Diseases of the blood BLOOD DISORDERS/DISEASES

ANEMIA THE MOST COMMON DISORDER OF THE BLOOD LACK OF RBC’S CARRYING OXYGEN Main classes of etiology include: Excessive blood loss – hemorrhage Excessive blood cell destruction – hemolysis Deficient red blood cell production

ANEMIA Diagnosing the type of anemia is based on: Nutrient deficiencies Presence of immature RBC’s Characteristic color and volume of RBC’s Lab values

GENERAL SIGNS AND SYMPTOMS (of all anemias) Pallor or lack of color-especially mucuous membranes Fatigue Dizziness Headaches Decreased exercise tolerance Rapid heartbeat Shortness of breath

GENERAL PROGNOSIS (if anemia is untreated) Heart failure Cardiovascular collapse Shock

IRON DEFICIENCY ANEMIA (most common cause of anemia) ETIOLOGY: Increased iron requirements, Impaired iron absorption Hemorrhage

WHAT DO I NEED TO KNOW ABOUT IRON DEFICIENT ANEMIA? Iron is needed to synthesize hemoglobin--- which is needed to transport oxygen Iron requirements are greatest from birth to age two

WHAT DO I NEED TO KNOW ABOUT IRON DEFICIENT ANEMIA? Pregnancy requires supplements to ensure fetus gets enough iron Sudden growth spurts and onset of menstruation are risks for iron deficient anemia

WHAT ELSE DO I NEED TO KNOW ABOUT IRON DEFICIENT ANEMIA? Absorption takes place in the GI tract so diseases that affect the mucosa of the GI tract can put your patient at a risk for anemia because they limit the availability of Iron needed to synthesize hemoglobin

EXAMPLES OF CAUSES Chronic diseases that cause inflammation (inflammatory changes can suppress red blood cell synthesis in bone marrow and shorten life of RBC’s) Removal of the stomach Chronic disease treatment Bowel disorders

DIAGNOSIS CBC- low H&H Reports of signs and symptoms in

TREATMENT OF IRON DEFICIENCY ANEMIA Diet rich in iron Vitamin supplements with iron IV Supplements if necessary NOTE: iron supplements are constipating and oral doses should not be taken on an empty stomach

PERNICIOUS ANEMIA Etiology: Vitamin B12 absorption or intake is deficient or utilization is inadequate Deficiency in intrinsic factor (a protein in the stomach needed so B12 can be absorbed from the small intestine) Removal of stomach or the bowel Abnormal bacterial growth in small intestine Strict vegetarianism

HOW DOES LACK OF B12 OR INTRINSIC FACTOR CAUSE ANEMIA? The deficiency causes the membranes of immature RBC’s to rupture easily leaving fewer RBC’s to carry oxygen HOW does lack of B12 or intrinsic factor cause anemia

SYMPTOMS OF PERNICIOUS ANEMIA Nausea, vomiting, burning of the tongue Neurological disturbances such as numbness, weakness, poor reflexes GENERAL SYMPTOMS OF ANEMIA SLIDE FOUR

DIAGNOSIS OF PERNICIOUS ANEMIA Health history to rule out inherited lack of intrinsic factor Physical exam shows jaundice, enlarged liver, irregular heart rate CBC abnormal Bone Marrow aspiration/biopsy(cells that make blood cells will be larger than normal)

TREATMENT OF PERNICIOUS ANEMIA Vitamin B12 supplementation (injectable if it cannot be absorbed) NOTE: Pernicious Anemia puts you at a higher risk for stomach cancer

HEMOLYTIC ANEMIA Etiology: Reduction in RBC’s by conditions that accelerate destruction of RBC’s such as: Inherited abnormalities such as hemoglobin defects, enzyme defects and membrane defects that impair intrinsic factor physical properties Infections/immune disorders (HIV, Lupus) Medications (chemotherapy)

SIGNS AND SYMPTOMS OF HEMOLYTIC ANEMIA Mild may have no signs or symptoms General s/s of anemia see slide four Increased serum bilirubin levels from the hemoglobin destruction in destroyed RBC’s Jaundice, dark urine and feces due to accumulation of bilirubin system

DIAGNOSIS OF HEMOLYTIC ANEMIA Enlarged spleen CBC Includes H&H Liver function blood tests Bone Marrow Aspiration and or biopsy

TREATMENTS FOR HEMOLYTIC ANEMIA Blood transfusions Medication Plasmaphoresis Surgery Stem Cell transplants

Blood Transfusions

Blood transfusion reaction Caused by antibodies forming and person rejecting blood being transfused. Stop transfusion immediately.

MEDICATIONS Corticosteroids (Prednisone) - limit immune system from making antibodies(proteins) against RBC’s Rituximab, Cyclosporine – drugs that suppress your immune system

PLASMAPHORESIS A procedure that removes antibodies from the blood using a needle inserted into a vein. The plasma is separated from the rest of the blood and then donor plasma is put back into the blood.

SURGERY Spleenectomy – removal of spleen. An enlarged or diseased spleen may remove more RBC’s than normal. Removing it can help reduce high rates of RBC destruction Note : a healthy spleen helps fight infection and filters out old or damaged blood cells.

APLASTIC ANEMIA bone marrow doesn’t make enough new blood cells because stem cells are damaged (Also caused bone marrow failure) Can be acquired or inheirted

ACQUIRED APLASTIC ANEMIA ETIOLOGY Exposure to toxins—pesticides Radiation and chemotherapy Antibiotics like chloramphenicol Hepatitis, HIV,CMV, Eptstein-Barr virus Lupus, rheumatoid arthritis

SIGNS AND SYMPTOMS OF APLASTIC ANEMIA General s/s from slide four Decreased RBC’s, WBC’s and platelets Nausea Skin rashes

DIAGNOSIS OF APLASTIC ANEMIA History and physical CBC Reticulocyte count (# of young blood cells) Bone marrow aspiration/biopsy

TREATMENT OF APLASTIC ANEMIA Removal of known cause if possible Blood transfusions Blood and marrow stem cell transplants Medications

Blood and Marrow Stem Cell Transplants

MEDICATIONS Stimulate bone marrow-Erythropoieten and colony stimulating factors Suppress immune system Antihymocyte globulin (ATG), cyclosporine and methylprednisone – all three given togeth

SICKLE CELL ANEMIA Blood cells are crescent shaped instead of round, because of abnormal hemoglobin. It is thready and cells are stiff and sticky. Sickled cells block blood flow, causing pain, infection and organ damage.

WHAT CAUSES THE ANEMIA? After days sickle cells die. Bone marrow can’t make new RBC’s fast enough to replace the dying ones

FACTS ABOUT SICKLE CELL ANEMIA It is inherited-one sickle gene from each parent People who have the disease are born with it If sickle cell gene from one parent and normal gene from other parent you have sickle cell trait. Most common in African Americans

SIGNS AND SYMPTOMS OF SICKLE CELL DISEASE General anemia s/s from slide four Acute Pain throughout body is called sickle cell crisis Chronic pain in bones Multiple organ failur Infections and dehydration can contribute to a sickle cell crisis

DIAGNOSIS Blood testing at newborn screening. Amniotic fluid sampling before birth.

TREATMENT If in sickle cell crisis treatment involves pain management with NSAIDS and or opiates, fluids and oxygen if levels are low. Hydroxyurea – medication that causes body to make fetal hemoglobin which helps blood cells from sickling and improves anemia Blood transfusions

COMPLICATIONS Stroke Eye damage Multiple organ failure Leg ulcers Gall stones Priapism (painful erection in males)

EMERGING TRENDS Research on blood and marrow stem cell transplants and gene therapy is ongoing. New medications: Decitabine – prompts body to make Fetal hemoglobin Adenosine A2a receptor agonists – may reduce pain related complications

Disseminated Intravascular Coagulation (DIC) ETIOLOGY: Sepsis Endothelial damage shock

Signs and symptoms Bleeding into intravascular spaces due to destruction of platelets and clotting factors.

Diagnosis Blood test Physical exam

Treatment Platelet transfusions

Hemophilia An inherited clotting disorder due to a deficiency of clotting factor VIII X linked recessive disorder primarily affects males

Signs and symptoms Bleeding If bleeding into joints can cause joint fibrosis Intracranial hemorrhage often cause of death

Diagnosis Genetic testing PT/PTT Platelet count H&H

Treatment Replacement of factor VIII

Leukemia Cancer of the blood Proliferation of immature forms of WBC’s Classified based on whether cells affected are B Cells, T Cells (lymphoid tissue) or Myeloid cells (bone marrow) Occurs 10x more often in children than adults

Etiology Etiology is unknown, however research indicates some cases of leukemia are caused by treatment of other cancers, environmental factors and possibly pre- exposure to viruses

Signs and symptoms Fatigue Weight loss Easy bruising Repeated infections low grade fevers Nosebleeds hemorrhages

Diagnosis CBC Bone marrow biopsy

Treatment Chemotherapy Bone marrow transplantation