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USMLE Hematology Leukemmia and Lymphoma > Normocytic Anemia > Flashcards

Normocytic Anemia Flashcards

1
Q

What do reticulocytes (young RBCs) look like on blood smear?

A
  • Large cells

- Blueish cytoplasm

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2
Q

Why does the reticulocyte look blue?

A

RNA within cytoplasm

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3
Q

What is the normal reticulocyte count?

A

1-2%

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4
Q

if the bone marrow is properly functioning what is the reticulocyte count in an anemia increased to?

A

> 3%

- Falsley elevated as this is a percentage of total RBC which will be lower

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5
Q

What is the normal reticulocyte level multiplied by to get the corrected reticulocye count?

A

Hct/45

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6
Q

How can hemolysis / peripheral RBC destruction also be divided into?

A

Extravascular and intravascular

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7
Q

What is the bone marrow response like in hemolysis or peripheral RBC destruction anemia?

A

Good bone marrow response

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8
Q

What destroys RBCs in an extravascular hemolysis?

A

Macrophages in reticuloendothelial system (in spleen, liver and lymph nodes)

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9
Q

What is globin broken down into?

A

Amino acids

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10
Q

What is protoporphyrin broken down into?

A

Unconjugated bilirubin

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11
Q

What is unconjugated billirubin bound to?

A

Serum albumin

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12
Q

What are the clinical and labratory findings in extravascular hemolysis?

A
  • Anemia w splenomegaly (RE system)
  • Jaundice due to unconjugated bilirubin
  • Increased risk for bilirubin gallstones
  • Marrow hyperplasia w corrected reticulocyte count >3%
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13
Q

What binds to free hemoglobin in the blood?

A

Haptoglobin

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14
Q

What are the levels of haptoglobin like in intravascular hemolysis?

A

Low as they are bound to hemoglobin

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15
Q

What are the clinical and labratory findings in intravascular hemolysis?

A
  • Hemoglobinuria
  • Hemoglobinuria
  • Hemosiderinuria (tubular cells fall off - days later)
  • Decreased serum haptoglobin
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16
Q

What kind of anemia is hereditary spherocytosis?

A

Normocytic anemia

- Extravascular hemolysis

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17
Q

What is affected in hereditary spherocytosis?

A
  • Inherited defect of RBC cytoskeleton membrane tethering proteins
  • Most commonly involves spectrin, ankyrin or band 3.1
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18
Q

What will be seen on blood smear in hereditary spherocytosis?

A
  • Differing RBC sizes - High RDW - RBC distribution width

- Non biconcave (no central pallor)

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19
Q

How does Hereditart Spherocytosis result in anemia?

A

Spherocytes are less able to maneuver through speinic sinusoids
- Splenic macrophages then consume them creating an anemia

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20
Q

What are the labratory and clinical findings in HS?

A
  • Spherocytes w loss of central pallor
  • Increased RDW and MCHC
  • Splenomegaly
  • Jaundice (possible billirubin gallstones)
  • Increased risk of aplastic crisis w. parvovirus B19 infection of erythroid precursors
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21
Q

What is MCHC?

A

Mean corpuscular hemoglobin concentration

  • Average amount of hemoglobin per RBC relative to the size of the cell
  • Hemoglobin / Hct

Increased in HS

22
Q

What does parvovirus B19 affect?

A

Erythroid precursors

- Aplastic crisis possibility

23
Q

How is HS (Herditary Spherocytosis) diagnosed?

A

Osmotic fragility test

  • Increased fragility in hypotonic solution
  • There is no extra membrane therefore it can’t expand as easily and will burst
24
Q

How is HS treated?

A

Splenectomy

- Splenic macrophages are what is causing the condition

25
Q

What will happen after splenectomy / what can be seen in Hereditary spherocytosis post-splenectomy?

A
  • Anemia resolves
  • Spherocytes persist
  • Howell-Jolly bodies emerge on blood smear - Speen usually removes these DNA fragments
26
Q

What do Howell-Jolly bodies look like on blood smear?

A

Small blue dots (DNA fragments)

27
Q

What kind of anemia is sickle cell?

A

Normocytic hemolytic extravascular (mostly) anemia

- Also involves intravascular hemolysis resulting in decreased haptoglobin and target cells

28
Q

What is affected in sickle cell?

A

Beta chain of hemoglobin

29
Q

What is replaced in Sickle cell?

A

Normal glutamic acid (hydrophilic) replaced with valine (hydrophobic)

30
Q

What happens to hemoglobin S when deoxygenated?

A

Poymerises

- Polymers aggregate into needle-like structures resulting in sickle cells

31
Q

What causes an increased risk of sickling?

A
  • Hypoxemia
  • Dehydration
  • Acidosis
32
Q

What does hydroxyurea do?

A

Increases levels of HbF

- Used in sickle cell

33
Q

What is massive erythroid hyperplasia?

A

Expansion of hematopoiesis into skull and facial bones (chipmunk)

  • Extramedullary hematopoeisis w. hepatomegaly
  • Risk of aplastic crisis w. parvovirus B19 infection of erythroid precursors
  • Can occur in extravascular and intravascular hemolysis (sickle)
34
Q

What is Dactylitis?

A
  • Sign of sickle cell in infants

- Swollen hands and feet due to vaso-occlusive infarcts of bones

35
Q

What is autosplenectomy?

A
  • Shrunken, fibrotic spleen
  • Associated w. sickle cell
  • Howell-Jolly bodies will be seen on blood smear
36
Q

What organism specifically may cause osteomyelitis in patients with sickle cell?

A

Salmonella paratyphi

- Autosplenectomy -> risk of encapulated organsim infection

37
Q

What is the most common cause of death in sickle cell adults?

A

Acute chest syndrome

38
Q

What is the most common cause of death in sickle cell children?

A

Encapsulated organism

39
Q

What is acute chest syndrome?

A
  • Vaso-occlusion in pulmonary microcirculation

- Presents with chest pain, SOB, and lung infiltrates; often precipitated by pneumonia

40
Q

What is Renal papillary necrosis?

A
  • Vaso-occlusive crisis of kidney

- Results in gross hematuria and proteinuria

41
Q

What percentage of Africans carry sickle cell?

A

~ 10%

42
Q

What is HbS?

A

Hb A (alpha2, Beta2) except the Beta chain is mutated with valine (hydrophobic) instead of glutamic acid

43
Q

What can carriers of sickle cell be affected by?

A

Generally asymptomatic however can get microinfarctions in renal medulla
- Microscopic hematuria and eventually decreased ability to concentrate urine

44
Q

What causes sickling?

A

Hypoxia and hypertonicity

45
Q

What does the metabisulfite screening test diagnose?

A

Sickle cell disease and trait

46
Q

What will electrophoresis show in sickle cell disease?

A

90% HbS, 8% HbF, 2% HbA2

- No HbA

47
Q

What will sickle cell trait show on electrophoresis?

A

55% HbA, 43% HbS, 2% HbA2

48
Q

How are sickle cell and Hemoglobin C disease inherited?

A

Aut Recessive

49
Q

What is glutamic acid replaced by in Hemoglobin C disease?

A

Lysine

50
Q

What is seen on blood smear in hemoglobin C disease?

A

Hemoglobin crystals

- Pnk/purple cylinder

USMLE Hematology Leukemmia and Lymphoma (13 decks)

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