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Hematalogy Lecture > Test 4: Hypoproliferative anemias > Flashcards

Test 4: Hypoproliferative anemias Flashcards

1
Q

What are the mechanisms of bone marrow failure?

A

-destruction of stem cells due to drugs, chemicals, radiation, viruses, or autoimmune mechanisms
-Premature apoptosis of stem cells due to mutations
-Ineffective Hematopoiesis due to B12 or folate deficiencies
-Disruption of marrow microenvironment
-decreased production of growth factors
-Loss of normal hematopoietic tissue due to infiltration of marrow space with abnormal cells

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2
Q

This marrow disorder is characterized by a reduction in the number or function of multipotential stem cells (CD 34) 

A

Aplastic anemia

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3
Q

Characteristic feature of aplastic anemia

A

Pancytopenia

Also, ridiculo cytopenia, bone marrow hypocellularity, and depletion of hematopoietic stem cells (Increase fat cell infiltration) 

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4
Q

Is aplastic anemia acquired or inherited? 

A

-most common is acquired (95%)
(Idiopathic)
-Inherited accounts for 30% of cases in children

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5
Q

Is acute or chronic aplastic anemia more common?

A

Acute (rapidly fatal) is most common

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6
Q

Aplastic anemia is most frequent in what age range?

A

Individuals 10 to 25 years and those over 60

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7
Q

Diagnosis of aplastic anemia is made when two of the three peripheral blood values are found. What are the three?

A

-WBC less than 500 cells
-Platelet count less than 20×10 to the 3rd µL
-Retic count less than one percent

(Pancytopenia definition)

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8
Q

How does the bone marrow appear in aplastic anemia?

A

Few cells, lots of fat

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9
Q

Acquired aplastic anemia can be further classified as ______________ When cause is unknown and ____________ When cause is known

A

Idiopathic 70%, Secondary

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10
Q

Secondary causes of aplastic anemia are usually related to what?

A

Drug related. Benzene, sulfa drugs, penicillin, tetracycline and chemotherapeutic agents

Can be reversible or irreversible
90% of cases are due to idiosyncratic reaction to drug/chemicals

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11
Q

What are the clinical characteristics of acquired aplastic anemia?

A

-Bleeding from thrombocytopenia
-Increase susceptibility to infection due to leukopenia
-All the symptoms of anemia
-Absence of splenomegaly
-Iron overload from repeated transfusions and decreased need of iron for erythropoiesis

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12
Q

What is the pathophysiology of acquired aplastic anemia?

A

Due to a qualitative and quantitative deficiency of the hematopoietic stem and progenitor cell population by a direct or indirect mechanism

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13
Q

What is the pathophysiology of a quired aplastic anemia that is direct?

A

A cytotoxic drugs, chemical, radiation, or virus damages the DNA of stem and progenitor cells

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14
Q

What is the pathophysiology of acquired aplastic anemia that is indirect?

A

-Exposure to certain drugs or chemicals that produces an auto immune T cell attack that destroys the stem and progenitor cells
-Elevated levels of growth factor in serum including EPO (Despite this, growth factors are unsuccessful in correcting the cytopenia’s)
-failure of cell growth could result from excessive suppression of hematopoiesis byT lymphs or macrophages

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15
Q

A type of primary inherited aplastic anemia, congenital problem originates within bone marrow itself as an autosomal recessive disorder

A

Inherited aplastic anemia Fancini’s syndrome 

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16
Q

Caused by mutation in one of seven different genes
Presents with skin pigmentation, short stature, hypogonadism, malformation of fingers, toes, and organs

A

Inherited aplastic anemia Fancini’s syndrome

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17
Q

Characterized by major chromosomal abnormalities Like breaks, gas, and Jean rearrangement
Peripheral blood abnormality show up in early childhood: Anemia, neutropenia, thrombocytopenia

A

Inherited aplastic anemia Fancini’s syndrome

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18
Q

What is the prognosis for Inherited aplastic anemia Fancini’s syndrome ?

A

Prognosis is still very poor, even with cytokines and androgen in therapy

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19
Q

Inherited aplastic anemia Fancini’s syndrome:

Death usually occurs from…

A

Overwhelming hemorrhage or infection before age 30 (This is the most common cause of death in any aplastic anemia)

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20
Q

What is a commonly use therapy for  Inherited aplastic anemia Fancini’s syndrome?

A

Bone marrow transplant commonly used however patient still pre-disposed to other malignancies due to widespread chromosomal damage. 

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21
Q

What are the peripheral blood findings for aplastic anemia?

A

• Anemic, neutropenic, AND thrombocytopenic
• Normocytic, normochromic anemia (or macrocytic)
• Decreased retic count and RPI
No immature cells present*
• Predominant WBC is lymphocyte (“lymph”)
• Toxic granulation of neutrophils may be observed

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22
Q

Other disorders that cause pancytopenia, such as leukemia, are associated with the presence of many…..

A

Immature cells

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23
Q

It is normal to see toxic granulation in…..

A

Infections

(Darker and more pronounced granules)

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24
Q

In aplastic anemia, serum iron and percent transferrin saturation are ___________.

A

Increased due to decreased use of iron for erythropoiesis

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25
Q

Bone marrow for aplastic anemia is….

A

Hypocellular or is a dry tap

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26
Q

Approximately 30% of patients with aplastic anemia develop…

A

Paroxysmal Nocturnal Hemoglobinuria
(PNH)

  • sensitive to complement
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27
Q

Paroxysmal Nocturnal Hemoglobinuria
(PNH):

Acquired stem cell mutation results in the hemolysis of RBCs by _____________

A

Complement.

• PNH erythrocytes are deficient in membrane regulators of
complement (PIGA gene), and are subject to chronic hemolysis in
vivo
• Disease characterized by attacks of intravascular hemolysis and
hemoglobinuria that occur chiefly at night while the patient is
asleep.

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28
Q

Paroxysmal Nocturnal Hemoglobinuria
(PNH):

Flow cytometry shows decreased ______ and ________.

A

CD55 and CD59

29
Q

Paroxysmal Nocturnal Hemoglobinuria
(PNH):

Positive _________ test (acidified serum lysis) Complement-Dependent Hemolysis. 

A

Ham

30
Q

What is the treatment for aplastic anemia?

A

Best treatment is to treat the symptoms, and if possible, find the cause of suppression
Blood transfusions given as needed
Long-term survival in patients who receive stem cell transplant from HLA identical sibling

31
Q

What is the most common peripheral blood findings in severe aplastic anemia?

A

Macrocytosis, thrombopenia & neutropenia

32
Q

A rare disorder characterized by a selective and severe disease in erythrocyte precursors and otherwise normal bone marrow

A

Pure red cell aplasia (PRCA) 

33
Q

Pure red cell aplasia (PRCA): 
Can be Acquired or congenital, But most common is…

A

Acquired due to acute or chronic illness

34
Q

Pure red cell aplasia (PRCA):
Patients present with…

A

Severe anemia, reticulocytopenia, and normal WBC and platelet count

35
Q

Extremely rare, inherited disorder involving EPO resistant stem cells of unknown cause

A

Diamond-Blackfan anemia 

36
Q

What is the morphology in Diamond-Blackfan anemia?

A

Normocytic (Or macrocytic), Normochromic anemia, reticulocytopenia, but normal wbc and playlets 

37
Q

What hemoglobin is increased in  Diamond-Blackfan anemia ?

A

Hemoglobin F**

Poor prognosis, Half of cases Present with physical abnormalities

38
Q

Diamond-Blackfan anemia:
Bone marrow biopsy is cellular, but with marked decrease in…

A

Erythrocytes (Normal myeloid cells and megakaryocytes) 

39
Q

Half of Diamond-Blackfan anemia pts present with what abnormalities?

A

physical abnormalities

40
Q

Diamond-Blackfan Anemia will have increased hgb ____.

A

F

41
Q

How common is Acquired Pure Red Cell Aplasia?

A

-rare
-occurs more in adults than children

42
Q

Is Acquired Pure Red cell Aplasia acute or chronic?

A

could be either

-May be idiopathic or autoimmune related

43
Q

Acquired Pure Red Cell Aplasia:

> 50% cases occur in patients with concurrent
_________.

A

thymoma (cancer cells in thymus)

44
Q

Acquired Pure Red Cell Aplasia:

characterized as ____-, ______- anemia, but WBCs and plt. counts are normal.

A

normo-, normo-

45
Q

Acquired Pure Red Cell Aplasia:

Bone marrow shows mostly normal cellularity,
but with severe decrease in which precursors?

A

erythroblasts

46
Q

Acquired Pure Red Cell Aplasia:

When found in children, it is referred to as…

A

transient erythroblastopenia of childhood

-Patients have history of viral infection

47
Q

Acquired Pure Red Cell Aplasia:

Anemia is normocytic and Hgb-F levels are ________.

A

Normal

48
Q

What is the standard therapy for Acquired Pure Red Cell Aplasia?

A

 Transfusions
 Disorder is usually transient

49
Q

Congenital Dyserythropoietic Anemias:

-Refractory (not responsive to therapy)
-Can cause _______ nuclei

A

multiple

50
Q

Group of rare disorders characterized by
refractory anemia (unresponsive to therapy),
reticulocytopenia, and hypercellular bone
marrow with markedly ineffective erythropoiesis

A

Congenital Dyserythropoietic Anemias

51
Q

Congenital Dyserythropoietic Anemias:

True or false? Megaloblastoid features are not due to vitamin
B12 or folate deficiency (gene expression)

A

True

52
Q

Characteristically include multinucleated forms and
internuclear chromatin bridges or nuclear strands between two erythroblasts (nuclear bridging)

A

Congenital Dyserythropoietic Anemias

53
Q

Diamond-Blackfan anemia differs from
inherited aplastic anemia in that in the former:

A. Retics are increased
B. Hb F is decreased
C. Only erythropoiesis is affected
D. Congenital malformations are absent

A

C. Only erythropoiesis is affected

54
Q

The infiltration of abnormal cells into the bone marrow and subsequent destruction and replacement of normal hematopoietic cells.

A

Myelophthisic Anemia

55
Q

Myelophthisic Anemia:

Premature release of immature cells from bone
marrow and _________ hematopoietic sites occur.

A

extra-medullary

56
Q

Myelophthisic Anemia is common in…

A

pts. with metastatic carcinoma (“space-occupying lesions”) or in leukemia/lymphoma
-Degree of anemia correlates with tumor burden

57
Q

Lab results with Myelophthisic Anemia?

A

Normocytic, normochromic erythrocytes,
reticulocytosis, with varying degrees of maturation found in peripheral blood.

58
Q

What can be seen on blood smear with Myelophthisic anemia?

A

Teardrop erythrocytes*, nRBCs, and immature myeloid cells, megakaryocyte fragments, and giant platelets
are seen on smear

59
Q

How is Myelophthisic Anemia distinguished from Aplastic Anemia?

A

by teardrop
RBCs, a leukoerythroblastic blood picture, and abnormal cells in the bone marrow

60
Q

Anemia that occurs in patients w/ end-stage renal disease

A

Anemia of Chronic Renal Insufficiency
(Anemia of Chronic Renal Disease)

61
Q

Anemia of Chronic Renal Insufficiency:

There is a general correlation between the
severity of the anemia and the degree of
elevation of the ______>

A

BUN

62
Q

Anemia of Chronic Renal Insufficiency:

When BUN is > 100
mg/dL, the Hct is usually < ____%

A

30

63
Q

What is the primary cause of Anemia of Chronic Renal Insufficiency?

A

decreased production of EPO

64
Q

What causes increased hct with Anemia of Chronic Renal Insufficiency?

A

-decreased plasma volume (due to decreased [plasma protein] & thus tissue edema)
-increased due to relative erythrocytosis – but this may be counterbalanced by the absolute anemia due to the lack of EPO!

65
Q

What morphology is associated with Anemia of Chronic Renal Insufficiency?

A

 Normo- normo-, with normal or decreased reticulocytes
 Burr cells (renal disease), and shistocytosis

66
Q

Anemia of Chronic Renal Insufficiency:

Generally shortened RBC survival; uremia causes RBC p.m.
defects such as?

A

Burr cells

-Uremia also causes decreased plt. function, & resulting chronic bleeds =
more anemia!

67
Q

Anemia of Chronic Renal Insufficiency:

What lab values correlate with the severity of uremia?

A

increased BUN & creatinine

68
Q

What is the treatment for Anemia of Chronic Renal Insufficiency?

A

 Recombinant erythropoietin injections are effective
 Iron therapy given if levels are too low for recombinant EPO to
be effective

Hematalogy Lecture (35 decks)

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