Blood and Blood Forming Organs Diseases and Disorders

1. Blood and Blood Forming Organs Diseases and Disorders Shelby Engel and Sarah Heins

2. Blood transports necessary nutrients and hormones • Color, volume, viscosity, and pH • RBCs/erythrocytes – transport oxygen • 4.2-6.3 million live 120 days • Hemoglobin typically 13.5-18g/100 ml for males, 12-16 for females • WBCs/leukocytes – infection prevention • 4,500-11,000 count • >11,00 indicates infection • Platelets/thrombocytes cause blood coagulation • Clump at site of injury • Calcium builds at site • Fibrin clot is formed • Clot is removed, scar tissue • A, B, AB, O +/- blood classified by antigens in blood • Blood forming organs • Lymph nodes – protection from pathogens • Bone marrow – major blood cell-producing organ • Spleen – produces lymphocytes, plasma cells, and antibodies; filters microorganisms in blood, removes old blood from body • Liver – blood-forming organ; aids in blood coagulation Anatomy & Physiology

3. Increases or decreases in production of cells – RBC, WBC, Platelets Common Signs & Symptoms

4. Complete blood count – identifies RBCs, WBCs, and platelets; see page 128 in book • Differential – more detailed count, identifies the number of each type of leukocyte • Hematocrit – reflects the amount of red cell mass as a proportion of whole blood • Hemoglobin – reflects oxygen-carrying potential available • Mean corpuscular volume – average size of RBC • Mean corpuscular hemoglobin – average hemoglobin content • Mean corpuscular hemoglobin concentration – average hemoglobin concentration • Blood smears • Biopsy of blood-forming organs • Bleeding time test – prick earlobe and measure time until clot Diagnostic Tests

5. Anemia • Iron Deficiency Anemia • Folic Acid Deficiency Anemia • Vitamin B12 Deficiency Anemia • Pernicous Anemia • Hemolytic Anemia • Sickle Cell Anemia • Hemorrhagic Anemia • Aplastic Anemia • Polycythemias • Polycythemia (Primary or Vera) • Secondary Polycythemia (Erythrocytosis) Disorders of Red Blood Cells

6. Anemia = Most common • A decrease in RBC mass that can be caused by a number of different processes • Can be serious enough if not determined or corrected • Symptoms: • Shortness of breath and headache • Rapid heart beat • Dizziness • Pale skin • Weakness and fatigue • Treatment –Depends on the type of anemia • Prevention – Eating a healthy diet with foods high in iron and b complex vitamins • Vitamin B12 Deficiency Anemia • Results from dietary deficiency in B12 or inability of the digestive tract to absorb it

7. Can be related to poor diet, overcooking vegetables, and alcoholism • Infancy and pregnancy • Symptoms: • Fatigue • Weight loss • Abdominal pain • Black or bloody stools • Chest pain • Treatment – Increase intake of green leafy vegetables, mushrooms, lima beans, and kidney beans • Prevention – Diet high in folic acids Folic Acid Deficiency Anemia

8. Usually affects older individuals and has an unusual cause • People affected usually have an autoimmune disorder • Symptoms: • Pallor (paleness) • Weakness • Confusion • Depression • Numbness in the hands and feet • Diagnosis – History, physical examinations, and blood testing for vitamin B12 • Treatment – Depends on the cause of the deficiency • Absorption and diet – can be treated with oral vitamin tablets or injectable vitamin B12 • Monthly injection for life • Not preventable Pernicous Anemia

9. Increased destruction on RBCs • Can be severe and lead to death • Can be brought on by exposure to chemicals such as benzene; medications, including aspirin and penicillin; and bacterial toxins • Symptoms • Pallor (paleness) • Weakness • Fatigue • Tachycardia (rapid heart rate) • Diagnosis – thorough history, physical examinations, and blood testing • Prevention – Genetic = not preventable; Acquired = preventable with proper screening Hemolytic Anemia

10. Hereditary anemia found in the African-American race • Causes an abnormal sickle shape of the erythrocyte • Thought to have developed a defense against malaria • Symptoms: Can be very mild to severe • Pain in the back, legs, and abdomen (most common) • Fatigue • Irritability • Swollen joints • Leg sores • Gum disease • Sickle Cell Crisis – Group of symptoms, pain is compared to cancer pain Sickle Cell Anemia

11. Diagnosis – History and blood testing • Two blood tests • 1st= Measures the amount of normal and abnormal hemoglobin in the blood • 2nd = Measures the percent of red sickle cells after mixing a small drop of blood with a deoxygenating agent • Treatment – No cure and treatment is symptomatic • Increased fluid intake can help by increasing blood volume and improve sickle cell movement • Prevention – Genetic counseling and decision to avoid childbearing Sickle Cell Anemia cont.

12. Loss of whole blood and can be called blood loss anemia • Common complication = hypovolemic shock • Symptoms • Pallor (paleness) • Cool clammy skin • Tachypnea • Tachycardia • If large amounts of blood is lost symptoms can include: • Dizziness • Fainting • Extreme thirst (result from dehydration) • Diagnosis – History and physical examinations • Treatment – Acute blood loss = apply oxygen, intravenous fluids and liquids taken by mouth; Severe blood loss = blood transfusion • Prevention – Accident prevention and controlling chronic bleeding Hemorrhagic Anemia

13. Failure of bone marrow to produce blood components • Due to injury or destruction of blood-forming area of the bone marrow • Causes : Chemotherapy, radiation, viruses, and chemical toxins • Symptoms: This decrease in blood cells leads to anemia, infection, and hemorrhage • Diagnosis – History and physical examination with blood testing • Treatment – Severe cases have a poor prognosis with 50% fatality • Discontinuing or avoiding the causative agent • Bone marrow transplantation or blood transfusion • Prevention – Avoiding causative agent, but often the agent is unknown which makes prevention impossible Aplastic Anemia

14. Condition of too many blood cells • Caused by hyperplasia of the cell-forming tissues of the bone marrow • Symptoms: • Raised blood pressure • Enlarged spleen • Bloodshot eyes • Redness of skin • Shortness of breath • Dizziness • Diagnosis – Can be accidently discovered through routine blood testing • Treatment – Reduce the red cell count, blood removal at regular intervals will reduce the volume (common treatment) • Prevention – Cannot be prevented Polycythemia (Primary or vera)

15. Red cell numbers increase • Symptoms: • Headaches • Weakness • Fatigue • Lightheadedness • Shortness of breath • Diagnosis – History, physical exam, and blood testing • Treatment – Treated by addressing the cause of the disorder • Prevention – Stopping the causative factor or by doing the things that deprive the body of needed oxygen, avoiding smoking, and avoid living in high altitudes Secondary Polycythemia(Erythrocytosis)

16. Common problems of the hematologic system • Mononucleoisis • Leukemia • Lymphoma • Hodgkin’s Disease • Non-Hodgkin’s Lymphoma • Multiple Myeloma Disorders of White Blood Cells

17. Primarily affects children and young adults • Contagious by direct contact with saliva • Last several weeks • Epstein-Barr Virus (EBV) • Fatigue, sore throat, fever, swollen lymph glands, spleen enlargement • Diagnosed by WBC count high • Rest, analgesics, throat gargles, avoiding sports activities • Prevent through good oral hygiene Mononucleoisis, the “kissing disease”

18. Malignant neoplasm of the blood-forming organs • High production of leukocytes, causing decrease in erythrocytes and platelets • Acute common in children; chronic common in older adults • Cause is unknown • Fatigue, headache, sore throat, dyspnea, bleeding of the mucous membranes, bone and joint paint, enlarged lymph nodes • Diagnosed by clinical history and blood studies, or bone marrow biopsy • Treated by aggressive chemotherapy and a bone marrow transplant • Complete remission occurs 50% of the time • No known ways to prevent Leukemia

19. Neoplasms that affect lymphoid tissue • Lead to impaired immunity • Most common type of blood cancer • Hodgkin’s Disease • Non-Hodgkin’s Lymphoma • Multiple myeloma Lymphomas

20. Most common lymphoma • Orderly spread of disease from one lymph node group to another • Cause is thought to be viral • Painless enlargement of the lymph nodes in the neck, weight loss, fever • Primarily affects young adults, average age of 35 • Men at higher risk than women • Reed-Sternberg • Diagnosed with lymph node and bone marrow biopsy • Treated with radiation and chemotherapy • Complete cure after 5 years of remission • 93% cure rate – one of the most curable forms of cancer • 20% mortality rate • No known preventive measures Hodgkin’s Disease

21. Not containing the Reed-Sternberg cell characteristics; more widespread than Hodgkin’s • Affects more older adults, average age of 50 years • Men are affected 1.5X more often than women • Cause is unknown • Patients who receive/have received immuno-suppressive medication 100X more likely chance of developing NHL • Painless enlargement of lymph nodes in neck, axilla, and inguinal areas; fever, night sweats, weight loss • Diagnosed by absence of Reed-Sternberg, and lymph node and bone marrow biopsy • Combination of radiation and chemotherapy • Those at increased risk – exposure to pesticides, solvents and fertilizers • Cure rate of 63% • Mortality rate of 33% Non-Hodgkin’s Lymphoma

22. Malignant neoplasm of plasma cells • Causes weakness in the bone, leading to fractures and bone pain • Cause is unknown • Occurs increasingly with age, peaking in 70s, more common in men • One of the most common neoplasms affecting the bone • Decrease in other blood components, causing anemia, leukocytopenia and thrombocytopenia • Causes excessive blood calcium levels, tissue damage, kidney failure • Diagnosed with X-ray, high calcium levels, presence of Bence Jones protein, excessive plasma cells • Treatment is poor – death is usually within 2-3 years • Prevent by avoiding herbicides, petroleum products, heavy metals and radiation Multiple Myeloma

23. Platelet disorders vary in terms of cause, severity, and treatment • All share common symptom of bleeding • Many of these disorders are inherited • Hemophilia • Thrombocytopenia • Disseminated Intravascular Coagulation (DIC) Disorders of Platelets and Clotting

24. Group of bleeding disorders characterized by abnormally slow clotting and long bleeding times • X-linked hereditary bleeding disorder • Commonly occurs in male children • Hemophiliacs lack a blood protein that plays a role in clot formation • Symptoms: • Frequent nosebleeds • Bruising • Prolonged bleeding in a male child (can indicate hemophilia) • Can be mild to severe • Severe cases are called hemarthrosisor bleeding into joints, which is painful, and recurrent episodes can lead to joint deformity Hemophilia

25. Diagnosis – Confirmed by obtaining a detailed medical history, physical examination, and blood testing • Blood tests include measurement of clotting time and the presence of blood proteins • Treatment – No cure • Aimed at prevention of injury and treatment of symptoms • Whole blood transfusions can be needed • Prevention – Because it is inherited it can only be prevented by genetic testing of possible carriers with the decision to not have children Hemophilia cont.

26. Decrease in platelets that leads to an inability to clot blood normally • Can be due to inadequate or abnormal platelet production or destruction • Cause is unknown • Symptoms: • Abnormal bleeding in the skin, mucous membranes, and internal organs • Small hemorrhagic spots on skin; larger purplish hemorrhagic spots • Frequent nosebleeds • Blood in urine • Diagnosis – Individual clinical history along with platelet count and bleeding time Thrombocytopenia

27. Treatment : • Avoid tissue trauma to reduce potential bleeding • Administration of vitamin K to improve clotting • Transfusion on platelets • Prevention- Most cases are not preventable, but there are two steps that can be taken to prevent complications • Avoid taking medications that decrease platelet aggregation or stickiness, thus making them less likely to clot. (Ex: Aspirin or Ibuprophen) • Avoid heavy drinking because alcohol slows platelet production Thrombocytopenia cont.

28. Abnormal clotting followed by abnormal bleeding • Blood starts to coagulate or clot throughout the entire body • Usually follows major trauma such as blood transfusion reaction, surgery, septicemia, complicated childbirth, massive tissue destruction, shock, malignancy, or snakebite • Symptoms: • Black and blue fingers and toes • Large thrombus formation (can lead to life-threatening pulmonary embolism) • Oozing of blood • Vomiting • Blood in stool • Symptoms associated with anemia Disseminated Intravascular Coagulation

29. Diagnosis – History of trauma and blood studies • Treatment – Identifying and treating underlying cause can stop DIC • Medications • Heparin – an anticoagulant medication to halt the formation of thrombi • Platelet administration to increase clotting ability • Difficult to manage • This condition is usually life-threatening and can lead to death • Often times it is referred to as “Death Is Coming” (DIC) • Prevention – Getting prompt medical treatment for any condition that might bring on this disorder DIC cont.

30. Thalassemia • Hereditary hemolytic anemia • Mediterranean descent • Dysfunctional RBCs leading to anemia symptoms • Von Willebrand’s Disease • Hereditary, congenital bleeding disorder • Deficiency in clotting and platelet function • Lymphosarcoma • Similar to Hodgkin’s Disease • With remission, prognosis is good Rare Diseases

31. Acquired Immunodeficiency Syndrome (AIDS ) affects thousands of children in the US • 1980s – HIV infection mainly acquired through blood transfusions • Today – Most all HIV infections in children are a result of maternal-fetal transfer through blood (also called perinatal transmission) • Symptoms: Many children to not experience symptoms and live a normal life for years • Diagnosis – When T-cell count drops below 200 cells per microliter • Treatment – Good nutrition, antiviral drugs, and other support therapies • Prevention- prevent mother-to-child transmission, provide pediatric treatment, and prevent infection among adolescents Childhood Diseases:Acquired Immunodeficiency Syndrome& Leukemia

32. Cancer.gov • CDC.gov • Human Diseases, 3rd Ed., M. Neighbors & R. Tannehill-Jones References