Hematology 1- Iron, Thalassemia, Chronic Dz, Sideroblastic, Lead 10/21/16 Flashcards
What is Ferritin?
Storage form of iron. Easily accessible and small.
What is Transferrin?
Iron transport protein
What is TIBC? What does it indirectly measure?
Total iron binding capacity. Measure of iron binding capacity (and indirect assessment of transferrin)
What is SI (Serum Iron)?
Amount of transferrin saturated of Iron Fe2+
What is % saturation and formula formula?
transferrin saturation SI/TIBC x 100
What is Reticulocyte Index? (RI)
Direct measure of bone marrow function. If keeping up with RBC need.
What is Hemosiderin? Can the body use it easily?
Complex iron stores in macrophages. Difficult for the body to use.
HCT normal range?
36-45
Hgb normal range?
12-15
WBC normal range?
5000-10,000
Platelet normal range?
150,000-400,000
Normal body iron range?
3-4g
Define Anisocytosis. Which lab value?
Different size RBCs. Low MCV.
Define Poiklocytosis
Different shaped cells
MCV normal range?
80-100
MCHC normal range?
33-35
Hypochromic from which value?
MCHC lower than 33.
Average American iron intake?
10-15mg/day
Percent of dietary iron absorbed?
10%
Iron requirement for male and non-mensturating females?
1mg/day
Iron requirement for mensturating females range?
3-4mg/day
Iron intake for pregger female range?
2-5mg/day
Normal body iron content range?
3-4g
How much iron lost through daily exfoliation in gut?
1mg/day
How much iron loss due to menses? Menses length range?
1mg/day, x4-7 days
Entire iron loss from preggers, delivery, and nursing?
About 1000mg
How much Fe lost in bleeding per 100mL of whole blood? (Trauma, acute bleed, loss d/t surgery, etc)
50mg
How much Fe in 100mL of whole blood?
50mg
Who gets Iron Deficiency Anemia most often?
Women more than men. D/t mensturation and preggers.
What is #1 cause of Iron Deficiency Anemia until proven otherwise?
BLEEDING!
Iron Deficiency Anemia causes NIMBLE stands for?
N=Need I=Intake M=Malabsorption B=Blood L=Loss E=Excessive donation
Iron Deficiency Anemia causes other than bleeding?
- Deficient diet
- Decreased absorption (Celiac dz, Zn deficiency)
- Increased requirements (preggers, lactation)
- Hemoglobinuria
- Iron sequestration
3 stages of Iron Deficiency Anemia?
- Depletion of iron stores without anemia
- Anemia with normal RBC size (normal MCV)
- Anemia with reduced RBC size (low MCV)
Signs and symptoms of Iron Deficiency Anemia?
Fatigue, dizzy/weak, HA, tachycardia, dyspnea on exertion, palpitations
Beeturia, pica (eating weird things), pagophagia (craving for ice), dysphagia d/t esophageal web), pallor, conjunctival and palm pallor, smooth tongue, brittle nails, kolionychia (nails spooning), cheiolosis (breakdown of skin in corner of mouth when severe)
Three signs/symptoms of severe anemia? Why?
Tachycardia, dyspnea on exertion, palpitations. Due to decreased oxygen carrying capacity.
In Iron Deficiency labs (Serum Ferritin, Serum Iron, TIBC, and % Sat) which are low and increased?
Serum Ferritin=low
Serum Iron=low
% Sat=low
TIBC=increased
MCV low in anemia w/reduced RBC size.
MCV and MCHC in initial Iron Deficiency Anemia?
Low MCV, low MCHC. Microcytic, hypochromic
MCV and MCHC in severe Iron Deficiency Anemia? Aniso or Poik?
Low MCV, low MCHC.
Progression to anisocytosis and poikilocytosis.
Aniso=target cells
Poik=pencil and cigar shapes cells
Essentials of Dx for Iron Deficiency Anemia
Iron deficiency present.
Serum Ferritin less than 12 in absence of scurvy
-OR-
Anemia and Serum Ferritin less than 30
How to prevent Iron Deficiency Anemia in pregnant women in deficient?
Low dose Iron 30mg/day PO if deficient
When to screen girls 12-18 and nonpreggers childbearing age women for Iron Deficiency Anemia?
Screen q5-10y, annual screening for women at risk
In Iron Deficiency Anemia a Serum Ferritin less than __ and in the absence of ______
Serum Ferritin less than 12 in absence of scurvy
In Iron Deficiency Anemia a PT with anemia and a Serum Ferritin less than __ has essential to dx?
Anemia and Serum Ferritin less than 30
What is Reticulocyte Index value in Iron Deficiency Anemia?
Normal at less than 2.5.
What is #1 think to do for treating Iron Deficiency Anemia?
Search for blood loss! Do stool guaiac or colonoscopy to check for occult blood.
What is the DOC for iron repletion in Iron Deficiency Anemia?
Ferrous Sulfate 325mg (65mg elemental iron) PO TID x1-2 months, then daily for 3-6 months
What will CBC be at 3 weeks, 2 months, and 6 months after starting tx for Iron Deficiency Anemia?
3 weeks=half way to normal
2 months=normal
6 months=normal (replace storage)
In Iron Deficiency Anemia if CBC is normal at 2 months why continue tx for 6 months?
To replete iron stores
What is #1 ADR or PO Iron?
Constipation
How often to check for Iron Deficiency Anemia after tx completed?
Once a year
What to avoid when taking PO Iron?
Avoid dairy, fiber, food, tea, tannins, phosphates, antacids. All bind iron and prevent absorption.
What helps PO Iron absorption?
Orange Juice! Drink OJ!
When to use IV iron for Iron Deficiency Anemia?
If they fail PO iron. Might have GI malabsorption from IBD or gastric bypass or might have continued blood loss that can’t be stopped (hemodialysis).
If PT on dialysis and not being transfused regularly and has Iron Deficiency Anemia what to do?
Supplement with IV Iron
If PT on dialysis and is being transfused regularly and has Iron Deficiency Anemia what to do?
Don’t supplement iron!
When and how to take PO Iron?
Empty stomach. Use straw to avoid teeth stain.
If PO Iron not tolerated BID or TID what to do?
Use low dose at 1-2x/day or slow absorption prep (SlowFe)
Most common ADRs of PO Iron?
Nausea, constipation, diarrhea, dark stool
If PT not responding to PO Iron for Iron Deficiency Anemia what to consider?
Incorrect dx, celiac dz, ongoing bleeding, non-compliance
When to refer Iron Deficiency Anemia to hematology
If H and H below 9 and below 27%
What are signs of Zinc Deficiency on hands and buttocks?
Rashes.
Buttocks=dry, scaly, eczematous skin. Can become infected with Candida albicans.
Hands=enlarged fingers, paronychia, bright erythema on terminal phalanges
What can Zinc Deficiency cause malabsorption of?
Iron
Thalassemia is a reduction of what? What does it do to hemoglobin synthesis?
Reduction in synthesis of alpha or beta globin chains. Causes reduced hemoglobin synthesis.
Is Thalassemia hereditary?
YES!
What happens to MCV and MCH in Thalassemia?
Low MCV, low MCH. Hypochromic and microcytic anemia.
Heme is made up of how many porphyrin and rings and iron molecules?
Heme=4 porphyrin rings, 4 iron molecules
What is normal adult Globin made up of?
HgA. 2 alpha and 2 betas chains.
Alpha globin on which chromosome, how many sites, and how many pairs? Total from parents?
Chromosome 16, 4 sites, 2 from each parent. 4 total.
Beta globin on which chromosome and how many pairs? Total from parents?
Chromosome 11, 2 sites, 1 from each parent.
Beta globin chains can be mimicked by what?
Delta chains or Gamma chains.
Can Alpha globin chains be mimicked?
No.
Hgb A normally made up of which globin chains? How many? What percent of normal adult circulation
2 alpha, 2 beta.
98% of normal adult circulation.
Fetal hemoglobin is called what, made up of which globin chains, and does what to oxygen? Wears off when?
Hgb F. 2 alpha, 2 gamma.
Very high affinity to oxygen, steals oxygen from mother. Wears off around 6 months.
Hgb H is made up of which and how many globin chains?
4 Beta chains
3 types of Thalassemia and transfusions?
- Trait= lab findings, no clinical impact
- Intermedia= moderate clinical impact, occasional transfusion
- Major= Life threatening, transfusion dependent
Thalassemia iron study lab trends (SF, SI, TIBC, % Sat)?
SF=norm or inc
SI=norm or inc
% Sat=norm or ince
TIBC=NORMAL
Thalassemia RBC, MCV, MCH, Reticulocyte Count?
RBC=norm or elevated
MCV=LOW (lower than iron deficinecy)
MCH=low
Reticulocyte Count=elevated, above 2.5
What are Heinz Bodies (found in Thalassemia)?
Denatured hemoglobin found inside RBCs
Are Nucleated RBCs (NRBCs) found with Thalassemia?
Yup, you bechya.
Thalassemia essentials of Dx
- Microcytosis disproportionate to degree of anemia
- fam history
- Norm or elevated RBC count
- Abnormal RBC morphology with microcytes, hypochromia, acanthocytes, and target cells
What are acanthocytes?
Spiked/spurred RBCs
Which 2 hemoglobins elevated in Beta-Thalassemia?
- Hgb A2
2. Hgb F
Alpha-Thalassemia epi?
Southeast Asia and China
Genetic eti of Alpha-Thalassemia?
Gene deletions from Chromosome 16.
Each Alpha-Thalassemia gene deletion causes reduced alpha-globin chain synthesis by how much?
1/4
If missing two Alpha-Thalassemia sites on Chromosome 16 how much alpha-globin reduced by?
50%
Can alpha chains be mimicked or replaced by other chains?
No! Beta chains can, but alpha aren’t beta chains.
What is the genotype of normal and silent carrier Alpha-Thalassemia?
Normal= aa/aa
Silent carrier= aa/a-
What are the MCV and hematocrit or normal and silent carrier Alpha-Thalassemia?
Normal MCV, normal crit
What are genotypes for Alpha-Thalassemia Minor?
aa/– or a-/a-
What are MCV and hematocrit for Alpha-Thalassemia Minor
MCV=60-75
Crit=28-40%