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50 Cards in this Set

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How can you categorize the types of anemias?

- Microcytic (MCV <80 fL)
- Normocytic (MCV 80-100 fL)
- Macrocytic (MCV >80 fL)

What are the types of microcytic anemias? Definition?

MCV <80 fL
- Iron deficiency (late)
- Anemia of chronic disease (may initially present as a normocytic anemia)
- Thalassemias
- Lead poisoning
- Sideroblastic anemia

What are the types of normocytic anemias? Definition?

MCV 80-100 fL

Non-hemolytic (reticulocyte count normal or ↓)

Hemolytic (reticulocyte count ↑)
- Intrinsic
- Extrinsic

What are the types of non-hemolytic, normocytic anemias? Definition?

MCV 80-100 fL and reticulocyte count normal or ↓
- Anemia of chronic disease (may progress to microcytic anemia)
- Aplastic anemia
- Chronic kidney disease
- Iron deficiency (early, may progress to microcytic)

What are the types of intrinsic hemolytic, normocytic anemias? Definition?

MCV 80-100 fL with increased reticulocyte count
- RBC membrane defect (hereditary spherocytosis)
- RBC enzyme deficiency (G6PD or pyruvate kinase)
- HbC defect
- Paroxysmal nocturnal hemoglobinuria
- Sickle cell anemia

What are the types of extrinsic hemolytic, normocytic anemias? Definition?

MCV 80-100 fL with increased reticulocyte count
- Auto-immune
- Microangiopathic
- Macroangiopathic
- Infections

What are the types of macrocytic anemias? Definition?

MCV >100 fL

- Megaloblastic
- Non-megaloblastic

What are the types of macrocytic, megaloblastic anemias? Definition?

MCV >100 fL
- Folate deficiency
- B12 deficiency
- Orotic aciduria

What are the types of macrocytic, non-megaloblastic anemias? Definition?

MCV >100 fL
- Liver disease
- Alcoholism
- Reticulocytosis

What is the most likely cause of this patient's conjunctival pallor?

What is the most likely cause of this patient's conjunctival pallor?

Anemia (possibly due to iron deficiency)

Anemia (possibly due to iron deficiency)

What causes iron deficiency anemia?

Decreased iron due to:
- Chronic bleeding (eg, GI loss or menorrhagia)
- Malnutrition / absorption disorder
- ↑ Demand (eg, pregnancy)

What are the implications of an iron deficiency?

Decreased completion of final step in heme synthesis

What lab findings are associated with iron deficiency anemia?

- MCV < 80 fL (microcytic)
- ↓ Iron
- ↑ TIBC
- ↓ Ferritin
- Hypochromia

- MCV < 80 fL (microcytic)
- ↓ Iron
- ↑ TIBC
- ↓ Ferritin
- Hypochromia

What other unrelated symptoms should you look for in a patient with iron deficiency anemia to check for another syndrome?

Plummer-Vinson Syndrome:
- Also esophageal webs and atrophic glossitis

What is the term for the triad of iron deficiency anemia, esophageal webs, and atrophic glossitis?

Plummer-Vinson Syndrome

What are the symptoms in Plummer-Vinson Syndrome?

- Iron deficiency anemia
- Esophageal webs
- Atrophic glossitis

What does this blood smear tell you?

What does this blood smear tell you?

Iron Deficiency Anemia
- Microcytosis
- Hypochromia (central pallor)

Iron Deficiency Anemia
- Microcytosis
- Hypochromia (central pallor)

What is the defect in α-thalassemia?

α-globin gene deletions → ↓ α-globin synthesis

What kind of deletions can lead to α-thalassemia?

- Cis deletion prevalent in Asian populations
- Trans deletion prevalent in African populations

How many copies of the α-globin gene do you have? Implications?

4 alleles:
- 1-2 allele deletion → no significant anemia
- 3 allele deletion → HbH disease, very little α-globin, excess β-globin forms β4 (HbH)
- 4 allele deletion → no α-globin, excess γ-globin forms γ4 (Hb Barts); incompatible with life (causes hydrops fetalis)

What causes hydrops fetalis?

4 α-globin allele deletion:
- No α-globin
- Excess γ-globin forms γ4 (Hb Barts)
- Incompatible with life

What causes HbH disease?

3 α-globin allele deletion:
- HbH disease, very little α-globin
- Excess β-globin forms β4 (HbH)

What form of α-thalassemia is more common in Asians?

Cis deletion of α-globin alleles

What form of α-thalassemia is more common in Africans?

Trans deletion of α-globin alleles

Who is more likely to have β-thalassemia?

Prevalent in Mediterranean poulations

What causes β-thalassemia?

Point mutations in splice sites and promoter sequences → ↓ β-globin synthesis

What are the types of β-thalassemia?

- β-thalassemia minor (heterozygote)
- β-thalassemia major (homozygote)
- HbS / β-thalassemia heterozygote

What is the most severe form of β-thalassemia?

β-thalassemia major (homozygote)
- β chain is absent → severe anemia

What are the consequences of having absent β chain (β-thalassemia major)?

- Severe anemia → requires blood transfusion
- Marrow expansion → skeletal deformities → "crew cut" on skull x-ray
- Chipmunk facies
- Extramedullary hematopoiesis → hepatosplenomegaly
- ↑ Risk of parvovirus B19-induced aplastic crisis

What kind of infection are patients with β-thalassemia major at risk for? Complications?

Parvovirus B19 → can induce an aplastic crisis

What kind of hemoglobin is more common in patients with β-thalassemia major?

HbF (α2γ2) - protective in the infant and disease only becomes symptomatic after 6 months

What is the medium severity form of β-thalassemia?

HbS / β-thalassemia heterozygote
- Mild to moderate sickle cell disease depending on the amount of β-globin production

What is the least severe form of β-thalassemia?

β-thalassemia Minor (heterozygote)
- β chain is underproduced, usually asymptomatic

How do yo confirm diagnosis of β-thalassemia minor (heterozygote)?

Confirm diagnosis by ↑ HbA2 (>3.5% on electrophoresis)

What happens to RBCs in patients with β-thalassemia major?

Note anisocytosis, poikilocytosis, target cells (arrows 1 and 2), microcytosis (arrow 3), and schistocytes (arrow 4)

Note anisocytosis, poikilocytosis, target cells (arrows 1 and 2), microcytosis (arrow 3), and schistocytes (arrow 4)

How does lead poisoning affect the blood?

- Lead inhibits ferrochelatase and ALA dehydratase → ↓ heme synthesis and ↑ RBC protoporphyrin
- Also inhibits rRNA degradation, causing RBCs to retain aggregates of rRNA (basophilic stippling)

Who is at risk for lead poisoning?

High risk in old houses with chipped paint

How does lead poisoning affect other organs besides the blood?

LEAD:
- Lead Lines on gingivae and on metaphyses of long bones on x-ray
- Encephalopathy and Erythrocyte basophilic stippling
- Abdominal colic and sideroblastic anemia
- Drops: wrist and foot drop

What does the arrow point at? Sign of?

What does the arrow point at? Sign of?

- Lead Lines on metaphyses of long bones on x-ray
- Sign of Lead Poisoning

- Lead Lines on metaphyses of long bones on x-ray
- Sign of Lead Poisoning

How does lead affect the gums?

Lead Lines on gingivae = Burton lines
- Sign of lead poisoning

How does lead affect the brain?

Can cause encephalopathy

How does lead affect the musculoskeletal system?

Drops: wrist and foot drops

How does lead affect the abdomen?

Abdominal colic

How do you treat lead poisoning?

First line treatments:
- Dimercaprol
- EDTA

Succimer used for chelation for kids ("it sucks to be a kid who eats lead")

What is wrong in this picture? Cause?

What is wrong in this picture? Cause?

Sideroblastic Anemia
- Defect in heme synthesis 
- Hereditary: X-linked defect in δ-ALA synthase gene

Sideroblastic Anemia
- Defect in heme synthesis
- Hereditary: X-linked defect in δ-ALA synthase gene

What causes sideroblastic anemia?

What causes sideroblastic anemia?

- Genetic
- Acquired (myelodysplastic syndromes)
- Reversible (alcohol is most common, lead, vitamin B6 deficiency, copper deficiency, and isoniazid)

- Genetic
- Acquired (myelodysplastic syndromes)
- Reversible (alcohol is most common, lead, vitamin B6 deficiency, copper deficiency, and isoniazid)

What causes acquired sideroblastic anemia?

What causes acquired sideroblastic anemia?

Myelodysplastic syndromes

Myelodysplastic syndromes

What causes reversible sideroblastic anemia?

- Alcohol is most common
- Lead
- Vitamin B6 deficiency
- Copper deficiency
- Isoniazid

What are the lab findings in sideroblastic anemia?

- ↑ Iron
- Normal TIBC
- ↑ Ferritin

How do you treat Sideroblastic Anemia?

How do you treat Sideroblastic Anemia?

Pyridoxine (B6, cofactor for δ-ALA synthase)

Pyridoxine (B6, cofactor for δ-ALA synthase)