Anemia Flashcards

1
Q

What is anemia?

A

A decrease in hemoglobin (Hgb),
hematocrit (Hct),
OR red blood cell count (RBC)

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2
Q

Which of the following patient lab profiles IS NOT consistent with ANEMIA?
A. Increased RBC; decreased Hgb; decreased MCV; normal HCT
B. Normal RBC; decreased Hgb; decreased MCV; normal HCT
C. Increased RBC; normal Hgb; decreased MCV; normal HCT
D. Decreased RBC; normal Hgb; increased MCV; decreased HCT
E. Decreased RBC; increased Hgb; normal MCV; decreased HCT

A

c

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3
Q

What are causes of anemia?

A

• Diminished production of red cell precursors by bone marrow
• Impaired production of mature red cells by bone marrow red cell precursors
• Decreased production of hemoglobin
• Increased destruction of red cells in circulation
(Hemolysis)
• Acute or chronic blood loss (Bleeding)
• A combination of the causes listed above

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4
Q

Describe normal erythropoiesis

A
  1. kidney senses hypoxia (anemia) and increases EPO production
  2. EPO act on E progeniotr cells in bone marrow to produce new RBCs
  3. kidney senses increased tissue oxygenation
  4. kidney decreases EPO production
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5
Q

What are causes of congenital non-hemolytic non blood loss anemia?

A

• Decreased red cell production
– Bone marrow erythroid hypoplasia (Decreased erythroid precursors in bone marrow)
– Impaired red cell production by bone marrow (Increased red cell precursors in bone marrow without red cells (reticulocytes) getting into circulation)
• Decreased hemoglobin production
– Decreased production of globin(s)
– Decreased production of heme

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6
Q

What are the most common causes congenital non hemolytic anemia (or ineffective bone marrow leading to decresaed RBC production)?

A

Bone marrow erythroid hypoplasia

  1. Diamond Blackfan anemia - mutation in gene coding for ribosomal protein (Chr 19)
  2. Fanconi Anemia
    – One or more mutations in genes coding for DNA repair
    proteins
    – DEB Test
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7
Q

What are causes of impaired red cell production by bone marrow?

A

congenital megaloblastic anemias

– All are characterized by impaired DNA synthesis without impairment of protein synthesis
» Lesch-Nyhan Syndrome
» Homocysteinuria
» Orotic aciduria
» ?Congenital dyserythropoietic anemias?

**The key thing with the congenital megaloblastic anemias (the point of this slide) is to understand that in these anemias you can make protein but not DNA, so the RBCs get really big. Each one of the causes of megaloblastic anemia ultimately has this problem at its base.

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8
Q

What forms Hgb?

A
FOUR globin (2a, 2b)
FOUR heme
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9
Q

What leads to decresaed production of globin?

A

Thalassemias and some hemoglobinopathies

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10
Q

What leads to decraesed production of heme?

A

• Some porphyrias
– Impaired porphyrin production
• Congenital sideroblastic anemias
– Lack enzyme to incorporate iron into porphyrin to make heme

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11
Q

What are hte MCC of acquired bone marrow erythroid hypoplasia?

A
•  Toxins
•  Radiation
•  Infections
–  Mainly viral (Parvovirus, CMV, EBV)
•  Autoimmune
–  Includes altered immune regulation associated with T-
cell neoplasms and thymoma
•  Acquired mutations
•  Relative erythroid hypoplasia caused by displacement (such as non-myeloid neoplasms)
•  Idiosyncratic
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12
Q

What are examples of acquired ineffective bone marrow production of mature red cells?

A

• Megaloblastic anemia
– Drugs or toxins that impair DNA synthesis
– Vitamin B12 or folate deficiency
• Myeloid neoplasms

While these causes produce an ADEQUATE number of cells these cells ARE NOT EFFECTIVE. Drugs like alcohol, methotrexate, AEDs, etc can all cause this. Further, AML and CML mimics drug-related causes. This is because both of them have so build up of precursor cells in the bone marrow that eventually die.

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13
Q

What causes decreased heme production?

A
  1. decreased Fe uptake by nucleated red cells (IDA and anemia of chronic dz)
  2. deficient Fe incorporated into porphyrin (lead, ethanol)
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14
Q

What is the MCC of sideroblastic anemia?

A

ETOH

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15
Q

What do you need to turn protoporphyrin into heme?

A

B6 and Cu

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16
Q

Why is sideroblastic anemia named?

A

Fe builds up in the mitochondria and can’t get inside the nucleus

17
Q

**Which of the following IS NOT associated with bone marrow erythroid hypoplasia (decreased red cell precursors in bone marrow)?
A. Fanconi anemia
B. Diamond-Blackfan syndrome C. Vitamin B12 deficiency
D. Radiation toxicity
E. Viral infection

A

c

18
Q

What labs/tests are used to evaluate anemia?

A
•  Automated CBC
•  Reticulocyte count
•  Blood smear morphology
•  Serum and plasma chemistries
•  Bone marrow biopsy morphology
–  Bone marrow cytogenetic, immunophenotypic and molecular diagnostic analysis
19
Q

What labs indicate anemia?

A

low RBC, Hgb, Hct

20
Q

What labs help you figure out cause of anemia?

A

MCV
RDW
MCHC

21
Q

what is a retculocyte?

stem cell > synthesizes hgb > lose nucleus > reticulocyte > RBC

A
22
Q

If the bone marrow is fully functional, an increase in erythropoietin caused by acute blood loss or hemolysis will result in increased numbers of circulating reticulocytes within approximately …

A

72 hours!

23
Q

How do we count reticulocytes?

A

Because reticulocytes are red cells that contain ribosomal RNA, we can STAIN them with a colored dye that binds to RNA in order to find them under the microscope or with a FLUORESCENT DYE to count them with a machine

24
Q

When counted manually under the microscope, reticulocytes are reported as a _____ if total cells counted. Why do we have to correct this?

A

Percentage

The reticulocyte count MUST BE CORRECTED FOR THE DEGREE OF ANEMIA. Even a high reticulocyte count might not be adequate for the degree of anemia of the patient.

25
Q

58 year old male brought to the Emergency Department (ED) by spouse because he is “confused”
A CBC is ordered as part of the routine ED evaluation:

Why order a CBC w/ autodiff?

A

HE’S CONFUSED! Low Hbg (anemia) could mean not enough oxygen to the brain! High WBC because of high neutrophils could mean that he is septic! Low Plt could mean that he’s bleeding in his head! Lot’s of clues can be found in an inexpensive lab test.

26
Q

What is the correction facotor calculation for reticuilocytes?

A

RPI = [ %retic x (patient hct/normal hct)] / correction factor

RPI <2 failure of erythropoiesis
RPI >3 mailure hyperpoliferation or appropriate response

27
Q

When RPI is calculated for our pt what is found?

Hgb 9.1 L
Hct 27 L
calculation .3
RBC 2.9 L
MCV 128 H
RDW 20.6 H
MCHC normal
Retic 3.2 (elevated)

normal HCT 45
correction factor 2 given patients hct is 27

A

RPI = [3.2 X (27/45)] ÷ 2.0 = 0.96

so when correctd for anemia the reticulocyte count is LOW

28
Q

Anemia w/ elevated aboslute retic count think…

A

– Bleeding
– Hemolysis
– Patient treated with and responding to erythropoietin or nutritional therapy

29
Q

patient with normal or low absolute reticulocyte count htink…

A

– Bone marrow erythroid hypoplasia
– Ineffective production of mature red cells by bone marrow
– Decreased hemoglobin production
– Bleeding or hemolysis combined with one of the above
– Acute bleeding or hemolysis (too acute for erythropoietin response)

30
Q

67 year old African-American male admitted UMMC with chest pain
• What is the rule that will guide you to the diagnosis?

RBC L 4.29
Hgb L 12.5
HCT L 33.3
formula: 4.2!
MCV normal
MCHC 37.5 H
RDW 15.8 H
A

She is looking for the hgb/hct discrepancy.