Chapter 37 Hematology

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Objectives

Describe physiology of blood components Discuss pathophysiology and signs and symptoms of specific hematological disorders Outline general assessment and management of patients with hematological disorders

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Scenario
You are called to a day camp on a sweltering summer day to care for a child who fainted. Your 6year-old patients lips and nail beds appear pale against her bronze skin. Her respirations and heart rate are rapid, and she is very anxious. She has a history of sickle cell disease and is complaining of severe abdominal pain. Counselors tell you they had just finished a strenuous game when she complained of a headache and then passed out.

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Discussion

Based on your present information, discuss the urgency of this call What complications of her disease could cause her present illness? What additional assessments do you need to perform? What are your priorities of care for this child?
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Hematological System

Blood and blood-forming organs Dysfunction affects other body systems

Results in diffuse clinical manifestations

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Blood and Blood Components

Cells Formed elements

Red blood cells (erythrocytes) White blood cells (leukocytes) Cell fragments (platelets)

Surrounded by plasma

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Blood and Blood Components

Chief functions

Delivery of substances needed for cellular metabolism in tissues Defense against invading microorganisms and injury Acid-base balance

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Blood and Blood Components

Blood cells formed in red bone marrow

AdultsRed marrow in membranous bone Vertebrae Pelvis Sternum Ribs Yellow marrow produces some white cells Composed mainly of connective tissue and fat

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Other Blood-Forming Organs

Lymph nodes

Produce lymphocytes and antibodies

Spleen

Produces lymphocytes, plasma cells, antibodies Stores large quantities of blood

Liver

Blood-forming organ during intrauterine life Important role in coagulation process

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Plasma

92% water Three proteins

Albumin Most plentiful Gives blood gummy texture Keeps water concentration of blood low so that water
diffuses from tissues into blood

GlobulinsTransport proteins Provide immunity to disease FibrinogenBlood clotting

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Plasma

Functions of plasma proteins

Maintain blood pH Transport fat-soluble vitamins, hormones, and carbohydrates Allow body to digest temporarily for food

Also contains salts, metals, and inorganic compounds

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Red Blood Cells (RBCs)

Most abundant cells in the body Responsible for tissue oxygenation

Mainly water and hemoglobin

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Red Blood Cells (RBCs)

Life span of about 120 days

As cells age: Internal chemical machinery weakens Lose elasticity Become trapped in small blood vessels in bone marrow,
liver, and spleen

Destroyed by specialized white blood cells Macrophages

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Mature Erythrocytes

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Red Blood Cells (RBCs)

Hemoglobin (Hgb) normal levels

Men: 13.5-18 g/100 mL Women: 12-16 g/100 mL

RBCs: 4.5-5 million cells/mm3

Hematocrit (Hct)

Fraction of total volume of blood that is RBCs Normally about 45%

Reticulocyte count

Information about rate of RBC production

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Hemoglobin Molecule

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White Blood Cells (WBCs)

Arise from bone marrow Functions

Destroy foreign substances Clear bloodstream of debris

Leukocyte production increases when infection occurs

Elevated WBC count in blood

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White Blood Cells (WBCs)

Normal WBC count

5000-10,000 cells/mm3 Monocytes: 5% Increase with chronic infections Lymphocytes: 27.5% Neutrophils: 65% Eosinophils and basophils together: 2.5%

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White Blood Cells (WBCs)

Leukocyte disorders

Leukemia Increased WBCs in tissues and/or blood Leukocytosis Abnormal increase in circulating WBCs Leukopenia Abnormal decrease in WBCs

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The Inflammatory Process

Redness, heat, swelling, pain Injured cells release histamine and other substances:

Cause blood vessels in injured tissue to dilate Increased blood flow carries neutrophils and monocytes (phagocytic cells) to site of injury

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The Inflammatory Process

Histamine and other substances cause walls of blood vessels to leak

Fluid that collects in tissues contains fibrinogen

Fluid and irritating chemicals accumulate at injured site

Stimulate pain receptors

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Immunity

Cellular immunity Humoral immunity

Autoimmune diseases
Alterations in immunologic response

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Platelets (Thrombocytes)

Small, sticky cells Role in blood clotting Blood vessel is cut:

Platelets to site, swell into irregular shapes Adhere to damaged vessel wall Platelets plug leak; cells stick to them, forming clot If vessel damage is too great, platelets signal clotting cascade

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Platelets

Clotting time normally 7-10 min Prothrombin time (PT time)

Time it takes plasma to clot

International normalized ratio (INR)

Assesses clotting efficiency

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Blood Groups

Mixed with foreign plasma, red blood cells either clump together or do not Four types of human blood

A, B, AB, and O Type A blood has anti-B antibodies Will clump type B blood Type B blood has anti-A antibodies Will clump type A blood

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Blood Groups

Type AB blood has neither antibody

Universal recipient

Type O blood has both anti-A and anti-B antibodies

Can only receive type O

Type O blood has neither antigen

Can be given to any blood type Universal donor

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Rh Factor

Presence or absence of Rh antigen on surface of red blood cells

Person with the factor is Rh positive Person without it is Rh negative

Antibodies to Rh factor acquired through exposure to Rh-positive blood

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Hemostasis

Stops bleeding after injury

Interaction of plasma and tissue factors with platelets and vessels

Leaky vessels sealed within minutes Vascular components Coagulation mechanisms

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Anemia

Hemoglobin or erythrocytes below normal Causes

Chronic or acute blood loss Decreased production of erythrocytes Increased destruction of erythrocytes

Iron-deficiency anemia Hemolytic anemia

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Iron-Deficiency Anemia

Iron critical to bind oxygen

Lack of iron inhibits hemoglobin production in bone marrow Cells small and pale centered Reduced oxygen-carrying capacity

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Iron-Deficiency Anemia

Causes

Diet low in iron Vitamin deficiency NSAID or ASA therapy GI disorders Bleeding (internal or external) Gastrectomy

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Hemolytic Anemia

Premature red cell destruction

Hemolysis

Inherited disorder inside red cell

Abnormal rigidity of cell membrane

Acquired disorder

Mechanical forces disrupt RBCs Autoimmune disorders Microorganisms

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AnemiaSigns and Symptoms

Fatigue and headaches Sore mouth or tongue Brittle nails Severe - breathlessness and chest pain Fever Skin or mucous membrane bleeding
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Anemia

Treatment to correct, modify, or diminish process leading to:

Defective red cell production Reduced red cell survival

Diagnosis

History Laboratory tests

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Leukemia

Several types of cancer Disorganized proliferation of WBCs in bone marrow Impairs normal RBC production

Acute or chronic
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LeukemiaSigns and Symptoms

Fatigue Bone pain Elevated temperature Diaphoresis Heat intolerance Abdominal fullness Bleeding

Bruising Headache Weight loss Night sweats Enlarged lymph nodes Enlarged spleen, testes, liver

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Leukemia

Diagnosed by bone marrow biopsy Treatment

Transfusion Antibiotics Anticancer drugs Radiation Bone marrow transplant

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Lymphomas

Group of diseases Slowly growing chronic disorders to rapidly evolving, acute conditions Hodgkins disease

Others called non-Hodgkins lymphomas

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Hodgkins Disease

Malignant disorder of lymphoid tissue

Mainly in lymph nodes and spleen

Enlargement of lymph nodes

Cancer cells multiply Displace healthy lymphocytes Suppress immune system

Sign and symptoms Diagnosis Treatment

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Hodgkins Disease

Swollen nodes in neck, armpits, groin

Fatigue
Chills Night sweats Itching, cough, weight loss Shortness of breath, chest pain Peak incidence in 20s

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Hodgkins Disease

Confirmed by finding Reed-Sternberg cells Treatment varies

Radiation Chemotherapy

Curable cancer

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Non-Hodgkins Lymphomas

Nature and activity of abnormal cells

Over 10 types of non-Hodgkins lymphoma Vary in severity

Signs and symptoms

Lymph node swelling Enlarged liver, spleen Fever Abdominal pain

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Non-Hodgkins Lymphomas

Cause mostly unknown

Some linked to Epstein-Barr virus, HIV

Treatment

Radiation therapy Anticancer drugs Bone marrow transplantation

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Polycythemia

Excess production of RBCs, WBCs, and platelets Response to hypoxia (secondary polycythemia) Unknown reasons (primary polycythemia)

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Polycythemia Vera

Increased RBC production Headache, dizziness Blurred vision Itching Red hands/feet; red-purple complexion Hypertension Splenomegaly

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Disseminated Intravascular Coagulopathy (DIC)

Complication of severe injury, trauma, or disease

Abnormal clotting disorder

Disrupts balance between:

Procoagulants and inhibitors Thrombus formation and lysis

Signs and symptoms Management

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DIC

Dyspnea, bleeding, hypotension Two phases

Free thrombin in blood, fibrin deposits, aggregation of platelets Hemorrhage from depletion of clotting factors

Management

Correction of underlying disorder Blood products

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Hemophilia

Inherited bleeding disorder

Hemophilia A Deficiency of factor VIII Hemophilia B Deficiency of factor IX

Bleeding may occur after minor injury or during procedures

Tooth extraction

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Hemophilia

Hemorrhage can occur anywhere

Joints, deep muscles, urinary tract, and intracranial sites most common

Controlled by infusions of factor VIII Most hemophiliacs seek emergency care for complicated problems and trauma

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Sickle Cell Disease

Sickle cell anemia

Debilitating recessive genetic illness

Affects persons of African descent

Less commonly Mediterranean origin)

Signs and symptoms Pathophysiology Management

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Sickle Cell Disease Signs and Symptoms

Weakness Aching

Bony deformities Jaundice

Chest pain and dyspnea

Severe abdominal pain

Fever
Joint pain

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Sickle Cell versus Normal Erythrocyte

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Sickle Cell Crisis

Triggered by:

Dehydration Stress Infection Trauma Exposure to temperature extremes Lack of oxygen Strenuous physical activity

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Sickle Cell Crisis

Management

Oxygen IV therapy Analgesia Transport for additional care

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Multiple Myeloma

Malignant neoplasm of bone marrow Tumor destroys bone

Results in:

Pain Fractures Hypercalcemia Skeletal deformities Kidney problems

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Multiple Myeloma

Diagnosed by:

X-ray Blood studies Biopsy

Treatment

Chemotherapy Radiation Bone marrow transplant

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Assessment and Management

Many emergency presentations Call to manage change in condition Management

Oxygen IV Analgesia Other symptomatic care Transport to hospital, where patient receives primary care if possible
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Conclusion
The paramedics knowledge of hematologic diseases enhances assessment skills and provides an understanding of treatment strategies needed for these patients.

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Questions?

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