HEMATOLOGY

1. HEMATOLOGY

2. OBJECTIVES • Upon completion, the student will be able to: • Identify the anatomy and physiology of the hematopoietic system. • Discuss the following: a) Plasma b) Red blood cells (erythrocytes) c) Hemoglobin d) hematocrit e) White blood cells (leukocytes)

3. OBJECTIVES f) Platelets, clotting, and fibrinolysis g) Hemostasis • Identify the following: a) Inflammatory process b) Cellular and humoral immunity c) Alterations in immunological response • Identify blood groups • List erythrocyte disorders • List leukocyte disorders

4. OBJECTIVES • List platelet and clotting disorders • Describe how acquired factor deficiencies may occur. • Identify the components of the physical assessment as the relate to the hematology system. • Describe the pathology and clinical manifestations and prognosis associated with: a) Anemia b) Leukemia c) Lymphomas

5. OBJECTIVES d) Polycythemia e) Disseminated intravascular coagulopathy f) Hemophilia g) Sickle cell disease h) Multiple myeloma 11. Given several scenarios involving patients with hematological problems, provide the appropriate assessment, management, and transport.

6. General Assessment & Management • Patients with hematopoietic system disorders may present with a variety of symptoms, including: • Fevers • Hemodynamic instability • History is the key. • Most of these disorders are chronic conditions that have an acute exacerbation. • Treatment is primarily supportive

7. General Assessment & Management • Some require fluid administration. • Oxygenation • Rapid transport is necessary, with hemodynamic instability

8. Scene Size UP • Approach is the same as for any other patient and includes BSI. • General Impression • Trauma vs. medical • How much distress is the patient in?

9. Initial Assessment • Determine responsiveness • ABCs • Transport priority

10. Focused History and Physical Exam • As always requires a conscious patient. • Let the history determine the physical in the conscious medical patient • In the unconscious trauma patient the physical exam becomes the driving force

11. SAMPLE History • Again requires a conscious patient • Determine vital signs and place the pulse oximeter

12. Physical Exam • Nervous System: • Level of consciousness • Numbness or motor deficits • Examine the eyes for abnormalities a) visual disturbances or visual loss

13. Physical Exam • Skin: • Jaundice (liver disease) • Florid (reddish) – polycythemia • Pallor – anemia • Petechiae – tiny red dots in the skin • Purpura – large purplish blotches related to multiple hemorrhages into the skin. • Pruritus – itching

16. Physical Exam • Lymphatic: • Lymphatic system is affected early in hematopoietic disease, especially those of the immune system. • Palpate lymph nodes of the neck, clavicle, axilla, and groin • Not any enlargement

17. Physical Exam • Gastrointestinal • Effects can be quite varied • Epistaxis is common • Bleeding of the gums is early finding • Liver failure: slows clotting time, jaundice due to bilirubin levels increasing • Abdominal pain is common

18. Physical Exam • Musculoskeletal • Autoimmune diseases such as rheumatoid arthritis result from the body’s immune system attacking various tissues in the joints. • Autoimmune diseases tend to affect more than one joint

19. Physical Exam • Cardiorespiratory • Effects are varied • Develop dyspnea, tachycardia and chest pain (anemia) • Heart failure

20. Physical Exam • Genitourinary • Typically due to bleeding disorders or infection • Hematuria • Menorrhagia (heavy menstrual bleeding) • Frank vaginal bleeding (dysfunctional uterine bleeding) • Sickle cell anemia, later stages, can cause priapism

21. General Management of Hematopoietic Emergencies • Close attention to airway and ventilation status • High-concentration oxygen, assist ventilations as necessary • Consider fluid volume replacement • Be alert for dysrhythmias and treat accordingly • Transport and provide comfort, including analgesia and psychological support

22. Diseases of the Red Blood Cells • Result from too many, to few or improperly functioning • Excess of RBCs: polycythemia • Inadequate RBCs: anemia • Function (either hemoglobin structure and function with the red blood cell membrane: thallasemias and sickle cell anemia

23. Anemias • Most common disease of RBCs. • Hematocrit of less than 37% in women and 40% in men • Most remain asymptomatic until hematocrit drops below 30% • Anemia is actually a sign of an underlying disease process that is either destroying red blood cells and hemoglobin or decreasing the production of RBCs and hemoglobin

24. Anemias • Blood loss, either acute or chronic, also can cause anemia. • Anemias that result from the destruction of RBCs are called hemolytic anemias • Genetic or acquired • Hereditary hemolytic anemias: a) sickle cell anemia b) thalassemia c) glucose-6-phosphate dehydrogenase deficiency

25. Anemias • Acquired hemolytic anemias can result from immune system disorders, drug effects, or environmental effects • Anemias caused by inadequate red blood cell production include such problems as: • Iron Deficiency anemia • Pernicious anemia • Anemia of chronic disease

26. Anemias • Anemia is a sign, not a disease process in itself. • Anemia will result in hypoxia. • S&S vary based on: • Rapidity of its onset • Patient’s age • Underlying general health

27. Anemias • Mild Anemia may not exhibit signs or symptoms until the body is stressed, then displaying mild dyspnea, fatigue, palpitations, and syncope. • Chronic anemias may present S&S of pica (craving of unusual substances such as clay or ice), headache, dizziness, ringing in the ears, irritability or difficulty concentrating, pallor, and tachycardia. Possibly angina pectoris

28. Anemias • If rapid development, the body does not have time to compensate for the change: Presentation of shock. • If slower onset, the body can adjust to oxygen’s reduced availability • Field Treatment is primarily symptomatic. • Maximizing oxygenation • Stemming blood loss, volume replacement as indicated • Transport

29. Sickle Cell Disease • Disorder of RBC production. • Abnormal chemical sequence that gives RBCs a C, or sickle, shape when oxygen levels are low • Patients have a chronic anemia that results from destruction of abnormal RBCs (hemolytic anemia) • Sickled cells also increase viscosity, leading to sludging and obstruction of the capillaries and small blood vessels.

30. Sickle Cell Disease • Blockage is common and usually follows periods of stress. • Process known as vasoocclusive crisis, characteristic of Sickle Cell Anemia • Sickle cell disease is inherited. • Primarily affects African-American as well as Puerto Ricans and people of Spanish, French, Italian, Greek and Turkish heritage.

31. Sickle Cell Disease • Patients will develop three types of problems: • Vasoocclusive Crises: causing musculoskeletal pain, abdominal pain, priapisms, pulmonary problems, renal crises, and CNS crises. • Hematological Crises: fall in hemoglobin level • Infectious Crises: functionally immunosuppressed making them particularly vulnerable to encapsulated bacteria. Infections become common and are usually the cause of death.

32. Sickle Cell Disease • Prehospital care is primarily supportive. • ABCs • Oxygenation • IV Therapy • Analgesics as necessary (may require large amounts of narcotics for pain relief) • Transport

33. Polycythemia • Abnormally high hematocrit. • Due to excess production of RBCs • Relatively rare and occurs in patients over 50 • Can occur secondary to dehydration • Increases the risk of thrombosis, which is the cause for most deaths

34. Polycythemia • Signs and symptoms vary: • Principal finding is a hematocrit of 50% or greater • Increased number of WBCs and platelets • Increased number of RBCs could result in platelet dysfunction, leading to: a) bleeding abnormalities b) spontaneous bruising c) GI bleeding 4. Complaints include: headache, dizziness, blurred vision, itching and GI disease

35. Polycythemia • Prehospital care is supportive: • ABCs • Oxygenation • IV therapy • Transport • Principal treatment is phlebotomy, which removes excess RBCs

36. Diseases of the WBCs • Result from too few white blood cells • Leukopenia • Leukocytosis (too many) • Improper WBC function

37. Leukopenia/Neutropenia • Normal WBC count ranges from 5,000-9,000 per cubic millimeter of blood. • A drop in the number of WBCs indicates a problem with WBC production in the marrow or destruction of WBCs • Patients are more vulnerable to bacterial infections • Treatment is primarily supportive

38. Leukocytosis • Increase in the number of circulating WBCs. • Occurs when the body is exposed to an infectious agent, and the body is attempting to combat the disease • WBC counts between 10,800 and 23,000 (characteristic of bacterial infection) • Causes include: bacterial infection, rheumatoid arthritis, DKA, leukemia, pain, and exercise.

39. Leukocytosis • WBC count higher than 30,000 is called a leukemoid reaction. • This is usually a problem with WBC production • Patient should be evaluated for leukemia

40. Leukemia • Cancers of hemoatopoietic cells • WBCs in the bone marrow begin to replicate abnormally • Cells proliferate initially in the bone marrow and then spread to the peripheral blood.

41. Leukemia • Most common types of leukemia: • Acute lymphocytic leukemia (ALL) • Acute myeologenous leukemia (AML) • Chronic lymphocytic leukemia (CLL) • Chronic myeologenous leukemia (CML) • Hairy cell leukemia

42. Leukemia • ALL is primarily a disease of children and young adults • CML occurs in both children and adults • AML,CLL and hairy cell occur in 6th to 7th decade • Treatments such as chemotherapy, radiation therapy and bone marrow transplantation have resulted in cures of certain types of leukemia. • Deaths secondary to infections or bleeding

43. Leukemia • Signs and symptoms vary: • Most will have a moderate to severe anemia as the cancerous cell production overwhelms the bone marrow • Thrombocytopenia (abnormal decrease in platelets) is common for the same reason • Patients will appear acutely ill • Febrile, weak • Enlarge lymph nodes, history of weight loss/anorexia • Sternum tenderness (increased bone marrow activity)

44. Leukemia • Prehospital treatment is primarily supportive • Position of comfort • ABCs • Oxygenation • IV • Consider analgesics

45. Lymphomas • Cancers of the lymphatic system • Malignant lymphoma is typically classified as follows: • Hodgkin’s lymphoma • Non-Hodgkin’s lymphoma

46. Lymphomas • Malignant lymphoma is classified by the cell type involved, which indicates the stem cell from which the malignancy arises. • Most common presenting sign of non-Hodgkin’s lymphoma is painless swelling of the lymph nodes. • Hodgkin’s lymphoma typically have no related symptoms.

47. Lymphomas • Patients typically complain of: fever, night sweats, anorexia, weight loss, fatigue, and pruritis. • Treatment is based on symptom presentation and includes: • ABCs • Oxygenation • IV • Analgesics

48. Diseases of the Platelets/Blood Clotting Abnormalities • Thrombocytosis (increased platelets) • Thrombocytopenia (reduced platelets) • Hemophilia A, Hemophilia B can affect coagulation system • Some of these are hereditary in nature while others are acquired

49. Thrombocytopenia • Abnormal decrease in the number of platelets. • Due to: • Decreased production • Sequestration of platelets in the spleen • Destruction of platelets • Any combination of the three • Acute idiopathic thrombocytopenis purpura (ITP): results from destruction of platelets by the immune system

50. Thrombocytopenia • ITP is seen most commonly in children following a viral infection • Characterized by bruising, bleeding, and a falling platelet count • Chronic ITP occurs in adult women and is often associated with autoimmune disease • Prehospital treatment is supportive