Heme Flashcards

1
Q

term for -

RBC’s that stick together like a stack of coins due to increased plasma proteins (immunoglobulins)

A

Roluleaux formation

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2
Q

Roluleaux formation is seen in what disease

A

Multiple Myeloma

or increase fibrin disorder like infection or inflammatory autoimmune disease

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3
Q

Term for:

Clumping of RBC’s due to IgM coating the surface leading to RBC destruction by macrophages

A

Cold agglutination autoimmune hemolytic anemia

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4
Q

Cold agglutination autoimmune hemolytic anemia is seen in what condition ?

A

Mononucleosis

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5
Q

Key terms for this diagnosis- Spherocytes with negative coomb’s test
+ osmotic fragility test

A

Herditary spherocytosis

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6
Q

Key terms for this diagnosis- Spherocytes with positive coomb’s test

A

Warm autoimmune hemolytic anemia

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7
Q

Impaired DNA synthesis with normal RNA synthesis will increase cytoplasmic proteins leading to what cell line?

A

Macrocytosis- with hypersegmented neutrophils

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8
Q

Key terms for this diagnosis- Marocytosis with hypersegmented neutrophils

A

B12 deficiency
liver disease
if all cell lines down- aplastic anemia

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9
Q

name all 3 micocytic anemia’s

A
  1. Iron deficiency
  2. Thalassemia
  3. lead poisoning
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10
Q

What is a small dense basophilic RBC inclusion

A

Howell-Jolly Bodies

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11
Q

Key terms for this diagnosis- Howell-Jolley Bodies

A

Megaloblastic anemias
hypersplenism
Severe hemolytic anemia

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12
Q

Denatured Hgb in cells

A

Heniz cells

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13
Q

Key terms for this diagnosis-Heniz cells

A

G6PD

Thalassemia

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14
Q

deformity in cell due to phagocyte removal of denated hgb

A

Bite cell

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15
Q

Key terms for this diagnosis- Bite cells

A
  • same as Heniz bodies because a bite cell is a Heniz body the hgb just got removed
    G6PD
    Thalassemia
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16
Q

Key terms for this diagnosis- schistocytes and keratocytes

A

Mechanical RBC damage in small vessles
microangiopathic hemolytic anemia
like TTP, HUS, DIC

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17
Q

Key terms for this diagnosis- Target cells

A

Severe Iron defiency
Sickle cell
thalassemia
Hgb S or C

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18
Q

Key terms for this diagnosis-Tear shaped cells

A

Thalassemia

hemolytic anemia

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19
Q

Key terms for this diagnosis-Basophilic Stippling

A

Lead poisoning

sideroblastic anemia- heavy metal posions

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20
Q

Key terms for this diagnosis- Echinocytes

A

aka burr cells
Uremia
Pyruvate kinase deficiency

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21
Q

Key terms for this diagnosis- Acanthocytes

A

Spur cells
liver disease like alcoholics
renal disease
autoimmune hemolytic anemia

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22
Q

Key terms for this diagnosis- Reticulocytes

A

Seen with blood loss

hemolytic anemia

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23
Q

30-40% of WBC’s

what is their function

A

Lymphocytes

viral infections

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24
Q

Key terms for this diagnosis- atypical lymphocytes

A

infectious mononucleosis- EBV

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25
Q

40-70% of WBC, and are multilobular

A

Neutrophils
infections, steroid use
reaction to gram neg sepsis

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26
Q

When are bands seen ?

A

Acute bacterial infection

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27
Q

when would basophils be increased

A

hypersentitivy reaction

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28
Q

when would monocytes be increased

A

viral infections

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29
Q

when would esoinophils be increased

A

parasitic infections

allergic reactions

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30
Q

an increase transferrin and increased TIBC

would be what disorder ?

A

Iron defiency

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31
Q

an decreased transferrin and decreased TIBC

would be what disorder ?

A

anemia of chronic diease

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32
Q

Ferritin is what?

A

Stored Iron

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33
Q

Decreased Ferritin would be what disorder?

A

Iron defiency - the body is using the stores first

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34
Q

an increased ferritin would be what disorder?

A

anemia of chronic disease

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35
Q

Is haptoglobin increased or decreased in iron deficency

A

it is decreased- it binds free hemoglobin
to reduce iron availability for microbes
seen in hemolytic anemias

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36
Q

How can you determine between Iron deficiency anemia and anemia of chronic disease ?

A

Ferritin and TIBC levels
Iron = Ferritin decrease, TIBC increased
anemia of chronic = Ferritin increased TIBC decreased

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37
Q

what disease is seen with Iron overload?

A

Hemochromatosis

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38
Q
what are these doing in hemolytic anemia
LDH
Bilirubin
Haptoglobin
smear cells ?
A

LDH is increased- it is usually inside the cell
Increase bilirubin is increased - jaundice
Haptoglobin is decrease- it is binding free hemoglobin
Schisotyctes and reticulocytosis

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39
Q

what is the best diagnostic test for sickle cell ?

A

Electrophoresis- Hgb S

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40
Q

Key terms for this diagnosis- MCV <80, increase serum iron, hemoglbin eletrophoresis with normal hemoglobin rations of Hgb A, A2 and F

A

Alpha Thalassemia

- blood smear is usually normal this is a diagnosis of exclusion

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41
Q

Key terms for this diagnosis- MCV <80, increase serum iron, hemoglbin eletrophoresis with decreased Hgb A, increase Hgb A2 and Hgb F

A

Beta Thalassemia

42
Q

Key terms for this diagnosis- episodic hemotlyic anemia with sulfa drugs, infection and oxidizing agents

A

G6PD

43
Q

what is the difference between TTP and HUS

A

Both ave Thrombocytopenia, hemolytic anemia and kidney damage
TTP has neuro and fevers
HUS more likely in children and to cause kidney involvement

44
Q

Key terms for this anemia- pallor, glossitis, stomatitis, peripheral neruopathy, weakness, atxasia, decrease deep tendon reflexes

A

Macrocytic anemia
B12 deficiency
folate is more common but does not have neruo s/s

45
Q

Homocysteine
Methoylmalonic acid
are see in what disorder?
Increased or decreased?

A

B12 deficiency
Homocysteine increased
Methoylmalonic acid increased

46
Q

what is the test for pernicious anemia

A

schilling test

47
Q

Treatment for B12 deficiency

A
  1. IM B12

watch for hypokalemia

48
Q

What will happen if you give a B12 deficiency patient Folate therapy treatment without B12 replacement?

A

Neuro symptoms will worsen

49
Q

what are other reasons for Macrocytic anemias besides B12 and folate

A

Alcohol abuse
liver disease
hypothyroidism

50
Q

what is the most common cause of Iron Deficiency anemia

A

Bleeding
menstration
colon cancer
parastic hook worms

51
Q

what are s/s of Iron Deficiency anemia

A

pagophagia- ice craving
pica
angular cheilitis
koilonychia

52
Q

Key terms for this diagnosis- iron deficiency with difficulty swallowing, esophageal webs and atrophic glossitis

A

Iron Deficiency anemia

Plummer Vinson syndrome

53
Q
Labs for Iron Deficiency anemia
MCV 
Serum Iron 
Ferritin
TIBC
RDW 
HBG/HCT 
Transferrin saturation %
A
MCV - low 
Serum Iron- low  
Ferritin- low 
TIBC- increase 
RDW - increase 
HBG/HCT- low 
Transferrin saturation decrease%
54
Q

Treatment for Iron Deficiency anemia

A

Iron pills

can cause constipation so go slow

55
Q

Key terms for this diagnosis- Microcytic anemia with normal iron levels

A

Alpha or Beta Thalassemia

56
Q

key terms for this diangosis - severe anemia, heniz sisbodies, hepatospleomegaly, microcytic hemolytic anemia, fonrtal skull and maxialla overgrowth, bone fractures and gallstones

A

Alpha T intermedia (Hgb H diesease)

- - / - A

57
Q

key terms for this diagnosis- associated with stillbirth or death soon after from high output failure

A

Alpha thalassemia major- hydrops fetalis

- - / - -

58
Q

how can you tell between alpha and beta thalassemia

A

the electrophoesis

alpha has normal Hgb ratios

59
Q

Treatment for severe alpha thalassemia

A
  1. folate
  2. blood transufsions weekly
  3. iron chelation agents- defroxamine
  4. bone marrow transplant
60
Q

what is Defroxamine for?

A

prevents iron overload and removes excess iron form diseases that need transfusions like
thalassemai’s

61
Q

what races is thalassemia most common in ?

A

alpha- asain, Mediterranean (greek, italian)

beta- Mediterranea (greek, italian)

62
Q

what is the most common type of beta thalassemia

A

B- Minor only one gene is defective

when you dont make the beta your will make more alpha

63
Q

What is Cooley’s Anemia

A

beta thalassemia Major both B genes are mutated

leading to more alpha

64
Q

Key terms for this diagnosis- At birth child has no s/s of anemia, at 6 months = jaundice, dyspena, pallor and osteopenia frontal bossing, iron overload and gall stones

A

beta thalassemia Major - Cooley’s anemia

patients are normal at birth because they have fetal hgb F making up for the missing beta chains

65
Q

What will the eletrophoresis of Beta thalassemia show?

A

Minor= HgbA2 and or Hgb F increased (no beta)
Major= HgbA2 and 90% Hgb F
little or no hgb A

66
Q

Treatment for Beta thalassemia

A
  1. minor- no treatment needed
  2. major- transusions weekly
  3. Deferoxamine for chelation therapy
  4. bone marrow transplant
67
Q
Labs for this diagnosis
MCV- Low 
RDW- Normal 
Ferritin- Normal 
Serum Iron- Normal 
Hgb- normal Hgb A, A2 and F
A

Alpha thalassemia

*remember their iron stores are not affected

68
Q
Labs for this diagnosis
MCV- Low 
RDW- Normal 
Ferritin- Normal 
Serum Iron- Normal 
Hgb- increased Hgb A, A2 and F
A

Beta thalassemia

  • remember their iron stores are not affected
  • remember that the Hgb beta are not present so they get replaced with other Hgb like A, A2 and F
69
Q

Key terms for this diangosis- child with abd pain, consitpation, numbness tingling, ataxia, fatigue, learning disabilities, coma and shock

A

Lead poisoning- sideroblastic anemia

70
Q

what can you seen on x-ray of lead posioning

A

Lead lines
linear hyperdensities of metaphyseal plates
in adults can be in their gums

71
Q

What are the 3 normocytic anemias

A
  1. anemia of chronic disease
  2. G6PD
  3. Sickle cell
72
Q

key terms for this diagnosis- normocytic anemia, with increased ferritin, decreased TIBC and decreased iron

A

anemia of chronic disease

73
Q

Treatment for anemia of chronic disease

A

treat underlying cause

if its kidney give them erythropoietin

74
Q

what is an X-linked recessive disorder primary affecting males mostly African Americans

A

G6PD

75
Q

what is G6PD in the body for?

A

Glucose 6 phosphate dehydrogenase is a enzyme that protects against oxidation stress
if oxidized it gets turned into methemoglobin which doesn’t carry oxygen well
hgb on the cell gets denatured into heniz bodies
and are destroy by the spleen
leading to hemolytic anemia

76
Q

what is an episode of G6PD s/s ?

A

back or abd pain with anemia
jaundice and splenomegaly
can lead to hemolytic crisis
rbc’s will rupture under oxidative stress

77
Q

what can cause oxidative stress?

A
Infection- DKA, pneumonia
Fava beans
Sulfa drugs- bactrum 
antimalarials 
meythlene blue 
fluoroquinoloes
nitrofurantoin
asprin
dapsone
78
Q

Treatment for G6PD

A

usually self limiting
avoid whatever caused it
iron supplements
blood transfusions if severe

79
Q

What is an autosomal recessive genetic disorder of Hgb SS. Valine is subsituted for what?

A

Sickle cells

Valine in place of Glutamic Acid on beta chain

80
Q

what is one benefit of Sickle cell trait?

A

AA are resistant to plasmodium falciparum - malaria

81
Q

what are some s/s of sickle cell disease

A
sickle under hypoxic conditions 
cause micro thrombosis
hemolytic anemia 
hematuria 
painful occlusive crises
82
Q

what is the most common presenting symptoms in infants with sickle cell

A

Dactylitis - swelling of fingers and toes

Hgb SS replaces fetal hemoglobin

83
Q

What are some infections seen with sickle cell

A
  1. osteomylitis- salmonella
  2. risk of encasulated organism like S. pneumonia, H.flu
  3. Aplastic crisis with parvovirus B19
84
Q

What are signs of microthrombosis or infractions with sickle cell disease

A
1. necrossi of the bones - humeral head 
H-shaped vertebrae 
2. acute splenomegaly- clots are getting suck in organs
3. Skin ulcers on the tibia* 
4. Stroke in a child
85
Q

what are sickle cells crises caused by?

what happens?

A
Cells are fragile and sickle under 'stress' leading to micro infractions in ograns
triggers- cold weather, hypoxia, infeciton, dehydration, alcohol, pregnancy 
acute onset of pain 
acute chest syndrome 
back 
abd pain 
bone pain 
priapism***
86
Q

what will show with sickle cell hemoglobin eletrophoresis

A
Hgb S increased 
Hgb F increased
NO Hgb A 
low Hgb and Hct 
on smear- target cells and sickled cells
Howell-jolly bodies- if not spleen
87
Q

Treatment for Sickle cell disease

A
  1. pain control - Mepridine (Demerol)
  2. Iv hydration
  3. Oxygen
  4. Hydroxyurea
  5. +/- transfusions
  6. Stem cell transplant only possible cure - serious side affects
88
Q

Hydroxyurea is used to treat what?

A

sickle cell disease with painful crisis

reduces the frequency of painful crisis

89
Q

What is important to do in children with sickle cell ?

A
Immunizaitons !!! SHIN! 
S. pneumonia
H. Influenza
Inlfuenzae B and N 
menigococcus
any encapsulated organisms
90
Q

Key terms for this diagnosis- autosomal dominant intrinsic hemolytic anemia causing a defect RBC membrane and increase cell fragility

A

Hereditary Spherocytosis

91
Q

key terms for this diagnosis- + osmotic fragility test with negative coombs

A

Hereditary Spherocytosis

92
Q

Treatment for Hereditary Spherocytosis

A

folate- helps stablize the membrane

spelenectomy in severe disease

93
Q

key terms for this diagnosis- Antibodies to own RBC’s surface leading to destruction by macrophages and the spleen.

A

Autoimmune hemolytic anemia - AIHA

94
Q

What other disorders are common with Autoimmune hemolytic anemia - AIHA

A

Systemic Lupus*
Rheumatoid arthritis
Chronic lymphocytic leukemia

95
Q

key terms for this diangosis- IgM Antibodies, Direct coombs tests +, microspherocytes

A

Autoimmune hemolytic anemia - AIHA

96
Q

Treatment for Autoimmune hemolytic anemia - AIHA

A
  1. warm steroids
  2. Splenectomy
  3. avoid cold exposure
97
Q

Key terms for this diagnosis- rare aquired stem cell mutation that activates complemnt destruction leading to thrombosis. Dark cola-colored urine during the night or early morning with venous clots

A

paroxysmal notctural hemoglobinuria

98
Q

What type of anemia is paroxysmal notctural hemoglobinuria

A

Hemolytic anemia
the protein that is suppose to protect it from complement breakdown is missing
so it gets SLICED!

99
Q

what is the best test for paroxysmal notctural hemoglobinuria

A

Flow Cytometry
will be osmotic fragility -
coombs -

100
Q

Treatment for paroxysmal notctural hemoglobinuria

A
  1. Eculizumab

2. steroids