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Flashcards in Approach to Cytopenias Deck (54)
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1

What is/are common symptoms of pancytopenia?

A) mouth sores
B) fever
C) shortness of breath
D) All of the above

D) All of the above

mouth sores from low platelets causing purpura

2

What is the most common etiology of aplastic anemia?
A) Benzene
B) Chloramphenicol
C) Parvovirus
D) Idiopathic

D) Idiopathic

3

T or F. The mechanism that cause pancytopenia are bone marrow failure and destruction of blood cells in the peripheral blood

T.

4

Pancytopenia includes what?

- blood cells (anemia)
- white blood cells (leukopenia)
- platelets (thrombocytopenia)

5

What are the symptoms of anemia?

- dyspnea
- chest pain
- fatigue

6

What are the symptoms of leukopenia/neutropenia?

-fever
-infection
-mouth sores

7

What are the symptoms of thrombocytopenia?

bleeding

8

What are the two basic causes of pancytopenia?

- increased destruction
- decreased production

9

What are some possible causes of increased destruction leading to pancytopenia?

-immune destruction (ITP)
-sepsis
-hypersplenism

10

What are some possible causes of decreased production leading to pancytopenia?

-myelodysplasia
-marrow infiltrate
-B12 deficiency
-asplastic anemia
-drugs
-viruses (hep, HIV)
-radiation

11

What things could lead to a hypercellular bone marrow in someone who is pancytopenic?

-bone marrow infiltration (heme malignancies, carcinoma, B12/folate deficiency)

12

What things could lead to a hypocellular none marrow?

-aplastic anemia

13

What are congenital causes of aplastic anemia?

-Fanconi's anemia
-Familial aplastic anemia

14

What are some acquired causes of aplastic anemia?

-idiopathic (most common)
-drugs/chemical
-radiation
-viruses
-pregnancy (rare)
-PNH
-myelodysplastic syndrome

15

What does aplastic anemia result in?

Aplastic anemia is a severe, life threatening syndrome in which production of erythrocytes, WBCs, and platelets has failed (pperipheral ancytopenia)

16

What patient population is common for aplastic anemia?

all age groups and both genders

17

Asplastic anemia is characterized by what?

-peripheral pancytopenia
-hypocellular bone marrow

18

What is a primary mechanism of idiopathic aplastic anemia?

Immune mediated destruction of hematopoietic stem cells

19

What is the pathophysiology of aplastic anemia?

a reduction in or depletion of hematopoietic precursor stem cells which results in decreased production of all cell lines.

This is what leads to the peripheral pancytopenia

20

What could cause a reduction in or depletion of hematopoietic precursor stem cells with decreased production of all cell lines?

This may be due to quantitative or qualitative damage to the pluripotential stem cell

or the result of a defective bone marrow microenvironment (no differentiation cytokines present)

or from cellular or humoral immunosuppression of hematopoiesis.

21

When does Fanconi anemia become symptomatic? What is it associated with?

~5 y/o
-associated with progressive bone marrow hypoplasia

22

What congenital clinical manifestations are seen in Fanconi anemia?

skin hyperpigmentation and small stature

23

What is Familial aplastic anemia?

a subset of Fanconi’s anemia in which the obvious congenital defects are absent but bone marrow is present. These paitents can present at older ages.

24

T or F. Most cases of aplastic anemia are idiopathic and there is no history of exposure to substances known to be causative agents of the disease

T.

25

How does ionizing radiation cause aplastic anemia?


Exposure to ionizing radiation – hematopoietic cells are especially susceptible to ionizing radiation. Whole body radiation of 300-500 rads can completely wipe out the bone marrow. With sublethal doses, the bone marrow eventually recovers.

26

What chemicals can cause aplastic anemia?

- chemical agents with a benzene ring
- chemotherapeutic agents
- certain insecticides

27

What drugs can cause aplastic anemia?

chloramphenicol or quinacrine

These are Idiosyncratic reactions

28

What kinds of infections can cause aplastic anemia?

viral or bacterial
- infectious mononucleosis
- infectious hepatitis
- parvovirus/ CMV
- miliary TB

29

Lab findings with aplastic anemia?

-Severe pancytopenia with relative lymphocytosis (lymphocytes live a longer time)

-Normochromic/macrocytic, normocytic RBCs (may be slightly macrocytic)

-Mild to moderate anisocytosis and poikilocytosis

-Decreased reticulocyte count

-Hypocellular bone marrow with > 70% yellow marrow

30

What is/are treatment options for aplastic anemia?

withdrawal of potentially offending agents

supportive care (eg, transfusion, antibiotics),

immunosuppressive regimens

hematopoietic cell transplantation (HSCT)