Hematology/anemia Pharmacology

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Hemophilia

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X linked, mom to son

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Hemophilia A

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VIII (most common)

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Hemophilia B

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Christmas Disease IX

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Intrinsic factors and Extrinisic factors combine..

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at Factor X --> prothrombin --> thrombin --> fibrinogen --> fibrin --> creats mesh that platelet adhere to

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Hemophilia inhibits:

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VIII and IX in intrinsic pathway (without theses never get to facto X) --> replace factors

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Adverese effects of giving factors

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- mild allergy (hives) --> antihistamine
- Anaphylaxis--> wheezing, tightness, SOB, facial swelling --> epinephrine subu

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Desmopressin

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- give if acutely hemorrhagin
- ADH
- Will stopp bleeding in mild Hem A
- CAN'T BE USED TO TREAT HEM B

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Antibody formation against factor (tx)

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- happens 9-12 months after tx
- more common in severe disease
- work with immunologist

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Heb B (IX) tx

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no risk for HIV or Hep (Hem A does), on demand and prophylactic therapy

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Leukopoietic growth factors names

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---ilagrastim

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Leukopoiteic growth factors use

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(don't give sooner than 24 hrs after tx and continue for 2 weeks)
- increase neutrophils during chemo
- bone marrow transplant (high dose)
- to tx chronic neutropenia
- costly

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Adverse effects of leukopoiteic growth factors

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- bone pain
- leukocytosis
- increased uric acid
- increased alk phosph and LFTs
- splenomegally ( where cells are captured)

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Monitoring for Epoetin alfa

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- BP
- hgb (baseline weekly until 10-12 gm/dl reached (over this causes cardio effects)
- Fe level
- CBC
- BUN/Creatinine
- Uric acid
- Potassium
- Phosphorus

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Darbepoetin (long acting alfa)

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can shorten survival in some cancer pts

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Epoetin Alfa names

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Epogen/Procrit

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Epoetin Alfa action and use

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- erythropoeisis stimulating agent
- CRF (maintains RBC count)
- chemo/radiation (USE ONLY IN NONMYELOID MALIGNANCIES)
- preoperative anemia

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CRF (chronic renal failure) tx on epoetin alfa

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initial effects: 1-2 weeks
Target levels: 2-3 months
- May have to supplement Fe, Folate, and B12

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Epoetin routes

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Subcu preferred to IV

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If hgb in therapeutic range then what do you do with Epoetin alfa?

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HOLD or it will cause HTN

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Adverse effects of Epoetin Alfa

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- HTN (monitor BP and adj HTN meds)
- cardiovascular (CHF, cardiac arrest)
- Thrombotic events- risk is higher if hgb is 12 gm.dL or a rise of hgb > 1gm/dL in 2 weeks --> co admin with anticoagulant

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Carbonyl Iron/Feosol

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less toxic

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Ferrous Sulfate

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- preferred drug for iron def anemia
- DANGEROUS (CHILDREN OD)

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Iron dextrate (IV iron)

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- anaphylaxis can occur (have epi near by)
- deep MI ( irritating ) --> use Z-track

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Symptoms of Iron OD

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- nausea/vomiting
- diarrhea
- shock
- gastric necrosis
- hepatic failure
- pulmonary edema

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Tx of Iron OD

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- measure serum Fe
- gastric lavage
- manage systemic effects
* DEFEROXAMINE if very high

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B12 deficiency

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Megaloblasts

- Macrocytic normochromic

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Most frequent cause of death from B12 anemia

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Cerebral hypoxemia

(or heart failure and arrhythmias)

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Progression of B12 deficiency anemia

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- demylenation of nerves --> paresthesias, reduction of deep tendon reflexes
- later--> memory loss, mood changes, hallucinations, psychoses
- Neruro damage is permanent (not fixed with folic acid- folic acid may actually exacerbate this)
- leukopenia, thrombocytopenia

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Schilling test

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tests B12 absorption

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Dx of B12 def

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normal RBCs, hct, hgb
- increased MCV and cell description

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Cyanocobalamin (B12) adverse effects

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may cause hypokalemia as RBC production increases

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Cyanocobalamin use

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- Do not give IV (give IM or subcu)
- for severe absorption defect
- lifetime therapy

"cobal" names

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Big cause of Folic acid deficiency

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alcoholism (and ciliac spruce)

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Folic acid deficiency and use

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- Similar to B12 but no neuro effects
- thrombocytopenia, leukopenia
- neural tube defects early in gestation (600mg/day before pregnant)
- colorectal cancer
- atherosclerosis (antoxidant effect)

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B12 rich foods

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- muscle meats, dairy

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Iron rich foods

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- apricots
- liver, egg yolk, brewers yeast, wheat germ, muscle meats, fish, fowl

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Signs of hypokalemia

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muscle weakness, irregular heart beat (extra u wave?)

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Folic acid foods

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green vegetables, liver