relative lymphocytosis (lymphocytes live a long time)
b. Normochromic, normocytic RBCs (may be slightly macrocytic)
c. Mild to moderate anisocytosis and poikilocytosis
d. Decreased reticulocyte count
Hypocellular bone marrow with > 70% yellow marrow

Question 12

What is/are treatment option(s) for aplastic anemia?

Answer 12

Immunosuppression (this suggests auto-immune destruction is present in idopathic cases)
Stem cell transplant
Transfusion

Question 13

Pure red cell aplasia

Answer 13

is characterized by a selective decrease in erythroid precursor cells in the bone marrow. WBCs and platlets are unaffected

Question 14

Causes of pure red cell aplasia

Answer 14

Acquired

a. Transitory with viral or bacterial infections
b. Patients with hemolytic anemias may suddenly halt erythropoiesis
c. Patients with thymoma – T-cell mediated responses against bone marrow erythroblasts or erythropoietin are sometimes produced

Treatment is supportive care and immunosupression

Question 15

What is the primary difference between myelodysplastic syndrome and aplastic anemia?

a. Presence or absence of pancytopenia
b. Presence or absence of impaired hematopoesis
c. Presence or absence of neoplastic cells in the bone marrow
d. Spleen size

Answer 15

c

Question 16

Myelodyspatic syndromes

Answer 16

a. primary, neoplastic stem cell disorders that tend to progress to acute leukemia (>20% blasts)
b. normocellular or hypercellular BM with dysplastic changes resulting ineffective erythropoiesis and or granulopoiesis and or megakaryopoiesis
c. increased level of apoptotic cell death in bone marrow cells
d. increase in blasts

Question 17

What is seen in PBS or MS

Answer 17

dysplastic (abnormality in development) cells including nucleated RBCs, oval macrocytes, pseudo-Pelger-Huet PMNs (hyposegmented neutrophils) with hyperchromatin clumping, hypogranulated neutrophils, and giant bizarre platelets.

Question 18

What is seen in the bone marrow of patient with myelodysplastic syndrome?

Answer 18

Normo- or hypo-cellular

Ringed sideroblasts

Question 19

What is the treatment for myelodysplastic syndrome?

Answer 19

Epo
transfusion if needed
some chemo
(only trying to prevent progression to AML)

Question 20

Lenalidomide

Answer 20

causes tumor cell apoptosis by inhibiting stromal cell support.
Used in myelodysplastic syndrome and multiple myeloma.

Question 21

autologous v. allogeneic BMT

Answer 21

in general think autologous for lymphoma and multiple myeloma

allogeneic for leukemia and myelodysplastic syndrome, aplastic anemia, some lymphomas

Question 22

18 year old African American male presented to Methodist Hospital with complaint of fatigue and ‘passing out’
On presentation, wbc = 0.8, hgb = 5.5, platelets = 40,000
No family history of blood problems
Exam was unremarkable
Reticulocyte count

Answer 22

aplastic anemia

Question 23

treatment for aplastic anemia

Answer 23

Anti-thymocyte globulin, steroids, and cyclosporine

Question 24

What do you do for an aplastic anemia patient who does not respond to drugs?

Answer 24

allogeneic BMT