3.24.14* Goorha - Bone Marrow Failure Flashcards Preview

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Flashcards in 3.24.14* Goorha - Bone Marrow Failure Deck (24):
1

Symptoms of anemia including

difficulty breathing, chest pain and fatigue.

2

Symptoms of leukopenia/neutropenia include

fever, infection and mouth sores.

3

causes of pancytopenia by increased destruction

immune destruction
sepsis
hyperpsplenism

4

Causes of pancytopenia by decreased production

myelodysplasia
marrow infiltrate
B12 deficiency
aplastic anemia
drugs
viruses
radiation

5

causes of hypercellular marrow

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

6

causes of hypercellular marrow

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

7

Aplastic anemia

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.

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

b

9

Fanconi’s anemia

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.

10

Infections that can lead to aplastic anemia

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

11

Lab findings in aplastic anemia

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

12

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

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

13

Pure red cell aplasia

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

14

Causes of pure red cell aplasia

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

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

c

16

Myelodyspatic syndromes

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

17

What is seen in PBS or MS

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.

18

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

Normo- or hypo-cellular
Ringed sideroblasts

19

What is the treatment for myelodysplastic syndrome?

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

20

Lenalidomide

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

21

autologous v. allogeneic BMT

in general think autologous for lymphoma and multiple myeloma

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

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

aplastic anemia

23

treatment for aplastic anemia

Anti-thymocyte globulin, steroids, and cyclosporine

24

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

allogeneic BMT