Microcytic Anemias Flashcards
What is heme composed of?
4 porphyrin rings
4 iron molecules
What is Globin composed of?
2 alpha chains, 2 beta chains
Where is iron absorbed?
Intestines
What is job of Transferrin?
Transports iron
What is Ferritin?
Simple storage form of iron
What and where is Hemosiderin?
Complex iron stored in macrophages
What is most common type of stored iron?
Ferritin
Daily Iron requirements for men and non-mensturating women?
1mg/day
Daily Iron requirements for mensturating women?
3-4mg/day
Daily Iron requirements for pregger women?
2-5mg/day
How often to screen men and non-mensturating women for anemia?
5-10 years
How often to screen mensturating women for anemia?
Annually
Cytic of Iron Deficiency, Thalassemias, Lead Poisoning, Sideroblastic anemia, and Anemia of Chronic Dz? (micro, normo, macro)
All microcytic. MCV <80
Cytic of B12 deficiency, Folate deficiency, Liver dz, and Thyroid dysfunction?
Macrocytic
Etiology of Iron Deficiency Anemia?
1 blood loss
Decreased iron causes what to RBC production?
Decreased RBC production
Define Pica? Which anemia?
Eating non-food things. Iron Deficiency anemia.
Define Pagophagia. Which anemia?
Eating ice. Iron Deficiency anemia.
Eye and palm color in Iron Deficiency Anemia?
Conjunctival pallor, palm pallor
Cell size and color in Iron Deficiency Anemia?
Microcytic Hypochromic. MCV <80, MCHC <33.
TIBC increased or decreased in Iron Deficiency Anemia?
Increased. All others decreased (SE, SI, % Sat).
Tx for Iron Deficiency Anemia?
Ferrous Sulfate 325mg BID PO x1-2mo, then qd x3-6 mo
What can decrease iron absorption?
Fiber, dairy, phospates, tea/tannins
When to refer iron deficiency anemia?
H & H < 9 and 27%
Etiology of Alpha-Thalassemia?
Gene deletion
Who gets Alpha-Thalassemia most often?
SE Asian and Chinese
Silent Alpha-Thalassemia genotype?
__ alpha/alpha alpha
Alpha-Thalassemia Trait 1/Minor genotype?
___alpha/___alpha
Who gets Alpha-Thalassemia Trait 1/Minor?
Asian population
Alpha-Thalassemia Trait 2/Minor genotype?
alpha alpha / ____ ____
Who gets Alpha-Thalassemia Trait 2/Minor?
Black population
Alpha-Thalassemia Intermedia/Hgb H Disease genotype?
___ ___ / ___ alpha
Alpha-Thalassemia results in what?
Decreased alpha globin chain synthesis
Hydrops Fetalis genotype?
___ ___ / ___ ___
Alpha-Thalassemia Trait/Minor RBC and MCV?
Elevated RBC, decreased MCV
Heinz Bodies in which anemia?
Alpha Thalassemia intermedia
PE in Alpha Thalassemia Intermedia?
Pallow, hepatosplnemomegaly, skull and maxilla deformed
Skull deformations in which anemia?
Alpha Thalassemia Intermedia
Cytic in Alpha Thalassemia Intermedia?
Microcytic 60-70
Tx for Silent and Trait/Minor Thalassemia?
None needed
Tx Alpha Thalassemia Intermedia?
Folic Acid supp 1mg PO/da. Avoid oxidative medications (Sulfa drugs)
What drugs should Alpha Thalassemia Intermedia avoid?
Oxidative drugs (Sulfa)
Who gets Beta-Thalassemia?
Mediterranian
What type of gene defect in Beta-Thalassemia?
Gene point mutations (NOT deletions)
Beta-Thalassemia results in decreased production of what?
Decreased production of Beta globin chains
Most common type of Beta-Thalassemia?
Beta-Thalassemia Minor
Beta-Thalassemia Minor genotype?
B/B+ or B/Bº
Genotype of Beta-Thalassemia Intermedia?
B+/B+
Beta-Thalassemia Intermedia and Hgb B synthesis rate?
Homozygous but increased rate of Hgb B synthesis. Mild form.
When does anemia appear in Beta-Thalassemia Intermedia?
Normal at birth, anemia at 6 mo
Percent Hgb A and A2 in Beta-Thalassemia Intermedia?
HgbA=30%
HgbA2=10%
What deformed and what enlarged in Beta-Thalassemia Intermedia?
Bone deformities. Hepatosplenomegaly.
Beta-Thalassemia Major/Cooley Anemia genotypes? (Hint: 2)
Bº/Bº or B+/B+
Why Beta-Thalassemia PT normal until 6 months?
Due to presence of HgbF
Beta-Thalassemia Major/Cooley Anemia PE?
Extramedullary erythropoiesis (Frontal bossing).
How severe hemolytic anemia in Beta-Thalassemia Major/Cooley?
Very severe hemolytic anemia
What type of cell will Peripheral Smear show in Beta-Thalassemia?
Target cell (also in alpha thalassemia)
If microcytic but normal serum Fe what to consider?
Thalassemia
Dx for Beta-Thalassemia?
Electrophoresis
What will Electrophoresis show for Beta-Thalassemia Major/Cooley?
HgbF (90%), HgbA2, little to no HgbA
What will Electrophoresis show for Beta-Thalassemia Trait/Minor?
Decreased HgbA, increased HgbA2
Tx of Beta-Thalassemia Major/Cooley?
Transfusions if Hgb 9-10, Folic Acid, iron chelation to prevent iron overload. Allogenic stem cell bone marrow transplant.
TOC of Beta-Thalassemia Major/Cooley?
Allogenic stem cell t/p
What is “Vampire Disease”?
Porphyria
Etiology of Porphyria
Porphyrin ring not formed due to altered enzyme activities in heme biosynthetic pathway
Skin in Porphyria?
Very pale, blisters in sun
Etiology of Sideroblastic Anemia?
Defect in heme synthesis causing ringed sideroblast.
Where does iron ring form in Sideroblastic Anemia?
Forms in bone marrow
Neutropenia in Sideroblastic Anemia due to what?
Copper deficiency
Which iron study low in Sideroblastic Anemia?
TIBC. Rest high.
Which dz are Pappenheimer Bodies found in?
Sideroblastic Anemia
Sideroblastic Anemia can be induced by what? Treatment?
INH. Vit B6.
Splenectomy in Sideroblastic Anemia?
NO!
Sideroblastic Anemia tx?
Transfuse and chelate as needed
Basophillic Stipling found in what dz?
Lead Toxicity
Lead Toxicity does what to heme synthesis?
Decreases heme synthesis
Test for Lead Toxicity?
Peripheral smear. Microcytic hypochromic anemia w/basophillic stippling.
Tx for Lead Toxicity?
Remove lead. Chelation if severe.
What is Hepicidin and which dz?
Inflammatory marker. Anemia of Chronic Dz-Inflammation
Resistance to Erythropoietin in which Anemia of Chronic Dz?
Elderly
Reduced Erythropoietin in which Anemia of Chronic Dz?
Organ Failure
Anemia of Chronic Dz is what type of dx?
Dx of exclusion
SI in Inflammatory and Organ Failure/Eldery Anemia of Chronic Dz?
Inflammatory=low
Org fail/elderly=normal
ESR and CRP are what type of markers?
Non-specific inflammatory markers
Tx for Anemia of Chronic Dz?
Tx underlying dz. EPO is Hgb <10. Folic Acid is concomitant anemia.