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Block 7; Week 3-Michelle > Anemia & Red Cell Disorders II-Usera > Flashcards

Anemia & Red Cell Disorders II-Usera Flashcards

1
Q

What are some potential causes of normocytic anemias–reticulocyte count of <3%?

A 
Acute blood loss
Early iron deficiency and ACD
Aplastic anemia
Chronic renal failure
malignancy
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2
Q

What are some intrinsic defects that can cause normocytic anemia?

A

Membrane defect
Abnormal Hb
Enzyme deficiency

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3
Q

What are some extrinsic defects that can cause normocytic anemia?

A

Trauma (e.g. aortic stenosis, prosthetic valves)

Immune destruction

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4
Q

What’s the deal with extravascular hemolysis

A

Rbc phagocytosis by splenic and hepatic macs
IgG bound, with or without c3b
Abnormal shape (e.g. spherocytosis, sickling)

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5
Q

What are the lab findings for patients who experience extravascular hemolysis?

A

Increase unconjugated bilirubin

Increased serum LDH

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6
Q

What’s the deal with intravascular hemolysis?

A 
Hemolysis occurs within blood vessels
Enzyme deficiency (g6pd)
Mechanical trauma
Complement/immune destruction
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7
Q

What are the lab findings for patients who are experiencing intravascular hemolysis?

A

Increase unconjugated bilirubin (minimal)
Increased serum LDH
Decreased serum haptoglobin
hemosiderinuria

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8
Q

What is haptoglobin?

A

an acute phase reactant that complexes with hemoglobin

CD163 receptor on macrophages recognizes the Hp-Hb complex.

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9
Q

What does the degradation of Hp-Hb complex by the macrophage produce/release?

A

bilirubin

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10
Q

What’s the deal with hereditary spherocytosis?

A

aut dom
US 1/5K
Intrinsic Defect-usu mutation in ankyrin
could also be caused by mutations in band3, spectrins, protein 4.2

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11
Q

What are the clinical features of hereditary spherocytosis?

A

Jaundice-chronic hemolysis
Gallstones-accumulation of calcified bilirubinate
Splenomegaly
Aplastic crisis in children

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12
Q

What are the lab findings for hereditary spherocytosis?

A

normocytic anemia
increased MCHC–sphere holds more hemoglobin & shrinks at the same time
increased osmotic fragility-bursts in hypotonic solution
elevated LDH & bilirubin

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13
Q

What is the treatment for hereditary spherocytosis?

A

splenectomy

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14
Q

What’s the deal with hereditary elliptocytosis?

A

aut dom
defect in spectrin or protein 4.1 that binds the alpha spectrins
>25% peripheral blood elliptocytes
not a fatal disease

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15
Q

What are the main clinical findings for patients with elliptocytosis?

A

mild anemia

splenomegaly

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16
Q

What causes paroxysmal nocturnal hemoglobinuria?

A

acquired membrane defect in myeloid stem cells
mutation in Pig-A gene
mutation in GPI causes loss of decay accelerating factor

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17
Q

What’s the deal with paroxysmal nocturnal hemoglobinuria?

A

shows intravascular complement mediated lysis
episodic hemoglobinuria
happens at night b/c of the acidosis brought on by shallowing breathing-favors complement
can lead to iron deficiency
increased risk of thrombosis b/c of all the random platelets running around.
increased risk of acute myeloid leukemia

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18
Q

What is the normal role of decay accelerating factor when it isn’t mutated in PNH?

A

it protects the cell from complement-mediated damage by inhibiting C3 convertase
should prevent the formation of the membrane attack complex
mutated GPI prevents DAF from connecting to cells

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19
Q

What would the labs look like for a patient with paroxysmal nocturnal hemoglobinuria?

A

Normocytic anemia with pancytopenia
Decreased haptoglobin
Increased serum/urin hb

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20
Q

What’s the deal with sickle cell anemia?

A

aut rec
missense mutation: glutamic acid substituted with valine
affects the beta globin chain
10% African Americans have the trait (heterozygous)
60% messed up beta globin or more–disease. Called HBS

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21
Q

What is HbS?

A

Hemoglobin with the following structure:

alpha2beta2S

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22
Q

When do you recognize that a patient has sickle cell anemia in the absence of testing?

A

only once the cells have sickled!

that is when they are deposited in capillaries. Macrophages desperately try to remove them from circulation.

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23
Q

Initial sickling is reversible via administration of ____.

A

Oxygen!

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24
Q

What type of hemolysis occurs in sickle cell anemia? What are its triggers?

A 
extravascular hemolysis mainly
Triggers:
low ph 
low oxygen tension
Volume depletion
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25
Q

When is the earliest age that sickle cell anemia can present? Why is this?

A

Earliest: 6 mo
b/c neonates still have their fetal hemoglobin HbF that does not consist of beta chains
(alpha 2gamma2)

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26
Q

What are the clinical findings for sickle cell anemia?

A 
Dactylitis—thing in the fingers
Acute chest syndrome (most common cause of death)
Stroke
Gallstones-calcified bilirubinate
priapism-yikes!
Aseptic necrosis of femoral head
Aplastic crisis (ass. With parvovirus)
Autosplenectomy (splenomegaly by 2 yo, then loses function) – Howell-jolly bodies
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27
Q

How does acute chest syndrome present in patients with sickle cell anemia?

A

dyspnea
wheezing
chest pain
back pain

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28
Q

Why are patients with sickle cell anemia more susceptible to infections? Which types?

A

b/c their spleen is dysfunctional
strep pneumonia
encapsulated organsims
salmonella paratyphi-can cause osteomyelitis

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29
Q

What are the renal findings seen with sickle cell anemia?

A

sickling in peritubular capillaries b/c of low O2 there
renal papillary necrosis
microhematuria (dead RBCs in tubules)

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30
Q

What is the treatment for sickle cell anemia?

A

Infectious prophylaxis
Pain management
Transfusion – acute chest syndrome, aplastic crisis—only 2 indications.

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31
Q

T/F You should transfuse a patient with sickle cell anemia who presents to the ER with priapism.

A

FALSE

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32
Q

What are some preventative measures for patients with sickle cell anemia–to keep them from experiencing crisis?

A

Hydroxyurea
Immunizations
Pneumococcal vaccine
Folic acid supplementation

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33
Q

What happens with G6pd deficiency?

A

lack of G6pd means lack of NADPH
lack of NADPH means that it is hard to make glutathione
this is critical for protecting the cell from oxidative stress
at risk for hemolysis & infection

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34
Q

Why are patients with g6pd deficiency at greater risk for infection?

A

b/c our body often uses oxidative stress to fight infections.
MPO deals with that oxidative stress
but it needs NADPH–produced by g6pd

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35
Q

What are some drugs & compounds that compromises enzyme fcn in patients with g6pd deficiency?

A

drugs: primaquine, an anti-malarial
compounds: fava beans (used in middle east)

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36
Q

Give some important genetic/demographic info about g6pd deficiency.

A 
recessive X--linked pattern 
affects 22% of U.S. blacks
females are asymptomatic 
older RBCs usually affected 
young RBCs have normal or near normal levels
37
Q

What important inclusion body do you see in g6pd deficiency histo?

A

heinz bodies: precipitated denatured hemoglobin

**See yellow RBCs & blue bodies.

38
Q

With which condition do you see bite cells on the histo slides? Why?

A

g6pd deficiency

the macrophage removes the damaged membranes

39
Q

What are the clinical & lab findings in patients with g6pd deficiency?

A

-sudden onset back pain and delayed hemoglobinuria
-Susceptibility to infections (impaired MPO no nadph)
-normocytic anemia
-Heinz bodies
RBC enzyme analysis after hemolytic episode has resolved:
*g6pd levels are ~normal in retic and young cells

40
Q

What type of anemia is immunohemolytic anemia? What are its subcategories?

A

normocytic
extrinsic hemolytic anemia with intra or extravascular hemolysis

Warm agglutinins
Cold agglutinins
complement
Drug induced
Paroxysmal Cold Hemoglobinuria (PCH)
41
Q

What’s the deal with warm agglutinin immunohemolytic anemia?

A 
happens 70% of the time
triggered by warm temps
IgG coated RBCs eaten by macrophages
happens in the central part of the body
extravascular hemolytic anemia
42
Q

Which conditions are associated with warm agglutinin immunohemolytic anemia?

A

associated with SLE, collagen vascular diseases (SLE), CLL, malignant lymphoma (esp. Hodgkin’s), viral infections, etc.

43
Q

What’s the deal with cold agglutinin immunohemolytic anemia?

A 
happens 30% of the time
triggered by colder temps
IgM coated RBCs eaten up
intra or extravascular hemolysis
happens in more distal parts of the body
includes Raynaud's Phenomenon
44
Q

What are some conditions associated with cold agglutinin immunohemolytic anemia?

A

causes include: Mycoplasma pneumoniae, infectious mononucleosis, CLL, drugs, etc.

45
Q

What is an example of complement mediated extravascular hemolysis?

A

C3b coated RBC phagocytosed by liver macrophages

46
Q

What is an example of complement mediated intravascular hemolysis?

A

RBCs coated by C5-C9 membrane attack complex

47
Q

What are some clinical findings of immunohemolytic anemia?

A

Jaundice
Hepatosplenomegaly
Raynaud’s phenomenon

48
Q

WHat are some lab findings associated with immunohemolytic anemia?

A 
Positive dat
Positive indirect
Unconjugated hyperbilirubinemia
Hemoglobinuria
Decreased haptoglobin
Normocytic anemia
Rbc agglutination (igm perhaps)
49
Q

Explain how penicillin relates to drug induced immunohemolytic anemia.

A 
penicillin binds to RBC membrane
antibody (IgG) binds to the penicillin fragment
extravascular hemolysis
direct coombs test positive
IHA
50
Q

Give some drug-antidrug immune complex examples.

A

IgG or IgM bind complement
IgG → extravascular hemolysis
IgM → intravascular hemolysis
drugs: quinidine, quinine, INH, sulfonamides

51
Q

What is the appropriate treatment for immunohemolytic anemia?

A 
discontinue the offending drug
corticosteroids
immunosuppression
splenectomy
IVIG--binds macrophages--tricks them into thinking they have bound the antigen.
52
Q

What types of things will you see on a peripheral smear if you are experiencing traumatic hemolysis?

A

cell fragments

schistocytes, burr cells, helmet cells

53
Q

What is the relationship b/w DIC: disseminated intravascular coagulation & traumatic hemolysis?

A

DIC can cause traumatic hemolysis

traumatic hemolysis can also leave a bunch of cell fragments lying around & lead to coagulation & DIC

54
Q

What are other possible causes of traumatic hemolysis?

A

long distance running
artificial heart valves
thrombotic thrombocytopenia purpura

55
Q

What are some causes of nonimmunologic hemolytic anemia?

A 
hypersplenism
microangiopathic hemolytic anemia (MAHA)
microorganisms, i.e. malaria, babesia, Clostridium perfringens
snake venoms, i.e. cobra venom
chemical
physical, i.e. burns
56
Q

What causes alloimmune hemolytic anemia?

A

transfusion reaction
allo–someone else’s blood
intra or extravascular hemolysis
the extra hemoglobin is toxic to the kidneys
**also newborn getting maternal antibodies–can lead to anemia & hyperbilirubinemia

57
Q

What are the lab findings for patients with alloimmune hemolytic anemia from a transfusion reaction?

A 
DAT 
anemia depending on severity 
total and indirect bilirubin elevated
haptoglobin ↓
LDH ↑
58
Q

What are the lab findings with alloimmune hemolytic anemia from a newborn getting maternal antibodies?

A

positive DAT
↑ total and indirect bilirubin
LDH ↑
initially hematocrit and hemoglobin may be within normal limits

59
Q

Which antibody is capable of crossing the placenta?

A

IgG

60
Q

Which systemic disease displays anemia?

A 
renal disease (uremia)
Normochromic, normocytic
mild anisocytosis
sometimes hypochromic, microcytic
burr cells – shrunken RBCs with irregular projections (echinocytes)
usually present when BUN is twice normal
61
Q

What are the possible mechanisms by which renal disease causes anemia?

A

bone marrow suppression
hemolysis from impaired renal excretion
coagulation defects (in severe disease) leading to blood loss
impaired erythropoietin production from renal endocrine failure

62
Q

Explain the deal with anemia with neoplasia.

A

often seen with bone marrow malignancies.

usually normochromic and normocytic unless there is:
blood loss
hemorrhage
a myelophthisic process—relating to the bone marrow
mild hemolytic component often present
severe with some lymphomas
possibly due to the altered endothelium of malignant tissues (a set-up for DIC)

63
Q

Which types of infection can cause anemias?

A

malaria

bacterial toxins–clostridium perfringens

64
Q

What are the 2 causes of megaloblastic macrocytic anemias?

A

folate deficiency

vit b12 deficiency

65
Q

What is the cause of the nonmegaloblastic macrocytic anemia?

A

alcoholism

66
Q

Which histo indication do you see in patients with megaloblastic anemia?

A

a huge nucleus b/c they are struggling in their mitotic dreams
pretty sure you will also see a hyper segmented neutrophil

67
Q

What are some causes of vit b12 deficiency?

A 
Pernicious anemia-autoimmune destruction of parietal cells
Pure vegan diet
Malnutrition
Malabsorption
↓ Intrinsic factor
↓ Gastric acid
↓ Intestinal absorption
68
Q

Describe how vit b12 is absorbed.

A

B12 combines with R binders (secreted by chief cells).
Acid frees elemental B12 from what you have consumed so that it can combine with R.
R goes thru duodenum.
Pancreatic enzymes cleave.
B12 can combine with intrinsic facotr.
This is absorbed in terminal ileum.

69
Q

HOw can PPIs mess with Vit b12 absorption?

A

don’t have enough gastric acid to free the B12 to bind with R binder.

70
Q

How can Crohn’s disease mess with Vit B12 absorption?

A

messes with SI

Vit B12 absorbed in the terminal ileum

71
Q

What are the 3 main categories of causes for folate deficiency?

A

decreased intake
malabsorption
drug inhibition

72
Q

What are some decreased intake causes of folate deficiency?

A

Malnutrition
Etoh-inhibits folate absorption
Goat milk
Infants/elderly

73
Q

What are some malabsorption causes of folate deficiency?

A

Celiac-not B12, but folate b/c that is absorbed in jejunum

Bacterial overgrowth

74
Q

What are some drug inhibition causes of folate deficiency?

A 
5-FU-chemo
MTX-chemo, arrest DNA maturation & synthesis
Tpm-sfx
Phenytoin
Ocp’s
etoh
75
Q

How long does it usu take to become Vit B12 deficient?

A

usu about 6-9 years b/c of the b12 that is stored in your liver

76
Q

What does IFB mean in terms of intestines?

A

Iron–absorbed in the duodenum
Folate–absorbed in the jejunum
B12–absorbed in the terminal ileum

77
Q

What is the pathogenesis of megaloblastic anemia?

A

Delayed nuclear maturation
Affects all rapidly dividing cells
Cellular RNA and protein synthesis unabated
**cytoplasm matures faster than nucleus

78
Q

If you are folate or VB12 deficient you could get elevated ______. What are the consequences of this?

A

homocysteine
consequences: damage to vascular endothelial cells
vascular disease & atherosclerosis

79
Q

What type of hypersensitivity reaction is involved in pernicious anemia? What else do these patients suffer from?

A

Type II Hypersensitivity
Autoimmune destruction of parietal cells (85-90%)
Antibodies that block B12-if binding (60-75%)
also suffer from achlorhydria b/c unable to have the parietal cells produce acid

80
Q

Which blood type is more susceptible to pernicious anemia…for whatever reason?

A

Blood Group A individuals

81
Q

What are some clinical findings for pernicious anemia? Which of these is def not seen in folate deficiency?

A

smooth/sore tongue & red (lose papillae)
peripheral neuropathy** loss of vibration & proprioception–can’t sense the tuning fork.
dementia

82
Q

What causes the loss of VP in Vit B12 deficiency?

A

demyelination of dorsal columns

83
Q

Which conditions do you see raised urine methylmalonic acid in?

A

Vit B12 deficiencies–pernicious or not.

84
Q

What are some lab/clinical findings shared in both Vit B12 & folate deficiencies?

A

hypersegmented neutrophils
high MCV
pancytopenia
high homocysteine

85
Q

What is the best test for detecting folate deficiency?

A

Decreased serum and RBC folate (best test)

86
Q

Folate deficiency puts kiddos in utero at risk for what?

A

neural tube defects

87
Q

What is the treatment for folate deficiency?

A

Intramuscular injections of b12

Oral administration of monoglutamic folic acid

88
Q

What’s the deal with nonmegaloblastic macrocytosis?

A

anemia not always present
chronic alcoholism is the most common cause
liver disease can cause it-check for elevated cholesterol levels
on histo you will see target cells

Block 7; Week 3-Michelle (6 decks)

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