ANEMIA

The reduction in the number of RBCs or the amount of Hgb or Hct. A clinical manifestation NOT a specific disease Can occur with many health problems Many types and causes Can result from Dietary problems Genetic disorders Bone marrow disease Excessive bleeding

Hemolytic anemia Many forms of hemolytic anemia More than 200 disorders have been identified. Jaundice Destroyed RBCs cause bilirubin Enlarged spleen and liver Hyperactive with macrophage phagocytosis of the defective RBCs Accumulation of the hemoglobin molecules can obstruct renal tubules Tubular necrosisMajor focus of treatment is to maintain renal function

Breaking down RBCs faster than they can be made.

G6PD
(congenital) Glucose-6phosphate dehydrogenase Screening for this deficiency is necessary before donating blood, because cells deficient in G6PD can be hazardous. Pt. is usually asymptomatic until exposed to infection or certain drugs i.e..) sulfonamides, aspirin, quinine derivatives, thiazide diuretics, high doses of vitamin C Treatment Hydration Osmotic diuretics Transfusions

Folic acid deficient anemia

(nerve function ok) 3 Common causes: Poor nutrition lacking green leafy veges, liver, yeast, citrus, dried beans and nuts Malabsorption Crohns, chronic alcohol abuse w/ malnutrition Drugs anticonvulsants, oral contraceptives slow or prevent the absorption and conversion of folic acid to its active form, methotrexate

Immunohemolytic anemia (autoimmune)

Iron deficiency anemia (microcytic)

Common form of anemia Can result from blood loss, poor intestinal absorption, inadequate diet. Serum ferritin values less than 12g/L. Pt experiences weakness, fatigue, pallor. Evaluate patients for abnormal bleeding.

Vitamin B12 deficiency anemia (macrocytic)

(lose nerve function) Results from poor intake of foods containing vit B12 (vegetarians, or diets lacking dairy products. B12 needed for normal nerve function. Pernicious anemia is due to failure of absorption of B12 May be mild or severe Pallor, jaundice, glossitis, fatigue and weight loss, parasthesis.

Aplastic anemia

Can result from RBC

destruction as a result of trauma, malarial infection, exposure to certain chemicals or drugs, and autoimmune reactions

RBCs are viewed as non-self by the immune system Immune system products attack a persons own RBCs for unknown reasons. 2 types:
Warm antibody Cold antibody

Management
Diet 10-15mg of iron per dayonly 5-10% of dietary iron is absorbed Red meat, organ meat (liver), egg yolks, kidney beans, leafy green vegetables, and raisins Oral iron supplements Ferrous sulfate IM iron solutions if anemia is severe

Treatment
Management depends on severity of symptoms
Steroid therapy - 1st line of treatment Splenectomy Intense immunosuppressive therapy Cytoxan Imuran Plasma exchange

Management
Diet Animal proteins, eggs, nuts, dairy products, dried beans, citrus fruit, leafy green vegetables Vitamin supplements if severe Shilling test for pernicious anemia Measures the presence of Vitamin B12 in the urine after the pt is given an oral dose of radioactive Vitamin B12 Monthly B12 injections for pernicious anemia CaloMist nasal spray

A deficiency of circulating RBCs because of failure of the BM to produce these cells. Can be caused by long-term exposure to ionizing radiation or infection. Usually occurs along w/ leukopenia (low WBC) and thrombocytopenia (low plts). Pancytopenia- a deficiency of all 3 cell types Quite common in aplastic anemia

Treatment
Blood transfusions are the main course of treatment. Immunosuppressive therapy helps pts. w/ types of aplastic anemia that have a similar disease course to that of autoimmune problems. BM transplant replaced defective stem cells and can cure the disorder. Splenectomy

Management
Prevention Identify highrisk patients Diet Scheduled folic acid replacement therapy

Manitol

Risk Factors: elderly debilitated alcoholics

Splenectomy
May be performed for pts w/ an enlarged spleen that is either destroying normal RBCs or suppressing their development. Infection
Rememberspleen performs many immune protection functions!

Polycythemia Vera
A cancer of the RBCs 3 Major Hallmarks 1. massive production of RBCs 2. excessive leukocyte production 3. excessive production of platelets. Disease with sustained increase in blood Hgb levels to 18 g/dL, RBC count of 6 million/mm3, or a Hct of 55% or >. Pts facial skin and mucous membranes are dark and flushed in appearance. Areas appear purplish and cyanotic. Pts c/o severe itching caused by dilated blood vessels and varied tissue oxygenation. Superficial veins are visibly distended Blood is thick and slow moving, placing increased demands on the heart, causing hypertension. Hypoxia

Myelodysplastic Syndromes
A group of disorders caused by the formation of abnormal cells in the BM. The abnormal cells are usually destroyed after being released into the bloodstream. Has cancer-like features and is considered to be a precursor to cancer. Many subtypes 30% of patients with MDS will develop acute leukemia Anemia Neutropenia/Thrombocytopenia

Hemachromatosis
A disorder of iron regulation. Inherited disease where excess free iron is deposited in various organs including the liver and pancreas. Iron exists in 2 states: Ferrous (state of free iron, very toxic) ferric The free radicals damage cells and tissues, leading to inflammation, cirrhosis of the liver, diabetes, spleen, pancreas and cardiac problems. The chemical properties of iron cannot be broken down and eliminated, leading to iron overload. Occurs more frequently in men Often a silent disorder Not often detected until permanent damage has occurred

Risk Factors
Normal physiological changes associated with aging Chemical exposures
Pesticides, benzene

Treatment
Malignant disease Progresses in severity over time. If untreated, death occurs. Few live longer than 2yrs after diagnosis if untreated. Conservative treatment of repeated phlebotomies (2-5 times per wk) can prolong life 10-15 yrs. Removing the RBCs to decrease the # thereby reducing blood viscosity. Treatment aims to prevent clot formation and includes anticoagulants.

Tobacco smoke Exposure to radiation and chemo

Treatment
Focuses on symptom management and supportive care
Blood transfusions for anemia Platelet transfusions for severe thrombocytopenia Deferasirox (Exajade) Deferoxamine mesylate (Desferal, Desferrioxamine)

As the disease progresses, treatment includes

Chemo Radiation Injections of radioactive phosphorus

Chelation therapy