Thalassemia
Autosomal recessive genetic disorder of inadequate production of normal hemoglobin. found in meditteranean ethnic groups. |
Clinical Manifestation |
Asymptomatic | Major retardation | Life threatening |
Splenomegaly, Heptomegaly |
Pathophysiology |
Genetic defect, synthesis of one of a or b globin chains, production of abnormal Hgb and RBC, Hemolysis, Anemia |
Collaborative Care |
• No specific drug or diet are effective for treatment |
• Minor (hetero-beta) : body adapts to low Hgb |
• Major (Homo-beta) : Blood transfusions with IV deferoxamine (used to remove excess iron from the body) |
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Polycythemia
A net increase in the total number of Red blood cells. Overproduction may be due to: |
- myeloproliferative syndrome in bone marrow |
- reaction to chronically low oxy lvls or malignancy |
Pathophysiology |
Myeloproliferative due to hypoxia, the stem cell grow uncontrollably, BM becomes Hypercellular, Blood becomes thick to sluggish circulation, BM becomes fibrotic. |
Complications |
- Increased viscosity of blood |
- Hemorrhage and thrombosis |
Treatment |
- Phlebotomy |
- Myelosuppressive agents : Intron A, Agrylin, Aspirin |
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Idiopathic Thrombocytopenia Purpura (ITP)
Disorder of decreased platelets (below 150,000) |
Causes |
•Low production of platelets |
•Increased breakdown of platelets |
Symptoms |
• Bruising, Nosebleeds, Petechiae |
Immune Thrombocytopenic Purpura |
• Abnormal destruction of circulating platelets |
• Autoimmune disorder |
• Destroyed in hosts' spleen by macrophages |
Thrombotic Thrombocytopenic Purpura |
• Increase agglutination of platelets, form microthrombi |
Diagnostic Studies |
•Platelet count, Hgb/Hct, prothrombin time (PT) |
•Activated partial thromboplastin time (aPTT) |
Treatments |
•Corticosteroids (Bethamethasone, prednisone, prelone) |
• Plasmapheresis, Splenectomy, platelet transfusion |
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