Heme Flashcards
(100 cards)
1
Q
term for -
RBC’s that stick together like a stack of coins due to increased plasma proteins (immunoglobulins)
A
Roluleaux formation
2
Q
Roluleaux formation is seen in what disease
A
Multiple Myeloma
or increase fibrin disorder like infection or inflammatory autoimmune disease
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
4
Q
Cold agglutination autoimmune hemolytic anemia is seen in what condition ?
A
Mononucleosis
5
Q
Key terms for this diagnosis- Spherocytes with negative coomb’s test
+ osmotic fragility test
A
Herditary spherocytosis
6
Q
Key terms for this diagnosis- Spherocytes with positive coomb’s test
A
Warm autoimmune hemolytic anemia
7
Q
Impaired DNA synthesis with normal RNA synthesis will increase cytoplasmic proteins leading to what cell line?
A
Macrocytosis- with hypersegmented neutrophils
8
Q
Key terms for this diagnosis- Marocytosis with hypersegmented neutrophils
A
B12 deficiency
liver disease
if all cell lines down- aplastic anemia
9
Q
name all 3 micocytic anemia’s
A
- Iron deficiency
- Thalassemia
- lead poisoning
10
Q
What is a small dense basophilic RBC inclusion
A
Howell-Jolly Bodies
11
Q
Key terms for this diagnosis- Howell-Jolley Bodies
A
Megaloblastic anemias
hypersplenism
Severe hemolytic anemia
12
Q
Denatured Hgb in cells
A
Heniz cells
13
Q
Key terms for this diagnosis-Heniz cells
A
G6PD
Thalassemia
14
Q
deformity in cell due to phagocyte removal of denated hgb
A
Bite cell
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
16
Q
Key terms for this diagnosis- schistocytes and keratocytes
A
Mechanical RBC damage in small vessles
microangiopathic hemolytic anemia
like TTP, HUS, DIC
17
Q
Key terms for this diagnosis- Target cells
A
Severe Iron defiency
Sickle cell
thalassemia
Hgb S or C
18
Q
Key terms for this diagnosis-Tear shaped cells
A
Thalassemia
hemolytic anemia
19
Q
Key terms for this diagnosis-Basophilic Stippling
A
Lead poisoning
sideroblastic anemia- heavy metal posions
20
Q
Key terms for this diagnosis- Echinocytes
A
aka burr cells
Uremia
Pyruvate kinase deficiency
21
Q
Key terms for this diagnosis- Acanthocytes
A
Spur cells
liver disease like alcoholics
renal disease
autoimmune hemolytic anemia
22
Q
Key terms for this diagnosis- Reticulocytes
A
Seen with blood loss
hemolytic anemia
23
Q
30-40% of WBC’s
what is their function
A
Lymphocytes
viral infections
24
Q
Key terms for this diagnosis- atypical lymphocytes
A
infectious mononucleosis- EBV
25
40-70% of WBC, and are multilobular
Neutrophils
infections, steroid use
reaction to gram neg sepsis
26
When are bands seen ?
Acute bacterial infection
27
when would basophils be increased
hypersentitivy reaction
28
when would monocytes be increased
viral infections
29
when would esoinophils be increased
parasitic infections
| allergic reactions
30
an increase transferrin and increased TIBC
| would be what disorder ?
Iron defiency
31
an decreased transferrin and decreased TIBC
| would be what disorder ?
anemia of chronic diease
32
Ferritin is what?
Stored Iron
33
Decreased Ferritin would be what disorder?
Iron defiency - the body is using the stores first
34
an increased ferritin would be what disorder?
anemia of chronic disease
35
Is haptoglobin increased or decreased in iron deficency
it is decreased- it binds free hemoglobin
to reduce iron availability for microbes
seen in hemolytic anemias
36
How can you determine between Iron deficiency anemia and anemia of chronic disease ?
Ferritin and TIBC levels
Iron = Ferritin decrease, TIBC increased
anemia of chronic = Ferritin increased TIBC decreased
37
what disease is seen with Iron overload?
Hemochromatosis
38
```
what are these doing in hemolytic anemia
LDH
Bilirubin
Haptoglobin
smear cells ?
```
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
39
what is the best diagnostic test for sickle cell ?
Electrophoresis- Hgb S
40
Key terms for this diagnosis- MCV <80, increase serum iron, hemoglbin eletrophoresis with normal hemoglobin rations of Hgb A, A2 and F
Alpha Thalassemia
| - blood smear is usually normal this is a diagnosis of exclusion
41
Key terms for this diagnosis- MCV <80, increase serum iron, hemoglbin eletrophoresis with decreased Hgb A, increase Hgb A2 and Hgb F
Beta Thalassemia
42
Key terms for this diagnosis- episodic hemotlyic anemia with sulfa drugs, infection and oxidizing agents
G6PD
43
what is the difference between TTP and HUS
Both ave Thrombocytopenia, hemolytic anemia and kidney damage
TTP has neuro and fevers
HUS more likely in children and to cause kidney involvement
44
Key terms for this anemia- pallor, glossitis, stomatitis, peripheral neruopathy, weakness, atxasia, decrease deep tendon reflexes
Macrocytic anemia
B12 deficiency
folate is more common but does not have neruo s/s
45
Homocysteine
Methoylmalonic acid
are see in what disorder?
Increased or decreased?
B12 deficiency
Homocysteine increased
Methoylmalonic acid increased
46
what is the test for pernicious anemia
schilling test
47
Treatment for B12 deficiency
1. IM B12
| watch for hypokalemia
48
What will happen if you give a B12 deficiency patient Folate therapy treatment without B12 replacement?
Neuro symptoms will worsen
49
what are other reasons for Macrocytic anemias besides B12 and folate
Alcohol abuse
liver disease
hypothyroidism
50
what is the most common cause of Iron Deficiency anemia
Bleeding
menstration
colon cancer
parastic hook worms
51
what are s/s of Iron Deficiency anemia
pagophagia- ice craving
pica
angular cheilitis
koilonychia
52
Key terms for this diagnosis- iron deficiency with difficulty swallowing, esophageal webs and atrophic glossitis
Iron Deficiency anemia
| Plummer Vinson syndrome
53
```
Labs for Iron Deficiency anemia
MCV
Serum Iron
Ferritin
TIBC
RDW
HBG/HCT
Transferrin saturation %
```
```
MCV - low
Serum Iron- low
Ferritin- low
TIBC- increase
RDW - increase
HBG/HCT- low
Transferrin saturation decrease%
```
54
Treatment for Iron Deficiency anemia
Iron pills
| can cause constipation so go slow
55
Key terms for this diagnosis- Microcytic anemia with normal iron levels
Alpha or Beta Thalassemia
56
key terms for this diangosis - severe anemia, heniz sisbodies, hepatospleomegaly, microcytic hemolytic anemia, fonrtal skull and maxialla overgrowth, bone fractures and gallstones
Alpha T intermedia (Hgb H diesease)
| - - / - A
57
key terms for this diagnosis- associated with stillbirth or death soon after from high output failure
Alpha thalassemia major- hydrops fetalis
| - - / - -
58
how can you tell between alpha and beta thalassemia
the electrophoesis
| alpha has normal Hgb ratios
59
Treatment for severe alpha thalassemia
1. folate
2. blood transufsions weekly
3. iron chelation agents- defroxamine
4. bone marrow transplant
60
what is Defroxamine for?
prevents iron overload and removes excess iron form diseases that need transfusions like
thalassemai's
61
what races is thalassemia most common in ?
alpha- asain, Mediterranean (greek, italian)
| beta- Mediterranea (greek, italian)
62
what is the most common type of beta thalassemia
B- Minor only one gene is defective
| when you dont make the beta your will make more alpha
63
What is Cooley's Anemia
beta thalassemia Major both B genes are mutated
| leading to more alpha
64
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
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
What will the eletrophoresis of Beta thalassemia show?
Minor= HgbA2 and or Hgb F increased (no beta)
Major= HgbA2 and 90% Hgb F
little or no hgb A
66
Treatment for Beta thalassemia
1. minor- no treatment needed
2. major- transusions weekly
3. Deferoxamine for chelation therapy
4. bone marrow transplant
67
```
Labs for this diagnosis
MCV- Low
RDW- Normal
Ferritin- Normal
Serum Iron- Normal
Hgb- normal Hgb A, A2 and F
```
Alpha thalassemia
| *remember their iron stores are not affected
68
```
Labs for this diagnosis
MCV- Low
RDW- Normal
Ferritin- Normal
Serum Iron- Normal
Hgb- increased Hgb A, A2 and F
```
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
Key terms for this diangosis- child with abd pain, consitpation, numbness tingling, ataxia, fatigue, learning disabilities, coma and shock
Lead poisoning- sideroblastic anemia
70
what can you seen on x-ray of lead posioning
Lead lines
linear hyperdensities of metaphyseal plates
in adults can be in their gums
71
What are the 3 normocytic anemias
1. anemia of chronic disease
2. G6PD
3. Sickle cell
72
key terms for this diagnosis- normocytic anemia, with increased ferritin, decreased TIBC and decreased iron
anemia of chronic disease
73
Treatment for anemia of chronic disease
treat underlying cause
| if its kidney give them erythropoietin
74
what is an X-linked recessive disorder primary affecting males mostly African Americans
G6PD
75
what is G6PD in the body for?
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
what is an episode of G6PD s/s ?
back or abd pain with anemia
jaundice and splenomegaly
can lead to hemolytic crisis
rbc's will rupture under oxidative stress
77
what can cause oxidative stress?
```
Infection- DKA, pneumonia
Fava beans
Sulfa drugs- bactrum
antimalarials
meythlene blue
fluoroquinoloes
nitrofurantoin
asprin
dapsone
```
78
Treatment for G6PD
usually self limiting
avoid whatever caused it
iron supplements
blood transfusions if severe
79
What is an autosomal recessive genetic disorder of Hgb SS. Valine is subsituted for what?
Sickle cells
| Valine in place of Glutamic Acid on beta chain
80
what is one benefit of Sickle cell trait?
AA are resistant to plasmodium falciparum - malaria
81
what are some s/s of sickle cell disease
```
sickle under hypoxic conditions
cause micro thrombosis
hemolytic anemia
hematuria
painful occlusive crises
```
82
what is the most common presenting symptoms in infants with sickle cell
Dactylitis - swelling of fingers and toes
| Hgb SS replaces fetal hemoglobin
83
What are some infections seen with sickle cell
1. osteomylitis- salmonella
2. risk of encasulated organism like S. pneumonia, H.flu
3. Aplastic crisis with parvovirus B19
84
What are signs of microthrombosis or infractions with sickle cell disease
```
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
what are sickle cells crises caused by?
| what happens?
```
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
what will show with sickle cell hemoglobin eletrophoresis
```
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
Treatment for Sickle cell disease
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
Hydroxyurea is used to treat what?
sickle cell disease with painful crisis
| reduces the frequency of painful crisis
89
What is important to do in children with sickle cell ?
```
Immunizaitons !!! SHIN!
S. pneumonia
H. Influenza
Inlfuenzae B and N
menigococcus
any encapsulated organisms
```
90
Key terms for this diagnosis- autosomal dominant intrinsic hemolytic anemia causing a defect RBC membrane and increase cell fragility
Hereditary Spherocytosis
91
key terms for this diagnosis- + osmotic fragility test with negative coombs
Hereditary Spherocytosis
92
Treatment for Hereditary Spherocytosis
folate- helps stablize the membrane
| spelenectomy in severe disease
93
key terms for this diagnosis- Antibodies to own RBC's surface leading to destruction by macrophages and the spleen.
Autoimmune hemolytic anemia - AIHA
94
What other disorders are common with Autoimmune hemolytic anemia - AIHA
Systemic Lupus*
Rheumatoid arthritis
Chronic lymphocytic leukemia
95
key terms for this diangosis- IgM Antibodies, Direct coombs tests +, microspherocytes
Autoimmune hemolytic anemia - AIHA
96
Treatment for Autoimmune hemolytic anemia - AIHA
1. warm steroids
2. Splenectomy
3. avoid cold exposure
97
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
paroxysmal notctural hemoglobinuria
98
What type of anemia is paroxysmal notctural hemoglobinuria
Hemolytic anemia
the protein that is suppose to protect it from complement breakdown is missing
so it gets SLICED!
99
what is the best test for paroxysmal notctural hemoglobinuria
Flow Cytometry
will be osmotic fragility -
coombs -
100
Treatment for paroxysmal notctural hemoglobinuria
1. Eculizumab
| 2. steroids
