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Heme > Normocytic Anemia > Flashcards

Normocytic Anemia Flashcards

1
Q

What normocytic anemia progresses to a microcytic anemia?

A

ACD

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2
Q

If a patient with normocytic anemia has pancytopenia on CBC, what should you expect?

A

Aplastic Anemia

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3
Q

What are the major causes of aplastic anemia?

A

Idiopathic
Chemicals
Infection (parvovirus)
Radiation

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4
Q

What do you see when you look at bone marrow in an aplastic anemia patient?

A

nothing–hypocellular and lots of fat

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5
Q

How do you treat aplastic anemia?

A
  • Remove cause (ex. drug)
  • Transfusions
  • Marrow-stimulating drugs (ex. epoetin alpha, darbepoetin, filgrastim, sargramostim)
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6
Q

How does chronic renal failure lead to anemia?

A

Renal tubular cells produce EPO (under hypoxic conditions), but with kidney failure, you have a decrease in EPO synthesis so decreased hematopoiesis

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7
Q

What do you see on a peripheral smear of a patient with chronic renal failure?

A

acanthrocytes (burr cells)

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8
Q

How do you treat non-hemolytic anemia due to renal failure?

A

Epoetin alpha
Darbepoetin
(agonists of EPO receptors)

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9
Q

What condition must you NEVER give a patient epoetin alpha and darbepoetin?

A

if they have hemoglobin over 12 g/cL

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10
Q

What are the adverse effects of erythropoietin-stimulating drugs?

A

hypotension and thrombosis

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11
Q

Is hereditary spherocytosis due to an intrinsic or extrinsic problem?

A

intrinsic defect in membrane band3, spectrin, or ankyrin leading to TOO LITTLE MEMBRANE

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12
Q

What does a peripheral smear of someone with hereditary spherocytosis show? Why? How does this relate to their presentation?

A

1) spherocytes (too little membrane)
2) Howell-Jolly bodies (must remove spleen in some cases, because you have splenomegaly due to premature removal of RBCs from spleen)

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13
Q

How do you diagnose hereditary spherocytosis?

A

positive osmotic fragility test

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14
Q

How can you treat hereditary spherocytosis?

A

splenectomy

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15
Q

Why is G6PD important for RBCs?

A

you cannot make NADPH, and without NADPH, you cannot prevent oxidative stress:

 - Hgb --> methemoglobin (keep iron in Fe2+)
 - Regenerate GSH from GS-SG
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16
Q

What are the two major causes of G6PD deficiency?

A

X-linked genetic disorder

Eating Fava Beans

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17
Q

What do you see on a peripheral smear of G6PD deficiency? Why?

A

1) Bite cells (spleen phagocytes removes chunks of oxidized hemoglobin from the cell)
2) Heinz bodies (precipitates of oxidized hemoglobin)
3) Blister cells (oxidative damage leads to unstable hemoglobin)

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18
Q

Patients with G6PD deficiency should avoid what?

A

Certain drugs (ex. dapsone and primaquine which are anti-malarial and sulfa drugs)

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19
Q

How do you diagnose G6PD deficiency?

A

Do an enzyme activity test (but wait to do it!)

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20
Q

Why is pyruvate kinase important for cells?

A

Helps produce ATP for:

  • RBC membrane maintenance
  • RBC Na/K ATPase (prevention of lysis)
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21
Q

What causes pyruvate kinase deficiency?

A

Autosomal Recessive genetic disorder

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22
Q

What does a peripheral smear look like for someone with pyruvate kinase deficiency? Why?

A

1) Polychromasia (bluish residual RNA with ramped up RBC synthesis)
2) nRBCs
3) Increased reticulocytes

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23
Q

How do you diagnose PK deficiency?

A

enzyme activity test

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24
Q

What causes paroxysmal nocturnal hemoglobinuria?

A

Mutation in PIG-A gene on X chromosome that codes a glycolipid that anchors proteins (like DAF and CD59) which remove complement (that is ramped up during sleep when we get respiratory acidosis).

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25
Q

What is the presentation of PNH?

A

intermittent hemolysis in adults

assoicated with thrombosis

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26
Q

What does a peripheral smear look like with someone who has PNH?

A

normal

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27
Q

How do you diagnose PNH?

A 
urine hemosiderin
flow cytometry (CD55-, CD59-)
28
Q

How do you treat PNH?

A

Bone marrow transplant

Ecluzimab

29
Q

What causes sickle cell disease (HbS)?

A

AR mutation leading to Base change in AA#6 Glu (negative)–> Val (neutral) of beta chain of Hgb

30
Q

What causes HbC?

A

Base change in AA#6 Glu (negative)–> Lys (positive)

31
Q

How does HbS lead to symptoms?

A

HbS polymerizes (due to valine cross-links) when deoxygenated forming needle-like structures that are vasooclusive and that lead to hemolysis of RBCs

32
Q

What triggers HbS sickling?

A 
hypoxia
anemia
cold
Acidosis
dehydration
33
Q

How do people present with HbS?

A

Newborns asymptomatic (because HbF is protective)
Dactylitis (children, vaso-occlusive infarcts in bone)
Pain
Leg ulcers
Asplenia

34
Q

Why do you get asplenia with sickle cell disease? What is the effect of this?

A

spleen atrophies from vascular occlusion

You are at increased risk of infection and death from encapsulated bacteria (ex. S. pneumoniae, H. influenzae, and Salmonella paratyphi)

35
Q

How do you diagnose sickle cell?

A

HPLC
Hgb electrophoresis
Sickle cells in peripheral smear

36
Q

How do you treat sickle cell disease?

A 
hydration
avoidance of triggers
pain management
hydroxea urea (hypomethylates DNA to "turn on" fetal Hgb)
Bone marrow transplant
37
Q

In what case would you see jaundice with an intravascular hemolysis?

A

if Hgb saturates haptoglobin you can get jaundice (this is NOT common)

38
Q

What causes a microantiopathic hemolytic anemia?

A

prosthetic heart valves

dysfunctional/calcified aortic valve

39
Q

What causes a microangiopathic hemolytic anemia?

A

DIC
TTP
SLE

40
Q

What cells do you expect to see in a peripheral smear of a microangiopathic hemolytic anemia?

A

schistocytes (helmet cells)- because cells are being crushed/sliced

41
Q

What leads to TTP?

A

antibodies made against AdamTS13 so that it cannot properly trip VWF. Leads to small clots formed in peripheral vessels that can slice RBCs as they zoom by

42
Q

A patient with TTP presents with what?

A 
FAT RN: (young adults, mostly females)
Fever
Anemia
Thrombocytopenia (thrombosis, hemorrhaging, bruising)
Renal failure
Neurological defects
43
Q

How do you treat TTP?

A

plasmapheresis

44
Q

What is the cause of a warm autoimmune hemolytic anemia?

A

IgG targeted at RBC leading to extravascular hemolysis (RES in spleen) or intravascular hemolysis (due to complement-mediated lysis)

45
Q

What does a peripheral smear of a patient with warm autoimmune hemolytic anemia show?

A

microspherocytes (partial phagocytosis from RES)

46
Q

How do you diagnose a warm autoimmune hemolytic anemia?

A

DAT (positive for Ab AND complement)

Direct Ab

47
Q

Warm autoimmune hemolytic anemia is usually associated with what?

A

malignancies (poor prognosis)

48
Q

How do you treat warm autoimmune hemolytic anemia?

A 
Steroids
Splenectomy
Rituximab
Azathrioprine
Blood transfusion
49
Q

What is the cause of cold autoimmune hemolytic anemia?

A

IgM targed RBC–> complement MAC leading to intravascular hemolysis (jaundice not as common) and hemoglobinuria

50
Q

What does a peripheral blood smear look like in a patient with cold autoimmune hemolytic anemia?

A

aggregates of RBCs

51
Q

How do you diagnose cold autoimmune hemolytic anemia?

A

Direct Ab test

DAT positive for complement

52
Q

Cold autoimmune hemolytic anemia is usually associated with what?

A

viral syndromes and infectious diseases (chronic/seasonal)

53
Q

How do you treat cold autoimmune hemolytic anemia?

A

steroids

54
Q

What is the most common and deadly protozoal infections in humans?

A

malaria

plasmodium falciparum is most lethal

55
Q

What are the presenting symptoms of malaria?

A

high fevers, dark urine, jaundice after travel to subtropics

56
Q

What is the vector for malaria?

A

mosquito vector

57
Q

How do you diagnose malaria?

A
  • Peripheral blood smear (very thick)

- Identify merozites and gametocytes

58
Q

What protozal infection can you get from ticks in NE USA?

A

babesia

59
Q

What does a peripheral blood smear look like in a patient with babesia?

A

Strange looking with large things that look like they come from a karyotype

60
Q

What parastie infection can you get from sand flies in Peru, Ecuador and Columbia?

A

Bartonella

61
Q

What are the presenting symptoms of Bartonella?

A

Acute: anemia
Chronic: Peruvian warts
Splenomegaly

62
Q

What does a peripheral blood smear look like in a patient with Bartonella?

A

basophlic intracellular parasite (looks like bits of string)

63
Q

What is the prognosis of Bartonella?

A

FATAL if not treated with antibiotics

64
Q

How does anemia occur after a C. Perfringens infection?

A

It is a normal skin flora that releases alpha toxin that cuases hemolysis and gas gangrene

65
Q

When would you expect anemia due to C. perfringens?

A

after trauma, septic abortion, cholecystitis

66
Q

What does a peripheral blood smear look like in a patient with C. perfringens?

A 
accumulation of microspherocytes
HgB shadow (very faint cells)

Heme (10 decks)

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