Chapter 24 Hematologic Emergencies. National EMS Education Standard Competencies Medicine Integrates...

Chapter 24Chapter 24

Hematologic Emergencies

National EMS Education Standard CompetenciesNational 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 CompetenciesNational EMS Education Standard Competencies

Hematology

•Anatomy, physiology, pathophysiology, assessment, and management of:

− Sickle cell crisis

− Clotting disorders

National EMS Education Standard CompetenciesNational 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 CompetenciesNational 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

IntroductionIntroduction

• EMS systems rarely respond to hematologic emergencies.

• Hematologic disorder: any disorder of the blood− Hemolytic disorders

− Hemostatic disorders

IntroductionIntroduction

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

blood components

Blood and PlasmaBlood and Plasma

• Blood performs the following functions:

Blood and PlasmaBlood and Plasma

• Blood is made of:

Blood and PlasmaBlood 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 PlasmaBlood and Plasma

• Three common lab tests:− RBC count

− Hemoglobin level

− Hematocrit measurement

Blood and PlasmaBlood 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

Blood-Forming Organs and RBC Production

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

− Liver

− Spleen

The Inflammatory ProcessThe 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 SystemThe Immune System

• Processes protect against pathogens and other unwanted materials.− Must distinguish between:

• Body’s own tissue

• Outside organs

The Immune SystemThe 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 SystemThe Immune System

• Humoral immunity− Secretion of

immunoglobins• Recognizes a

specific antigen

• Cell-mediated immunity− Macrophages and

T-cells destroy pathogens.

The Immune SystemThe 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 SystemThe Immune System

Blood ClassificationsBlood Classifications

• Developed to prevent medical problems during replacement − ABO system

Blood ClassificationsBlood Classifications

• Blood contains a secondary antigen called the Rh antigen.− Possible complications in pregnancy

• Hemolytic disease

HemostasisHemostasis

• The process of stopping bleeding through:− Vasoconstriction (immediate response)

− Platelet plugging

− Coagulation• Clots are made up of fibrin.

HemostasisHemostasis

• Clotting cascade− Can be initiated through:

• Intrinsic pathway

• Extrinsic pathway

− Coagulopathy: process that interferes with the clotting cascade or hemostasis

Patient AssessmentPatient Assessment

• Note any life-threatening signs or symptoms.− Ask about patient’s

history and SAMPLE history.

− A nonjudgmental approach is essential.

Scene Size-UpScene Size-Up

• Ensure the scene is safe for entry.

• Consider the mechanism of injury.

• Determine the number of patients.

• Assess for hazards.

Primary AssessmentPrimary Assessment

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

Primary AssessmentPrimary 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 AssessmentPrimary Assessment

• Circulation− Manage life-threatening conditions.

− If hemophilia is suspected, watch for:• Acute blood loss

• Bleeding of unknown origin

• Hypoxia or shock

Primary AssessmentPrimary Assessment

• Transport decision− Depends on severity and patient’s wishes

− Transport to the closest facility if:• Sickle cell crisis

• Uncontrolled bleeding

History TakingHistory 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 TakingHistory Taking

History TakingHistory 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 TakingHistory 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 AssessmentSecondary Assessment

• Performed on scene, en route, or not at all

• Perform a physical exam, focusing on major joints.

• Obtain vital signs.

Secondary AssessmentSecondary 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.

ReassessmentReassessment

• Reassess frequently.

• Administer supplemental oxygen.

• Provide ventilation as appropriate.

• Cover to maintain body temperature.

ReassessmentReassessment

• According to local protocol, administer:− IV fluid

− Nitrous oxide

• Prehospital care for hemophilia can include IV therapy.

ReassessmentReassessment

• 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

• Emergency medical care should include: − Oxygen

− Fluids

− ECG

− Comfort

− Pharmacology

− Support

Sickle Cell CrisisSickle 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 CrisisSickle Cell Crisis

• Pathophysiology (cont’d)− Can lead to:

• Aplastic crisis: RBC production temporarily stops.

• Hemolytic crisis: Acute RBC destruction

Sickle Cell CrisisSickle Cell Crisis

• Pathophysiology (cont’d)− May manifest as:

• Vaso-occlusive crisis

• Acute chest syndrome

• Splenic sequestration crisis

Sickle Cell CrisisSickle Cell Crisis

• Assessment− Characterizations may include:

• Shortness of breath

• Inadequate perfusion of the skin

• Jaundice

• Mild dehydration

Sickle Cell CrisisSickle 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 CrisisSickle Cell Crisis

• Management− Administer high levels of oxygen.

− Rapidly transport patient.

− Consider IV fluid therapy.

− Maintain patient’s body temperature.

− Recommend that patient rest.

AnemiaAnemia

• Pathophysiology− Low hemoglobin or

erythrocyte level

− Usually associated with an underlying disease

AnemiaAnemia

• Iron deficiency anemia− Most common type

− Causes include:• Gastrointestinal blood loss

• Menstrual bleeding

• Frequent donations or diagnostic test

AnemiaAnemia

• Pathophysiology (cont’d)− May be caused by an inherited hemolytic

disorder or a hematologic disorder

− Can have serious consequences in high altitudes

AnemiaAnemia

AnemiaAnemia

• 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.

AnemiaAnemia

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

− Consider rapid transport for:• Abrupt change in consciousness

• Hypotension

• Significant perfusion inadequacies

LeukemiaLeukemia

• Pathophysiology− Cancer in the

lymphoid system

− Blood cells develop abnormally and/or excessively, causing:• Anemia

• Thrombocytopenia

• Leukocytosis

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LeukemiaLeukemia

• 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

LeukemiaLeukemia

• Pathophysiology (cont’d)− Survival depends on several factors:

• Stage of detection

• Underlying medical condition

• Response to treatment

− Treated with chemotherapy and radiation

LeukemiaLeukemia

LeukemiaLeukemia

• Management− As appropriate, provide:

• Airway support

• Oxygen therapy

• IV fluid therapy

• Analgesics

• Positive support

LeukemiaLeukemia

• 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.

LymphomasLymphomas

• Pathophysiology− Group of malignant diseases within the

lymphoid system

− Classified in two categories

LymphomasLymphomas

• 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

LymphomasLymphomas

• Assessment− Require chemotherapy or radiation.

− Signs and symptoms may include:• Pallor

• Feeling hot and then cold or both

• Inadequate perfusion

• Abnormal ECG rhythms

LymphomasLymphomas

• Management− Aggressive pain

management

− Treat inadequate perfusion and abnormal heart rhythms.

− Initiate rapid transport if condition does not improve.

PolycythemiaPolycythemia

• Pathophysiology− Overabundance or overproduction of RBCs

− Multiple causes

− Can lead to other conditions

− Treatment usually includes phlebotomy or cancer-type therapy

PolycythemiaPolycythemia

• Assessment− Findings may widely vary

− Note the extent and duration of dyspnea.

− Has the patient experienced pruritus or changes in skin temperature?

PolycythemiaPolycythemia

• Management− Consists of supportive care and transport to the

appropriate facility.

− Administer oxygen and establish IV as needed.

Disseminated Intravascular Coagulation

Disseminated Intravascular Coagulation

• Pathophysiology− Number of causes

− Two stages:• Defibrination

• Uncontrolled hemorrhage

− High mortality rate

Disseminated Intravascular Coagulation

Disseminated Intravascular Coagulation

• Assessment− Identify associated signs and symptoms.

− Patients may have:• Respiratory difficulty

• Signs of shock

• Skin changes

Disseminated Intravascular Coagulation

Disseminated Intravascular Coagulation

• Management− Identify underlying cause and establish

treatment early.

− Maintain an airway.

− Treat for shock.

HemophiliaHemophilia

• Pathophysiology− A bleeding disorder in which clotting:

• Does not occur

• Occurs insufficiently

− Two primary types: A and B

HemophiliaHemophilia

• Pathophysiology (cont’d)− Signs and symptoms may include:

• Acute and chronic bleeding

− Patients may require:• Hospitalization for transfusion

• Infusion of factors VIII and IX

HemophiliaHemophilia

• 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.

HemophiliaHemophilia

• Management− Patients may need high-flow oxygen.

− Note ECG findings.

− IV therapy may be necessary.

− Analgesics may be appropriate.

Multiple MyelomaMultiple 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 MyelomaMultiple 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 MyelomaMultiple Myeloma

Multiple MyelomaMultiple Myeloma

• Management:− IV therapy

− Pain management

− Supportive care

Transfusion ReactionsTransfusion 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 ReactionsTransfusion 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 ReactionsTransfusion Reactions

• Assessment (cont’d)− Complications include:

• Hemolytic

• Febrile

• Allergic

• Transfusion-related lung injury

• Circulatory overload

• Bacterial infection

Transfusion ReactionsTransfusion Reactions

• Management− Immediately stop the transfusion.

− Provide hemodynamic supportive care.

− Maximize kidney perfusion.

Transfusion ReactionsTransfusion Reactions

• Management (cont’d)− A hemodynamically unstable patient requires:

• Early invasive monitoring

• Vasopressors

• Promotion of diuresis

− Administer high-flow oxygen.

SummarySummary

• 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.

SummarySummary

• 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.

SummarySummary

• 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.

SummarySummary

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

SummarySummary

• 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.

SummarySummary

• 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.

SummarySummary

• 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.

CreditsCredits

• Chapter opener: © Monkey Business Images/ShutterStock, Inc.

• Backgrounds: Orange—© Keith Brofsky/Photodisc/Getty Images; Blue—Courtesy of Rhonda Beck; Lime—© Photodisc; Purple—Courtesy of Rhonda Beck.

• 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.