Chapter 24

Hematologic Emergencies

National EMS Education Standard Competencies Medicine

Integrates assessment findings with principles of epidemiology and pathophysiology to formulate a field impression and implement a comprehensive treatment/disposition plan for a patient with a medical complaint.

National EMS Education Standard Competencies

Hematology

• Anatomy, physiology, pathophysiology, assessment, and management of: − − Sickle cell crisis Clotting disorders

National EMS Education Standard Competencies

• − − − − Anatomy, physiology, epidemiology, pathophysiology, psychosocial impact, presentations, prognosis, and management of common or major hematological diseases and/or emergencies Sickle cell crisis Blood transfusion complications Hemostatic disorders Lymphomas

National EMS Education Standard Competencies

• Anatomy, physiology, epidemiology, pathophysiology, psychosocial impact, presentations, prognosis, and management of common or major hematological diseases and/or emergencies (cont ’ d) − Red blood cell disorders − − White blood cell disorders Coagulopathies

Introduction

• EMS systems rarely respond to hematologic emergencies. • Hematologic disorder: any disorder of the blood − − Hemolytic disorders Hemostatic disorders

Introduction

• Hematopoietic system − Organs and tissues involved in the production of blood components

Blood and Plasma

• Blood performs the following functions: − − − Respiratory Nutritional Excretory − − Regulatory Defensive

Blood and Plasma

• Blood is made of: − Plasma: • • 92% water 55% of total blood volume − Formed elements: • 45% of total blood volume • Includes RBCs, WBCs, and platelets

Blood and Plasma

• The production of RBCs occurs within stem cells.

− − Stimulated by erythropoietin Five days to mature • Average life span: 4 months − Hemoglobin carries oxygen to the tissues.

Blood and Plasma

• Three common lab tests: − − − RBC count Hemoglobin level Hematocrit measurement

Blood and Plasma

• White blood cells − − Larger than RBCs Immunity against “ foreign invaders ” − Derived from stem cells − Several types • Platelets − Smallest formed element − Clot the blood • Hemostasis − Two thirds circulate through the blood.

Blood-Forming Organs and RBC Production

• The major players in the hematologic system are the: − − − Bone marrow Liver Spleen

The Inflammatory Process

• All of the body ’ s cells and blood contain antigens at birth.

− Antigens activate the immune system.

• Autoimmune diseases − Identify the body ’ s own antigen as foreign

The Immune System

• Processes protect against pathogens and other unwanted materials.

− Must distinguish between: • • Body ’ s own tissue Outside organs

The Immune System

• Native immunity − Nonspecific, maximal response − First line of defense − Associated with initial inflammatory response • Acquired immunity − Specific to vertebrates − A pathogen specific response

The Immune System

• Humoral immunity − Secretion of immunoglobins • Recognizes a specific antigen • Cell-mediated immunity − Macrophages and T-cells destroy pathogens.

The Immune System

• White blood cells − − − Produced in the bone marrow Laboratory values provide information on the immune system.

Baseline count is normally 5,000 –10,000 cells/µL.

The Immune System

Blood Classifications

• Developed to prevent medical problems during replacement − ABO system

Blood Classifications

• Blood contains a secondary antigen called the Rh antigen.

− Possible complications in pregnancy • Hemolytic disease

Hemostasis

• The process of stopping bleeding through: − − − Vasoconstriction (immediate response) Platelet plugging Coagulation • Clots are made up of fibrin.

Hemostasis

• Clotting cascade − Can be initiated through: • • Intrinsic pathway Extrinsic pathway − Coagulopathy: process that interferes with the clotting cascade or hemostasis

Patient Assessment

• Note any life threatening signs or symptoms.

− Ask about patient ’ s history and SAMPLE history.

− A nonjudgmental approach is essential.

Scene Size-Up

• Ensure the scene is safe for entry.

• Consider the mechanism of injury.

• Determine the number of patients.

• Assess for hazards.

Primary Assessment

• An African American or Mediterranean patient reporting severe pain may have sickle cell disease. − Perform cervical spine stabilization if necessary.

Primary Assessment

• Form a general impression.

− Perform a rapid scan and determine LOC.

• Airway and breathing − Inadequate breathing or altered mental status should receive high-flow oxygen or ventilation.

Primary Assessment

• Circulation − − Manage life-threatening conditions.

If hemophilia is suspected, watch for: • • Acute blood loss Bleeding of unknown origin • Hypoxia or shock

Primary Assessment

• Transport decision − − Depends on severity and patient ’ s wishes Transport to the closest facility if: • • Sickle cell crisis Uncontrolled bleeding

History Taking

• Obtain patient and SAMPLE history.

• Do not take a sickle cell crisis lightly.

− Life-threatening situations, characterized by: • • • Signs of pneumonia Swelling of fingers and toes Jaundice

History Taking

• Look for: − − − − − − Changes in LOC Vertigo Feelings of fatigue Syncope Dyspnea Chest pain − Changes in pulse rate and rhythm − − − − Coughing up blood Visual disturbances Muscle pain Stiffness

History Taking

• Is pain isolated or felt throughout?

• Has the patient experienced: − − − − − Skin changes?

Bleeding?

History of liver problems?

Pain for unknown reasons?

Genitourinary or gastrointestinal problems?

History Taking

• If known sickle cell disease, ask: − − − − Have you had a crisis before?

When was the last time?

How did it resolve?

Have you had any illness, unusual amount of activity, or stress lately?

Secondary Assessment

• Performed on scene, en route, or not at all • Perform a physical exam, focusing on major joints.

• Obtain vital signs.

Secondary Assessment

• Evaluate and document mental status.

• In patients experiencing a sickle cell crisis: − − − Respirations are normal to rapid.

Pulse is weak and rapid.

Skin is pale and clammy with low blood pressure.

Reassessment

• Reassess frequently.

• Administer supplemental oxygen.

• Provide ventilation as appropriate.

• Cover to maintain body temperature.

Reassessment

• According to local protocol, administer: − − IV fluid Nitrous oxide • Prehospital care for hemophilia can include IV therapy.

Reassessment

• Inform hospital staff about: − − − Patient history Present situation Assessment findings − Interventions and their results • Document: − − − − Each assessment Your findings Treatment Time of interventions − Changes in patient condition

Emergency Medical Care

• Emergency medical care should include: − − − − − − Oxygen Fluids ECG Comfort Pharmacology Support

Sickle Cell Crisis

• Pathophysiology − Leading inherited blood disorder − Gene defect of the adult-type hemoglobin (HbA) − Defective RBCs have an oblong shape.

Courtesy of Bill Branson/National Cancer Institute

Sickle Cell Crisis

• Pathophysiology (cont ’ d) − Can lead to: • • Aplastic crisis: RBC production temporarily stops.

Hemolytic crisis: Acute RBC destruction

Sickle Cell Crisis

• Pathophysiology (cont ’ d) − May manifest as: • • Vaso-occlusive crisis Acute chest syndrome • Splenic sequestration crisis

Sickle Cell Crisis

• Assessment − Characterizations may include: • • Shortness of breath Inadequate perfusion of the skin • • Jaundice Mild dehydration

Sickle Cell Crisis

• Assessment (cont ’ d) − Patients may have significant pain.

• Pediatric patients typically present with pain in the hands and feet.

• Adults typically report back and proximal extremity pain.

Sickle Cell Crisis

• Management − − − − − Administer high levels of oxygen.

Rapidly transport patient.

Consider IV fluid therapy.

Maintain patient ’ s body temperature.

Recommend that patient rest.

Anemia

• Pathophysiology − Low hemoglobin or erythrocyte level − Usually associated with an underlying disease

Anemia

• Iron deficiency anemia − − Most common type Causes include: • • Gastrointestinal blood loss Menstrual bleeding • Frequent donations or diagnostic test

Anemia

• Pathophysiology (cont ’ d) − May be caused by an inherited hemolytic disorder or a hematologic disorder − Can have serious consequences in high altitudes

Anemia

• Assessment − Patients may feel: • • • Worn down Lack of energy Overexerted • Unable to “ catch their breath ” − Patients may have: • Anginal-type chest pain • • Leukopenia Thrombocytopenia

Anemia

• Management − − − − Check and monitor airway breathing.

Check vital signs frequently.

For chest pain, apply a cardiac monitor.

Blood pressure management and fluid replacement may be needed.

Anemia

• Management (cont ’ d) − − Allow the patient to rest.

Consider rapid transport for: • • Abrupt change in consciousness Hypotension • Significant perfusion inadequacies

Leukemia

• Pathophysiology − Cancer in the lymphoid system − Blood cells develop abnormally and/or excessively, causing: • • Anemia Thrombocytopenia • Leukocytosis

Leukemia

• Pathophysiology (cont ’ d) − Acute leukemia: Bone marrow is replaced with abnormal lymphoblasts − • • • • Chronic leukemia: Abnormal cells accumulate in: Bone marrow Lymph nodes Spleen Peripheral blood

Leukemia

• Pathophysiology (cont ’ d) − Survival depends on several factors: • • Stage of detection Underlying medical condition • Response to treatment − Treated with chemotherapy and radiation

Leukemia

• Assessment − Patient presentation depends on: • • Stage of leukemia Current treatment − Patients may present with: • • Fatigue Headaches • • Bone pain Unexplained bleeding

Leukemia

• Management − • • • As appropriate, provide: • • Airway support Oxygen therapy IV fluid therapy Analgesics Positive support

Leukemia

• Management (cont ’ d) − You may be called because loved ones are uncertain about what to do.

• • Discuss with medical control.

Document all findings.

• Have a refusal/release form signed.

Lymphomas

• Pathophysiology − Group of malignant diseases within the lymphoid system − Classified in two categories

Lymphomas

• Non-Hodgkin − − − Any age Can be hereditary Characterized by progression • • • Indolent Aggressive Highly aggressive • Hodgkin: − Progressive enlargement of lymphoid glands − − Highly rare Peaks between: • • 15 –35 years of age After age 55 –60

Lymphomas

• Assessment − − Require chemotherapy or radiation. Signs and symptoms may include: • • Pallor Feeling hot and then cold or both • • Inadequate perfusion Abnormal ECG rhythms

Lymphomas

• Management − Aggressive pain management − Treat inadequate perfusion and abnormal heart rhythms. − Initiate rapid transport if condition does not improve.

Polycythemia

• Pathophysiology − − − − Overabundance or overproduction of RBCs Multiple causes Can lead to other conditions Treatment usually includes phlebotomy or cancer-type therapy

Polycythemia

• Assessment − − − Findings may widely vary Note the extent and duration of dyspnea.

Has the patient experienced pruritus or changes in skin temperature?

Polycythemia

• Management − Consists of supportive care and transport to the appropriate facility. − Administer oxygen and establish IV as needed.

Disseminated Intravascular Coagulation

• Pathophysiology − − Number of causes Two stages: • • Defibrination Uncontrolled hemorrhage − High mortality rate

Disseminated Intravascular Coagulation

• Assessment − − Identify associated signs and symptoms.

Patients may have: • • Respiratory difficulty Signs of shock • Skin changes

Disseminated Intravascular Coagulation

• Management − Identify underlying cause and establish treatment early.

− − Maintain an airway.

Treat for shock.

Hemophilia

• Pathophysiology − A bleeding disorder in which clotting: • • Does not occur Occurs insufficiently − Two primary types: A and B

Hemophilia

• Pathophysiology (cont ’ d) − Signs and symptoms may include: • Acute and chronic bleeding − Patients may require: • Hospitalization for transfusion • Infusion of factors VIII and IX

Hemophilia

• Assessment − − − − Take care of the ABCs.

Be alert for signs of acute blood loss.

Note any bleeding of unknown origin.

Patients may show signs of hypoxia.

Hemophilia

• Management − − − − Patients may need high-flow oxygen.

Note ECG findings.

IV therapy may be necessary.

Analgesics may be appropriate.

Multiple Myeloma

• Pathophysiology − Number of plasma cells in the bone marrow increase abnormally • • Forms tumors in the bone Results in anemia and susceptibility to infection

Multiple Myeloma

• Pathophysiology (cont ’ d) − Neoplastic cells may accelerate protein development in the bloodstream.

− − Patients may have weakness in the bones.

Chemotherapy and other anticancer treatment may be given.

Multiple Myeloma

• Assessment − Findings depend on stage − Early stage: • • Fatigue Mild pain − Later stage: • • • • Hemorrhage Weight loss Bone fractures Pain

Multiple Myeloma

• Management: − − − IV therapy Pain management Supportive care

Transfusion Reactions

• Pathophysiology − − Similar to anaphylactic reaction Monitor a patient receiving a blood transfusion very closely for the first 30 –60 minutes − Occurs when a patient receives a blood type different than their own

Transfusion Reactions

• Assessment − Symptoms can be subtle in patients who are unresponsive or intubated.

− An acute reaction may include a rapid onset of: • • Chills Back pain • Hypotension

Transfusion Reactions

• Assessment (cont ’ d) − • • • • Complications include: • • Hemolytic Febrile Allergic Transfusion-related lung injury Circulatory overload Bacterial infection

Transfusion Reactions

• Management − − − Immediately stop the transfusion.

Provide hemodynamic supportive care.

Maximize kidney perfusion.

Transfusion Reactions

• Management (cont ’ d) − A hemodynamically unstable patient requires: • • Early invasive monitoring Vasopressors • Promotion of diuresis − Administer high-flow oxygen.

Summary

• Most EMS systems rarely respond to hematologic emergencies.

• Blood performs respiratory, nutritional, excretory, regulatory, and defensive functions. • Blood is made up of plasma and formed elements, including RBCs, WBCs, and platelets.

Summary

• Common lab blood tests are RBC count, hemoglobin level, and hematocrit measurement. • Blood tests measuring subtypes of WBCs can provide valuable information about the status of the immune system. • The ABO system is commonly used to classify blood types.

Summary

• During the primary assessment, note any signs and symptoms that may be immediately life threatening. • While taking a history and during the secondary assessment, look for changes in the level of consciousness.

• General blood disorder management should include oxygen, fluids, ECG, transport, medications, and psychological support.

Summary

• Hematologic disorders include sickle cell crisis, anemia, leucopenia, thrombocytopenia, leukemia, lymphomas, polycythemia, DIC, hemophilia, multiple myeloma, and complications of blood transfusions.

Summary

• A patient experiencing a sickle cell crisis will experience significant pain. • A patient with anemia has a low hemoglobin or RBC level. • Leukopenia is a reduction in the number of WBCs. Thrombocytopenia is a reduction in the number of platelets. • Leukemia is a cancer that affects the production of WBCs.

Summary

• Lymphomas are a group of malignant disorders that occur within the lymphoid system. The two types are non-Hodgkin (most common) and Hodgkin lymphoma. • Polycythemia is an overabundance or overproduction of RBCs. • Disseminated intravascular coagulation (DIC) may result from a massive injury, sepsis, or obstetric complications.

Summary

• Hemophilia is a bleeding disorder in which clotting does not happen or is insufficient. • Multiple myeloma is a cancer of the bone marrow caused by malignant plasma cells.

• Complications of blood transfusions are similar to anaphylactic reactions. They are caused by a mismatch of the patient ’ s blood type to that received, or an allergic reaction to preservatives or agents in the transfused product.

Credits

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Chapter opener

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Backgrounds

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Unless otherwise indicated, all photographs and illustrations are under copyright of Jones & Bartlett Learning, courtesy of Maryland Institute for Emergency Medical Services Systems, or have been provided by the American Academy of Orthopaedic Surgeons.