Anemia Flashcards
(115 cards)
1
Q
Hb < for anemia in males
Hb
A
Hb<13.5 males
Hb<12.5 in females
2
Q
MCV for Microcytic, normocytic, macrocytic
A
MCV <80 microcytic
MCV=80-100 normocytic
MCV>100 macrocytic
3
Q
Microcytosis seen in microcytic anemia is caused by a decrease in
A
Hemoglobin
4
Q
Components of Hemoglobin?
A
Heme (Iron+protoporphyrin ring) and Globin chains
5
Q
Anemia of chronic disease is caused when
A
iron is locked away in macrophages
6
Q
Sideroblastic Anemia results in a deficient?
A
Protoporphyrin rin
7
Q
Thalassemia is a deficiency or defect in the
A
globin component of Hgb
8
Q
Most common nutritional deficiency in the world?
A
Iron deficiency anemia
9
Q
Iron is absorbed in the
A
duodenum by the enterocyte
10
Q
What cell in the duodenum regulates iron absorption?
A
Enterocyte in the duodenum
11
Q
What transporter is used to move iron from the enterocyte to the blood?
A
Feroportin
12
Q
How does the body regulate whether or not to allow iron into the blood?
A
Feroportin transporter in the enterocyte
13
Q
In the blood, iron is transported by _______ to the liver and bone marrow macrophages
A
Transferritin
14
Q
Once the iron is transported by transferritin, is it stored intracellularly bound to
A
Ferritin, this binding prevents iron from creating free radicals via the Fenton Reaction
15
Q
What is the fenton reaction?
A
H202 + Fe2+ –> OH- + OH+ + Fe
16
Q
Iron in the blood is measured via
A
iron in the blood
17
Q
meausring the amount of trasnferrin molecules in the blood lab value?
A
TIBC
18
Q
Iron deficiency in infants could be caused by
A
Breast feeding
19
Q
Serum Ferritin measures
A
iron in the bone marrow and liver
20
Q
Children w/ iron deficiency anemia
A
poor diet
21
Q
Adult male w/ iron deficiency anemia?
A
Peptic Ulcer disease
22
Q
Elderly w/ iron deficient anemia?
A
Colon polyps/carcinoma in the western world
Hookworm in the developing world
23
Q
Necator and Ancylostoma both cause iron deficiency anemia because of
A
blood loss
24
Q
Disease seen w/ HLA-DQ2, Dermatitis Herpetiformis on shoulder/elbows/knees, iron deficiency anemia from involvement of duodenum (villous blunting, patchy)
A
Celiacs
25
Fe2+ is more readily absorbed than Fe3+, and iron maintains Fe2+ state when in acidic or basic environment?
Acidic
26
Gastrectomy creates an environment that is less acidic which can lead to iron deficient anemia because the iron is mainly in Fe2+ or Fe3+ state?
Fe3+
27
what happens to TIBC when tranferrin goes down
it goes up
28
the very early stages of iron deficiency anemia because the bone marrow simply makes less red blood cells without adapting/compensating for less heme
Normocytic anemia
29
why would iron deficiency anemia ultimately lead to hypochromasia?
Less heme, so the central ring of pallor grows and also microcytic
30
Koilonychia is
spoon shaped nails
31
Red blood cell distribution width describes?
range of RBC size.
32
Why would the red blood cell distribution (RDW) increase in iron deficiency anemia?
At first the anemia is normocytic and then microcytic so wide range of sizes
33
Free erythrocyte protoporphyrin present with iron deficient anemia because?
there is no iron to make the ring and bind to form heme
34
Treatment of iron deficient anemia?
Ferrous Sulfate
35
Esophageal web (mucosal protrusion) and atrophic glossitis associated with
Plummer-Vinson syndrome
anemia, dysphagia, beefy-red tongue
36
Beefy-red tongue could be seen with esophageal web in
Plummer Vinson syndrome, seen with iron deficient anemia
37
Anemia of chronic disease is the most common anemia in
hospitalized patients, seen in cancer patients and chronic inflammation
38
Hepcidin is an acute phase reactant, its function?
sequesters iron in storage sites to keep iron from being transferred (prevents iron from macrophages to erythroid precursors)
39
Hepcidin is an acute phase reactant that prevents iron transfer from ______ to ______ ______ and it suppresses EPO production
macrophages to erythroid precursors
40
Giving exogenous EPO to patients w/ Anemia of chronic disease because hepcidin
hepcidin decreases EPO production
41
where does protoporphyrin synthesis occur?
mitochondria
42
**HY**Rate limiting steps of Protoporphyrin synthesis?
Amino Lavulinic Acid Synthase (+B6) and ALAD
43
ALA ---> porphobilinogen
ALAD
44
**HY**Where does iron remain if protoporphyrin is deficient?
in the mitochondria, it will pile up and create a ring around the nucleus
45
When iron builds up in the mitochondria, what do we see histologically?
Ringed Sideroblasts seen with prussian blue stain
46
**HY**Sideroblastic Anemia congenital defect associated with what rate limiting enzyme?
ALAS (Succinyl CoA--->Amino Lavalunic Acid)
47
Acquired Sideroblastic aneminina can be a result of
```
Chronic alcoholism
Lead poisoning (ALAD and Ferrokolotase)
Vitamin B6 deficiency (cofactor for ALAS)
```
48
Isoniazid therapy for TB can cause what sort of deficiency leading to Sideroblastic anemia?
Vitamin B6
49
Iron overloaded state lab findings?
Ferritin and TIBC
Serum Iron and %Saturation
Ferritin increased TIBC decreased
| Serum Iron increased % Saturation increased
50
Iron overloaded lab findings in Sideroblastic anemia also look like
Hemochromatosis
51
thalessemia is not a defective globin chain, rather
decreased synthesis of globin chains
52
Thalessemia is protective against with species of malaria?
Plasmodium Falciparum
53
3 normal types of hemoglobin:
HbF (alpha2gamma2)
HbA (alpha2beta2)
HbA2 (alpha2gamma2)
54
alpha chain is incredibly important and usually has ____ alleles and are present on chromosome ____
alpha chain: 4 alleles, on chromosome 16
55
alpha thalessemia is usually due to
gene deletion
56
1 alpha gene deletion of the globin chain is asymptomatic, 2 alpha genes deleted can be cis (on the same chromosome) or trans, which is worse and associated with increased risk of what in offspring?
Cis alpha deletion associated with increased risk of severe thalessemia in offspring and associated with spontaneous abortion in asians.
Trans deletion not as severe and seen in Africa
57
3 alpha globin chain deletions seen in alpha thalassemia will result in damage to the RBCs because the beta chains form _____
tetramers known as HbH
58
Tetramers of beta globin chains are known as HbH which can be seen on
electrophoresis
59
4 alpha genes deleted in alpha thalassemia would lead to a gamma chain tetramer formation known as Hb Barts which will do what to a baby in utero?
hydrops fetalis, this is a lethal gene deletion
60
What's a significant difference between the genetic nature of alpha thalassemias versus beta thalassemias?
alpha thalassemias due to gene deletions
beta thalassemia due to gene mutations
61
B-thalassemia: 2 beta genes on which chromosome?
Chromosome 11
62
B-thalassemia: least severe form=
Normal Beta and B+
63
Target cells seen with
Beta thalassemia minor
64
Beta-thalassemia seen with
isolated increased HbA2 (5%)
65
B-thalassemia major: both beta chains knocked out doesn't affect the fetus because
fetus uses a gamma chain not a beta
66
Severe anemia in neonate a few months after birth could be
Beta-thalassemia because baby starts switching to AB hemoglobin chains from HbF
67
in B-thalassemia major alpha chains form tetramers which damages the RBCs while being made, but if the RBC is able to escape, what will happen?
the spleen will destroy the RBC
68
The severe anemia seen in B-thalassemia will cause
massive erythroid hyperplasia
69
Expansion of hematopoesis into the marrow of the skull and facial bones seen in B-thalassemia will present with what radiological findings? and what will happen to the face?
Crewcut appearance of the skull (massive expansion of hematopoeisis) and chipmunk face
70
chronic transfusions seen in patients with b-thalassemia major can lead to
hemochromatosis
71
no HbA seen in
b-thalassemia major
72
2 most common cause of megaloblastic anemia (macrocytic anemia)
Folate or Vitamin B12 deficiency
73
folate enters the body as methylated-THF, to enter the body it THF needs to be demethylated which Vit B12 takes off the THF. methylated Vitamin B12 passes off the methyl group to _______ which turns into _____
Homocysteine turns into methionine (these are needed for DNA synthesis)
74
there is a different between macrocytic and megaloblastic anemias
Macrocytic due to another cause that isn't vitamin deficiency then that is simply called macrocytic anemia and not megaloblastic.
Causes of macrocytic anemias:
Alcoholism
Liver Disease
Drugs (5-FU)
75
Findings in neutrophils w/ megaloblastic anemia due to folate or vit b12 deficiency
hypersegmented neuts (also will show really large RBCs)
76
Macrocytic Anemia won't present with what findings as compared to Megaloblastic anemias?
Macrocytic won't present w/ hypersegmented neutrophils or megaloblastic cells in rapidly dividing cells. RBCs will still have MCV>100 though
77
Where is Folate absorbed?
Jejunum
78
Causes of Folate Deficiency?
```
Poor diet (EtOH, elderly)
Increased demand (pregnant, cancer, hemolytic anemia)
Folate Antagonists (methotrexate)
```
79
Methotrexate can lead to folate deficiency which can lead to Megaloblastic Anemia because it is inhibits ________
Dihydrofolate reductase
80
**HY** Why is there an increase in serum homocysteine with folate deficiency?
there isn't enough THF for Vit b12 to demethylate and then pass on to Homocysteine so homocysteine piles up
81
Methylmalonic acid gets converted to Succinyl CoA by Vitamin B12 and is normal in ______ deficiency which helps distinguish from a similar clinical presentation of Vitamin B12 deficiency.
Folate deficiency
82
Salivary gland produces what binding protein that attaches to Vitamin B12
Haptocorrin (R-Factor) which is cleaved and in the intestine it is bound to intrinsic factor that is produced in the body of the stomach by parietal cells
83
Parietal cells of the stomach are important because they produce ______ which bind to Vitamin B12 after Vitamin B12 has been cleaved from Haptocorrin
Intrinsic factor.
84
The Intrinsic Factor + Vit B12 complex is absorbed in the
Ileum
85
Most common cause of Vitamin B12 deficiency?
Pernicious Anemia
86
Pernicious Anemia is the autoimmune destruction of the parietal cells in the stomach which is an issue for Vitamin B12 because
Vitamin B12 needs to bind to IF before being absorbed in the ileum
87
The P's of Parietal Cells
Pernicious Anemia
Proton Pumps: make acid
Pink
88
Other than Pernicious anemia, other causes of Vitamin B12 deficiency?
Pancreatic Insufficiency
Damage to terminal ileum from Crohn disease or Diphyllobothrium latum
Dietary deficiency is rare except in vegans
89
Iron deficiency seen in _________ disease whereas Crohn's disease presents w/ _______ deficiency
Iron deficiency seen in Celiacs
Vitamin B12 deficiency seen in Crohn's (because of terminal ileum involvement)
90
Why could Pancreatic insufficiency cause a vitamin B12 deficiency?
B12 from diet + Haptocorrin from saliva---Proteases from pancreas-->cleaved free B12 in small bowel + Intrinsic factor from parietal cells----> terminal ileum-->absorbed
So the R-binder and Vitamin B12 complex aren't cleaved
91
Why could Pancreatic insufficiency cause a vitamin B12 deficiency?
B12 from diet + Haptocorrin from saliva---Proteases from pancreas-->cleaved free B12 in small bowel + Intrinsic factor from parietal cells----> terminal ileum-->absorbed
So the R-binder and Vitamin B12 complex aren't cleaved
92
Inherited hereditary spherocytosis is autosomal
dominant
93
Hereditary spherocytosis is a defect causing blebs of the membrane jutting out because of a defect in what protein?
tethering proteins commonly spectrin, ankyrin, band 3.1.
94
Hereditary Spherocytosis will present with what on peripheral smear?
loss of central pallor, round, wide range of sizes
95
An increase in MCHC is seen in along w/jaundice with unconjugated bilirubin and and increased risk for bilirubin gallstones
Hereditary Spherocytosis (also in autoimmune anemia you see the increased MCHC)
96
What virus affects the erythroid precursors and those w hereditary spherocytosis are particularly susceptible to infection
Parvovirus B19
97
Diagnosis of Hereditary spherocytosis
osmotic fragility test (increased fragility in hypotonic solution)
98
Splenectomy is recommended to alleviate associated anemia in the treatment of
Hereditary Spherocytosis. Will still present w/ spherocytes and will present w/ Howell-Jolly bodies because of splenectomy.
99
Autosomal recessive mutation in the Beta chain of the Hgb in which the normal ________ acid which is hydophilic is replaced w/ _____ which is hydrophobic
Normal hydrophilic glutamic acid replaced w/ valine which is hydrophobic
100
what do HbS chains form?
polymers causing sickling of the RBCs, they sickle when oxygen is removed and desickle while passing through circulation leading to membrane damage of the RBC
101
Increased risk of polymerization of HbS includes
Hypoxemia
Dehydration
Acidosis
102
Protective against sickling?
HbF. which is why people with sickle cell disease won't present until 6months in neonate.
103
Why would treatment w/ hydroxyurea prevent sickling?
Hydroxyurea increases the levels of HbF (for unknown reasons)
104
the membrane damage that occurs via the sickling and desickling of HbS leads to extravascular hemolysis as the spleen removes these damaged cells from the circulation. this presents as
Anemia
Jaundice w/ unconjugated hyperbilirubinemia
Increase risk for bilirubin gallstones
Target cells in blood smear
105
There is some intravascular hemolysis w/ HbS. This presents with a decrease in the scavenger molecule that tries to save the hemoglobin?
Haptoglobin
106
Extramedullary hematopoeisis can lead to what on xray and what facial appearance?
Crew cut on xray
| Chipmunk facies
107
Irreversible sickling can lead to vasoocclusion which will present as swollen hand and feet due to vaso-occlusive infarcts of bones. A 6 months old black baby's hands shown that are swollen?
Dactylitis from irreversible sickling leading to vaso-occlusion
108
Why would a kid w/ sickle cell disease be at an increased risk of encapsulated organisms?
Because of autosplenectomy as complication, so you'd see howell-jolly bodies on blood smear
109
Osteomyelitis is most commonly caused by Staph Aureus, however in those with sickle cell anemia, what gram-negative motile rod is more commonly implicated in osteomyelitis in those w/ SCA
Salmonella paratyphi
110
Most common cause of death in those w/ sickle cell anemia for
kids
adults
Kids: infection by encapsulated organisms
Adults: Acute Chest syndrome (vaso-occlusion in pulmonary microcirculation often precipitation by pneumonia)
111
Vaso-occlusive crisis complications in those with sickle cell anemia
Acute Chest Syndrome: occlusion in pulmonary microcirculation usually after pneumonia, most common cause of death in adults w/ SCA (presenting w/ dactylitis)
Pain crisis
Renal papillary necrosis: gross hematuria and prorteinuria
112
how much HbS do you need to actually sickle and get the symptoms?
45%
113
if you have less than 45% HbS, you will asymptomatic but because of the hypoxia and hypertonicity of the renal medulla, the low amount of HbS will still cause sickling. What is the classic findings from microinfarctions in the renal medulla in someone w/ sickle cell trait
Microscopic Hematuria and a decreased ability to concentrate urine
114
What screen identifies HbS in any cell (so sickle cell trait and disease)
Metabisulfite screen
| Can also do Hb Electrophoresis to confirm presence and amount of HbS
115
In Sickle Cell disease, Glutamic acid is replaced by valine. In hemoglobin C, what is glutamic acid replaced w/?
Lysine
| You'll see Hgb C on PBS
