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N114 Hematology
N114 - Hematologic dysfunction
| Question | Answer |
|---|---|
| What is anemia? | A decrease in the oxygen carrying capacity of the blood caused by underlying disease or injury |
| What is the most common type of anemia? | Iron deficiency anemia |
| What can lead to iron deficient anemia? | Iron deficiency anemia occurs when dietary iron is not supplied to meet growth demands once fetal iron stores are depleted. |
| Why is iron so important? | Iron is needed for the formation of hemoglobin. |
| What are some clinical manifestation directly related to the severity of the tissue hypoxia? | Muscle weakness, easy fatigability, poor sucking, paleness, hair loss, rapid pounding heart beat, underdevelopment, headache, light-headedness, slowed thought procress, depression |
| How is anemia treated? | The underlying cause must be treated first, then replacement of depleted iron stores can occur. |
| What is sickel cell anemia? | It is an autosomal recessive disorder. Normal hemoglobin (HbA) is partly or completely replaced by abnormal sickle hemoglobin (HbS). |
| What is the patho of sickled cells? | Features of SCA are the result of obstruction caused by sickled RBCs or increased RBC destruction. When sickled cells clog vessels, hypoxemia occurs. |
| Can sickled cells return to normal? | Yes, most cells can regain a normal shape when rehydrated or reoxygenated. |
| When are symptoms first seen? | Most are asymptomatic in the first 6 months due to the presence of fetal hemoglobin (HgF) which does not sickle. Symptoms are seen in the toddler or preschooler during a crisis following an acute respiratory or GI infection. |
| What areas and symptoms are seen during a crisis? | Hand-foot syndrome - tender, warm & swollen extremities. Cerebrum-CVA - peak between ages 4-6. Chest-symptoms resembling pneumonia. Genitals-priapism. Other effected areas-lumbosacral spine, knee, shoulder, elbow or femur. |
| What is a sequestration crisis? | Excessive pooling of blood in liver & spleen. Can lead to cardiovascular collapse & shock since the spleen can hold 1 |
| What is an aplastic crisis? | Diminished RBC production resulting in profound anemia. Sickled cells live for only 10-20 days resulting in abnormally low numbers of RBCs. |
| What is a sickledex test? | Sickle-turbidity test - via finger stick with results in 3 minutes, does not distinguish between trait & disease. |
| What test is done to distinguish between the trait & the disease? | Chorionic villus sampling or amniocentesis can be done before birth. Hemoglobin electrophoresis is done after birth. |
| What is hemophilia A? | Classic hemophilia caused by a deficiency of factor VIII |
| Where does bleeding usually occur? | Can occur anywhere, most frequently internal bleeding is into joint cavities and muscles. |
| When can bleeding occur? | Depending on the concentration of factor VIII. It can occur spontaneously or after trauma or surgery. |
| How is hemophilia diagnosed? | Can be diagnosed during circumcision. Usually by PTT (partial thromboplastin time) or thromboplastin generation test - can identify which factors are deficient - VIII or IX |
| How is hemophilia treated? | Administration of factor VIII - 3 or 4 times per week. DDAVP - vasopressin - nasal spray, PO, liquid, nose drops, increases factor VIII by releasing from storage sites. |
| What is immune or idiopathic thrombocytopenic purpura? | ITP is an acquired hemorrhagic disorder characterized by thrombocytopenia - decreased platelet count, and purpura - a discoloration caused by petechiae. |
| What are the symptoms of ITP? | Onset occurs 1-4 weeks following a viral infection. May have bleeding from mucous membranes. |
| How is ITP treated? | Activity is restricted while platelet count is low. Treatment may include prednisone, IV immune globulin (IVIG), and anti-D antibody |
| What are the symptoms of lead poisoning (plumbism)? | May be asymptomatic. Can affect any part of body, renal, hematologic & neuro. Brain & neuro system are vulnerable in young children. Lead is stored in the bone. Black lines between gums & teeth |
| How is lead poisoning treated? | Levels over 45 micrograms/dL require chelation therapy. Calcium disodium edentate - EDTA, succimer - DMSA, British altilewiste - BAL. All have potential toxic side effects |
| How is EDTA administered? | given IV over several hours, IM if on fluid restrictions |
| How is BAL administered? | Given deep IM - contraindicated with peanut allergy |
| How is Succimer given? | Orally over 19 day period. |
Created by:
jrb265
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