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60 Cards in this Set

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anemia
A reduction in oxygen carryng capacity resulting in decreased tissue oxygenation.
measured as a decrease in RBC, Hgb or Hct.
Symptoms of Anemia
A reduction in oxygen carryng capacity resulting in decreased tissue oxygenation
Clinical Manifestations of Mild Anemia
Skin Mucosa: Normal
Heart: No Change
CNS: No Change
Clinical Manifestations of Moderate Anemia
Skin Mucosa: Pallor
Heart: Tachycardia, Murmur
CNS: Headache
Clinical Manifestations of Severe Anemia
Skin Mucosa: Marked Pallor
Heart: Tachycardia, Murmur, Heart failure
CNS: Fainting, Syncope, seizures
Signs of hemolytic anemia or dyserythropoiesis
jaundice
splenomegaly
maxillary hyperplasia
frontal "bossing"
What are anemia clues for iron deficiency?
Excessive milk intake
Pagophagia
Pica
Koilonychia
What are anemia clues for hemolysis?
- neonatal jaundice
- splenectomy
- cholecystectomy
What are anemia clues for Vit. B12 deficiency?
- Abdominal surgery (ileal resection)
- Glossitis
- Decreased proprioception and vibratory perception
Pica
A perverted appetite for substances not fit as food or of no nutritional value;
Dirt, cloth, clay...
Koilonychia
spooning of nails...indicative of iron deficiency
Skin Mucosa:
Heart:
CNS:
- COMPLETE BLOOD COUNT (CBC)
- RETICULOCYTE COUNT
- PERIPHERAL BLOOD SMEAR
WHAT’S IN THE CBC ?
1. HGB
2. RBC
3. MCV
4. RDW
5. HCT
6. MCH
7. MCHC
Microcytosis
A reduction in the size of red cells (erythrocytes). Measured as a decrease in the mean cell volume (MCV) of red cells.
What causes macrocytes to be so large?
Defects in DNA synthesis
What causes microcytes to be so small?
Defects in Hgb synthesis
What do microcytes look like?
microcytic (low MCV)
hypochromic (low MCH)
What is the function of ferrochelatase?
to combine protoporphyrin IX with Fe2+ ...making heme
Causes of Microcytic Anemias
1. Iron deficiency anemia
2. Disorders of iron metabolism
3. Sideroblastic anemias
4. Thalassemias
5. Hemoglobinopathies
CBC in Iron Deficiency
LOW: RBC, Hb, Hct, MCV, MCH, MCHC
HIGH: RDW
Why is RDW high in iron deficiency?
becuase there is not enough iron for all the cells and some cells get more iron than others
Components of IRON PROFILE
1. Serum Iron
2. TIBC / Transferrin
3. % Iron Saturation of TIBC
4. Serum Ferritin
Lab Tests in Iron Deficiency
1. IRON PROFILE (standard)
2. Soluble Transferrin Receptor
3. Free Erythrocyte Protoporphyrin
TIBC
Total Iron Binding Capactiy
What must an iron profile show in order to be diagnostic of iron deficiency?
low serum iron with high transferrin
(b/c transferrin is increased when there is a demand for iron)
this dx is true except for pregnant individuals
What are the states from normal to iron deficient anemia?
Normal
Iron Depletion
Iron Deficient Erythropoiesis
Iron Deficiency Anemia
Which of these is altered in iron depletion: Plasma Ferritin, Transferrin Saturation, RBC Protoporphyrin, Hemoglobin?
plasma ferritin is low (<12)
Which of these is altered in iron deficient erythropoiesis: Plasma Ferritin, Transferrin Saturation, RBC Protoporphyrin, Hemoglobin?
plasma ferritin is low (<12)
transferrin saturation is low (<16)
RBC Protoporphyrin is high (>100)
Which of these is altered in iron deficient erythropoiesis: Plasma Ferritin, Transferrin Saturation, RBC Protoporphyrin, Hemoglobin?
Plasma Ferritin is low (<12)
Transferrin Saturation is low (<16)
RBC Protoporphyrin is high (>100)
Hemoglobin is low (<12)
What is the most common cause of iron deficiency in adults?
BLOOD LOSS
- tusually from large bowel especially males and post-menopausal females
What are causes of iron deficiency in adults?
Blood loss
Pregnancy and Lactation (increased requirements)
Decreased Absorption
- decreased gastric acidity
- small bowel disease
Excessiver menstrual blood loss
What percent of toddlers and adolescent girls have iron deficiency (with anemia)?
9% iron deficient
2% with anemia
How much of dietary iron is from supplemental feedings in breast fed infants from 6-34 months?
>95% is from supplements
How many people have nutritional iron deficiency worldwide?
2 billion
How much iron does breast milk have?
0.2-0.4mg/L
How much iron does cow milk have?
0.7mg/L
Why is breast milk the best iron source?
because breast milk is more readily absorbed that cow's milk
What makes an infant at Risk for Iron Deficiency?
1. Low birthweight/prematurity
2. Perinatal bleeding
3. Low hemoglobin at birth
4. High growth rate
5. Early cow’s milk and solid food intake
6. Frequent tea intake by mom (tea chelates iron)
What are diagnostic clues for infants with iron deficiency anemia?
-EXCESSIVE MILK INTAKE
-PICA
Pagophagia
Compulsive and repeated ingestion of ice
Where is Iron absorbed in GI tract?
proximal portion of jejunum
Hemoglobin E / Beta Thalassemia
(compound heterozygotes)
MODERATELY SEVERE ANEMIA
SPLENOMEGALY
GROWTH DELAY
TRANSFUSION REQUIREMENT
Hemoglobin E
1. B-CHAIN MUTATION (B26lys..glu)
2. PREVALENT IN S.E. ASIA
3. MICROCYTIC, HYPOCHROMIC
4. INTERACTS WITH B-THALASSEMIA
Beta Thalassemia Major
a.k.a. Cooley's anemia
- VERY LOW HEMOGLOBIN
- TRANSFUSION DEPENDENT
- AUTOSOMAL RECESSIVE
- BONE MARROW TRANSPLANT CAN BE CURATIVE
Beta Thalassemia Minor
a.k.a. B thalassemia trait
heterozygote
- MILD MICROCYTIC HYPOCHROMIC ANEMIA.
- ELEVATED Hb. A2 (basically diagnostic)
- AUTOSOMAL DOMINANT
- Low MCV
- Normal RDW
Beta Thalassemias are because of mutations or deletions?
MUTATIONS
Alpha Thalassemias are because of mutations or deletions?
DELETIONS
Hemoglobin H Disease
- 3 alpha genes deleted
>moderately sever hemolysis
>elevated reticulocytes
>red cell inclusions on supravital staining
Alpha Thalassemia Trait
Thalassemia Minor (2 alpha genes deleted)
> microcytic, hypochromic RBCs
>Target cells
>Decreased MCV and MCH
>Normal RDW
>Mild anemia
What is your classification is you have one alpha hemoglobin gene deletion?
Silent Carrier
What is your classification is you have four alpha hemoglobin gene deletions?
Hydrops fetalis
>fatal in utero because of heart failure
alpha 0 trait
when one chromosome has two alpha deletions, but the other has two alpha genes
(thalassemia minor)
alpha + trait
when there is one alpha gene deletion on a chromosome
homozygous alpha + trait
when both chromosomes have one alpha gene deletion
Hemoglobin Barts
gamma chain tetramer
Hemoglobin H
beta chain tetramer
thalassemias are the result of globin chain synthesis _____
imbalance
quantitative disease
Sideroblastic anemia
- microcytic, hypochromic anemia
- iron accumulated in mitochondria
Congenital causes of Sideroblastic anemia
X-linked
Autosonal Recessive
Mitochondrial Disease (mutation/deletion in mitochondrial DNA, defect in enzyme fxn)
Acquired causes of sideroblastic anemia
1. Drugs (isoniazid)
2. Toxins (lead...interferes with protoporphyrin synthesis)
3. Neoplastic (myelodysplastic syndrome)