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43 Cards in this Set

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AP II HEMATOPOIETIC DISORDERS
AP II HEMATOPOIETIC DISORDERS
Erythropoiesis
Erythron: rbc production
Erythropoietin (EPO)
Produced and released by kidney.

Functions in the presence of iron.
What is the stimulus for EPO?
O2
Hemoglobin has what binding capacity for O2?
1.34mL O2/gram of Hb
Structure of hemoglobin?
Globin portion consists of 4 chains.

Each chain has a heme group.

Each heme group has 4 pyrroles with ferrous Fe(+2)

Each heme can bind 1 molecule of O2, therefore, each Hb can bind 4 molecules of O2.
O2 Carrying Capacity (CaO2) =
Hbx1.34xSaO2 + PaO2(0.003mL O2/dL)

NOTE: unit of CaO2 is ml/dl
Acidosis
Inc 2,3 DPG
Hyperthermia
Pregnancy
All shift curve to the right
Anemia
Reduction in number of RBCs

Losses of 10-15% of total blood lead to signs of vascular instability.

30-40%--> shock
How does the body compensate for anemia?
1. inc CO and RR
2. inc 2,3 DPG
3. pull fluid from interstitium to expand plasma vol
4. redistribution of blood to vital organs.
Main cause of anemia?
Iron deficiency
At what level does serum iron fall to in order to incur impairment of Hb synthesis?
15-20%
Causes of iron deficiency
1. when there is more demand for iron.
2. inc iron loss
3. dec iron intake
Sickle cell dz
1. hemoglobinopathy resulting from abnormality of structure.
Defect in the globin fraction in HbA.
2. autosomal recessive disorder.
3. shape change on deoxygenation.
4. signs and symptoms result from vascular occlusion.
Shape of rbcs is altered by?
Deoxygenation
Aplastic
Means defective in development
Mutation of what causes sickle cell dz?
In beta chain gene.

Leads to decreased solubility, deformity of RBC, and hemolysis.
What are the initiation conditions of sickle cell dz?
1. hypoxia
2. hypothermia
3. dehydration
4. acidosis
Manifestations of sickle cell disease?
1. hemolytic anemia
2. reticulocytosis/granulocytosis
3. painful crises
4. microinfarction (due to occlusion)
Tx for sickle cell disease?
1. fluids, O2, folic acid supplementation, and narcotic analgesic.
2. blood transfusion
What are the examples of dyshemoglobinemias?
1. methemoglobinemia
2. sulfhemoglobinemia
3. carboxyhemoglobinemia
Methemoglobinemia
Ferric form (+3) causing it not being able to bind O2.

Cyanosis
MetHb causes which direction of shifting on the oxy-hb curve?
Left
What is the treatment for MetHb?
Methylene blue 1mg/kg every 60 mins.
Causes of SulfHb?
1. sulfur atom gets incorporated into porphyrin ring.
2. drug induced
3. cyanosis
SulfHb shifts which direction on the oxy-hb curve?
Right
Tx for SulfHb?
NONE

Eventually destroyed
CarboxyHb
Occurs when CO binds to Hb molecule.

Pulse Ox gives false high reading

NOTE: 200X greater binding than O2
Tx for CarboxyHb
1. supplemental O2
2. Hyperbaric chamber
Polycythemia and its types
Vol exceeds normal
Primary
Secondary
Relative polycythemia
What is primary polycythemia?
Myeloproliferative disorder
Secondary polycythemia
Hemoconcentration

Renal dysfunction
Relative polycythemia
Smoker's polycythemia
Neutrophils
Increase in presence of infection.
Lymphoma
Malignancy of lymphatic system
Thrombocytopenia
Low platelet count
Von Willebrand Disease (vWD)
MOST COMMON hereditary coagulation abnormality.
Tx of vWD
DDAVP (desmopressin)

Prognosis: excellent
Thrombocytopenia can be caused from?
Liver dz
Aspirin
Inhibits platelet aggregation and secretion.
Heparin
Antibody mediated
Penicillin
Interfere with platelet membrane and membrane receptors
Dextran/Hetastarches
Interferes with platelet surface membrane.