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Block 7 Week 3 > Red Blood Cell Disorders II-Usera > Flashcards

Red Blood Cell Disorders II-Usera Flashcards

1
Q

What are the five types of normocytic anemia?

A
  • acute blood loss
  • early iron deficiency and ACD
  • aplastic anemia
  • chronic renal failure
  • malignancy
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2
Q

What are the intrinsic defect causes of normocytic anemia?

A
  • membrane defect
  • abnormal HB
  • enzyme deficiency
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3
Q

What are the extrinsic defect causes of normocytic anemia?

A

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

immune destruction

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4
Q

How come you get extravascular hemolysis?

A

RBC phagocytosis by splenic and hepatic macrophages due to IgG bound with or without complement, or abnormal shape (e.g. spherocytosis, sickling)

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5
Q

What are the laboratory findings of normoncytic anemia?

A

increase unconjugates bilirubin

increased serum LDH

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6
Q

How come you get intravascular hemolysis?

A

RBC phagocytosis by splenic and hepatic macrophages

-hemolysis occurs within blood vessels (enzyme deficiency G6PD, mechanical trauma, complement/immune destruction)

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7
Q

What are the lab findings in intravascular hemolysis?

A
  • increase unconjugates bilirubin (minimal)
  • increased serum LDH
  • decreased serum haptoglobin
  • hemosiderinuria
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8
Q

(Blank) is an acute phase reactant that complexes with Hb?

A

haptoglobin

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9
Q

Is herediatary spherocytosis autosomal dominant or recessive?

A

autosomal dominant

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10
Q

What causes hereditary spherocytosis?

A

intrinisic defect caused by mutation in ankyrin Howvere can have problems in Band 3, spectrins, protein 4.2

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11
Q

What are the clinical features of hereditary spherocytosis?

A

jaundice
gallstones
splenomegaly
aplastic crisis in children

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12
Q

What are the lab findings of hereditary spherocytosis?

A

normocytic anemia
increase MCHC
increase osmotic fragility (rupture in hypotonic solutions)
elevated LDH and Bili

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13
Q

Are all spherocytes due to genetic diseases?

A

no

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14
Q

What are 2 non genetic causes of sphereocytes?

A

warm autoimmune hemolytic anemia

ABO incompatibility

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15
Q

How do you treat spherocytosis?

A

splenectomy.

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16
Q

How come in spherocytosis, MCV may vary?

A

due to reticulocytosis

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17
Q 
What is this:
autosomal dominant
defect in spectrin tetramers or 4.1
mild anemia
splenomegaly
A

Hereditary ellipotcytosis

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18
Q

What can hereditary ellipotcytosis cause?

A

mild anemia

splenomegaly

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19
Q

What percent of your peripheral blood must be elliptocytes to be considered hereditary elliptocytosis?

A

greater than 25%

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20
Q

How do you get sickle cell anemia?

A

autosomal recessive missense mutation

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21
Q

What amino acid causes sickle cell?

A

valine substitution for glutamic acid in beta-globin chain

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22
Q

A lot of people (mainly A.A) are heterozygotes for sickle cell anemia but are not (blank)

A

anemic

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23
Q

If you have more (blank) HBS is threshold for sickle cell anemia

A

60%

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24
Q

(blank) concentrations prevent sickling in newborns for 5-6 months.

A

HbF

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25
Q

What are the clinical findings of sickle cell anemia?

A 
dactyitis
acute chest syndrome (most common cause of death)
stroke
gallstones
priapism
aseptic necrosis of femoral head
aplastic crisis (ass. w/ parvovirus)
autosplenectomy (splenomegaly by 2 years, then loses function)
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26
Q

Where do you see howel-jolly bodies?

A

sickle cell anemia

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27
Q

Why do you get suscpetibility to infections with sickle cell anemia?

A

due to dysfunctional spleen

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28
Q

What are some infections often associated with sickle cell anemia?

A

strep pneumonia, other encapsulated organisms

osteomyelitis by salmonella paratyphi

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29
Q

What are the renal findings in sickle cell anemia?

A

may occur in peritubular capillaries
microhematuria
renal papillary necrosis

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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
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31
Q

What are some preventive measures for sickle cell anemia?

A
  • hydroxyurea
  • immunizations
  • pneumococcal vaccine
  • folic acid supplementation
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32
Q

How do you get G6PD deficiency?

A

recessive x linked pattern

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33
Q

In G6PD deficiency which RBCs appear normal and which dont?
Does it affect men or women more?
whites or blacks?

A 
young RBCs-normal
old-affected
females-asymptomatic
males-symptomatic
affects 22% of blacks
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34
Q

There are over 100 genetic variations of G6PD deficiency but most represent (blank) substitutions

A

amino acid

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35
Q

(blank) protects RBCs and their membranes from oxidants

A

Glutathione (reduced state)

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36
Q

If you dont have G6PD (thus no glutathione) you will get (blank) and thus hemolysis

A

oxidative damage

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37
Q

What are some inciting causes of G6PD?

A

infection

drugs; primaquine, sulfas

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38
Q

If you have G6PD then you will have decreased (blank) throughout life span of RBCs

A

enzyme activity

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39
Q

In G6PD you get accumulation of (blank)

A

H202

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40
Q

What does accumulating H202 do?

A

injuries RBC and results in hemolysis

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41
Q

What will you see in G6PD mediterranean ?

A

heinz bodies-> oxidized hemoglobin precipitates

Bite cells-> macrophage removal of damaged membrane

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42
Q

What are the clinical and lab findings of G6PD?

A
  • sudden onset back pain and delayed hemoglobinuria
  • suscpetibility to infections (impaired MPO-No NADHPH)
  • normocytic anemia
  • Heinz bodies
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43
Q

What is immunohemolytic anemia?

A

extrinisc hemolytic anemia with intra- or extravascular hemolysis
caused by antibody mediated destruction of RBCs (IgG or IgM)

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44
Q

What will you find in immunohemolytic anemia?

A 
warm agglutinins (70% in IgG)
cold agglutinins (30% in IgM)
complement
drug induced
paroxysmal cold hemoglobinuria (PCH)
45
Q

In immunohemolytic anemia (blank) mediated diseases usually involves extravascular hemolysis

A

IgG

46
Q

What is the most common hemolytic anemia?

A

extravascular immunohemolytic anemia (IgG0

47
Q

In immunohemolytic anemia IgG RBCs are phagocytosed by (blank)

A

splenic macrophages

48
Q

(blank) percent of immunohemoytic anemias are idiotpathic (arise spontaneously)

A

50%

49
Q

What diseases are immunohemolytic anemia associated with?

A

SLE
Collagen Vascular diseases (SLE)
CLL
Malignant lymphome (Esp hodgkins), viral infections etc

50
Q

Where do you see cold agglutinins in immunohemolytic anemia?

A

IgM (30%) immunohemolytic anemia

51
Q

What causes IgM immunohemolytic anemia?

A

mycopasma pneumoniae, infectious mononucleosis, CLL, drugs

52
Q

Does IgM immunohemolytic anemia cause intravascular or extravascular hemolysis?

A

either or :)

53
Q

What phenomenon do you commonly see in immunohemolytic anemia?

A

Raynauds phenomenon

54
Q

What is complement mediated hemolysis caused by immunohemmolytic anemia?

A
  • C3B coated RBC are phagocytosed by liver macrophages-extravascular
  • RBCs coated by C5-C9 membrane attack complex-intravascular
55
Q

What are the clinical findings of immune hemolytic anemia?

A

jaundice
hepatosplenomegaly
Raynauds phenomenon

56
Q

What are the lab findings for immune hemolytic anemia?

A 
Positive dat
Positive indirect
Unconjugated hyperbilirubinemia
Hemoglobinuria
Decreased haptoglobin
Normocytic anemia
Rbc agglutination (igm)
57
Q

What is drug induced IHA?

A

antibody (IgG) to drug binds to RBC membrane

58
Q

What is a drug that can induce IHA and what do you see in this?

A

penicilin
-extravascular hemolysis
-direct coombs positive
“innocent bystanders”

59
Q

What are the drugs that can cause drug-antidrug immune complexes?

A

quinidine, quinine, INH, sulfonamides

60
Q

Igm drug-antidrug immune complex results in (Blank) hemolysis

A

intravascular

61
Q

IgG drug-anti-drug immune complex results in (blank) hemolysis

A

extravascular

62
Q

How do you treat drug induced IHA?

A

discontinue offending drug

  • corticosteroids/immunsuppresion
  • splenectomy
  • IVIG-IgG coated macrophages can not phagocytize RBCs
63
Q

What is this:

acquired membrane defect in myeloid stem cells-mutation in Pig-A gene.

A

Paroxysmal nocturnal hemoglobinuria

64
Q

What does the Pig A gene mutation in paroxysmal nocturanl hemoglobinuria cause?

A

loss of decay accelerating factor (DAF)

65
Q

What happens if you lose DAF (when mutated)?

A

you cant destabilize compliment so you cant lyse blood cells and they end up in urine.

66
Q

What does IVIG do?

A

Give IgG via IV which will coat macrophages so they are unable to phagocytize RBCs

67
Q

In paraoxsmal nocturnal hemoglobinuria you cant activate the (blank)

A

membrane attack complex

68
Q

Why do you call it paroxysmal NOCTURNAL hemoglobinuria?

A

because it happens in periods of acidosis which usually occurs during sleeping hence nocturnal

69
Q

(blank) is a clonal stem cell disorder so there is increased risk for other clonal disorders such as AML

A

Paroxysmal nocturnal hemoglobinuria

70
Q

What is this:

  • episodic hemoglobinuria
  • may lead to iron def
  • increased risk of thrombosis
  • increased risk of AML
A

Paroxysmal nocturnal hemoglobinuria

71
Q

What do the labs look like in paroxysmal nocturnal hemoglobinuria?

A
  • normocytic anemia with pancytopenia
  • decreased haptoglobin
  • increased serum/urin HB
72
Q

(blank) are when you have a whole range of abnormal shaped cells

A

schistocytes

73
Q

What will you see in traumatic hemolysis?

A

cell fragments: shcistocytes, burr cells, helmet cells

74
Q

Why do you get disseminated intravascular coagulation (DIC)?

A

RBCs damaged by fibrin buildup or clots in small vessel lumen

75
Q

What are some causes of traumatic hemolysis?

A 
long distance running
artificial heart valves
thrombotic thrombocytopenia (purpura)
76
Q 
What is this:
hypersplenism
microangiopathic hemolytic anemia (MAHA)
Common in patients with malignant hypertension 
microorganisms, i.e. malaria, babesia, Clostridium perfringens
snake venoms, i.e. cobra venom
chemical, i.e. plumbism (lead poisoning)
physical, i.e. burns
A

non-immunologic hemolytic anemia

77
Q

A transfusion reaction can cause (blank) hemolytic anemia

A

alloimmune

donor and recipient blood is incompatible

78
Q

What are the lab findings of alloimmune hemolytic anemia?

A
  • DAT
  • anemia depending on severity
  • total and indirect bilirubin elevated
  • haptoglobin decreased
  • increased LDH
79
Q

How can newborns get an alloimmune anemia (called hemolytic disease of the newborn)?
What does this result in?

A

maternal antibodies greater than babys RBCs

Anemia and hyperbilirubinemia

80
Q

What are the lab findings hemolytic disease of the newborn?

A
  • positive DAT
  • increased total and indirect bilirubin
  • increased LDH
  • initially hematocrit and hemoglobin may be within normal limits
81
Q

Renal disease is associated with anemia. What will the RBCs look like with renal disease?

A

Normochromic, normocytic
mild anisocytosis
sometimes hypochromic, microcytic
burr cells – shrunken RBCs with irregular projections (echinocytes)

82
Q

When do you usually see anemia with renal disease?

A

when BUN is twice the normal

83
Q

What are the possible mechanisms for anemia occurring in renal disease?

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
84
Q

WHat do the RBCs look like with anemia with neoplasia?

A

normochromic and normocytic UNLESS there is blood loss, hemorrhage, a myelopthisic process (affects the bone marrow)

85
Q

There is a mild (blank) component often present with neoplasia

A

anemic

86
Q

THe hemolytic component associated with neoplasm can be severe with (blank) possibly due to the altered endothelium of malignant tissues (a set up for DIC)

A

lymphomas

87
Q

(blank) can be associated with infection

A

hemolytic anemia

88
Q

What are some infections that can cause hemolytic anemia?

A 
bacterial toxins (c. perfringens)
malaria
89
Q

WHat are the 2 types of macrocytic anemias?

A

megaloblastic (folate or B12 deficiency)

Nonmegaloblastic (alcoholism)

90
Q

What is this:

large nucleus in erythroid precursors (have a more mature cytoplasm than the nucleus i.e nuclear cytoplasmic dysynchrony

A

megaloblastic

91
Q

What are the causes of B12 deficiency?

A 
Pernicious anemia 
(autoimmune destruction of parietal cells )
Pure vegan diet
Malnutrition
Malabsorption
↓ Intrinsic factor
↓ Gastric acid
↓ Intestinal absorption (chrone’s disease)
92
Q

(blank) cells in the stomach release intrinsic factor (and HCL) which is required for B12 absorption in the terminal (blank)

A

parietal

ileum

93
Q

Folate is absorbed in the (blank) which can be affected by celiac disease

A

jejunum

94
Q

What can cause folate deficiency?

A

decreased intake
malabsorption
durg inhibition

95
Q

What are the reasons for decreased intake in folate deficiency?

A

Malnutrition
Etoh (inhibits absorption of folate)
Goat milk
Infants/elderly

96
Q

What are the reasons for malabsorption in folate deficiency?

A

celiac

bacterial overgrowth

97
Q

What are the reasons for drug inhibition in folate deficiency?

A 
5-FU-chemotherapy
MTX-chemotherapy
Tpm-sfx
Phenytoin
Ocp’s
etoh
98
Q

What is the pathogenesis of megaloblastic anemia?

A

Delayed nuclear maturation
Affects all rapidly dividing cells
Cellular RNA and protein synthesis unabated

99
Q

If you dont have folate or vit B12 what will you have increase of?

A

homocysteine

100
Q

What happens if you have elevated homocysteine?

A

damaged to vascular endothelial cells

101
Q

How can you get pernicious anemia?

A

Type II hypersensitivity, Blood group A individuals, Achloryhydria (decreased/no production of HCL)

102
Q

What is this:
Autoimmune destruction of parietal cells (85-90%)
Antibodies that block B12-if binding (60-75%)

A

pernicious anemia

103
Q

What are signs of pernicious anemia?

A

smooth/sore tongue
peripheral neuropathy
dementia
decreased vibratory and proprioception

104
Q

Peripheral neuropathy is associated with (B12/folate) deficiency

A

B12 ONLY!!!!! (due to demyleination of dorsal columns affecting propioception and TVP)

105
Q

What is this:

decreased serum and RBC folate (best test)

A

folate deficiency

106
Q

What is the treatment of folate deficiency?

A

Intramuscular injections of b12 (to cure B12 def. not folate)
Oral administration of monoglutamic folic acid

107
Q 
WHat is this:
Mcv 105 ±10 
Anemia may not necessarily be present
Etoh most common cause
Liver disease (increased cholesterol)
A

Nonmegaloblastic macrocytosis

108
Q

Does nonmegaloblastic macrocytosis have a problem with maturation of bone marrow?

A

no just have large cells

109
Q

Why does nonmegaloblastic have large cell size?

A

due to increased cholesterol in the membrane because of liver disease-target cell appearance due to presence of more membrane (due to cholesterol)

Block 7 Week 3 (4 decks)

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