Hematology

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Hematology

• By
• Joyce Smith RN BSN

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Introduction

• Red Blood Cell Disorders
• White Blood Cell Disorders
• Coagulation Disorders
• Clotting Factor Disorders

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RBC Destruction Sickle Cell Disease

• Hereditary, chronic form of anemia
• Abnormal sickle or crescent-shaped erythrocytes
  are present
• D/t abnormal Hemoglobin S (Hb S) in the RBCs
• High presence of disease in Mediterranean
  African population African American population
  in US

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Sickle Cell Disease

• Sickle Cell Crisis
• Exact cause of sickling crisis unknown
• 2 factors
• 1. Hypoxia caused by ? O2 tension in blood
• 2. ? blood viscosity/? plasma volume, ? hydrogen
  ion concentration, ? plasma osmolality, caused
  by concentration of cells dehydration

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Sickle Cell Crisis

• Sickle cell interferes with O2 transport
• Obstruction of capillary blood flow
• Causes fever severe pain in joints abdomen
• Crisis is precipitated by ? O2

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Diagnostic Tests

• Stained blood smear
• Sickle cell observed
• Sickle cell preparation of blood
• Sickling noted after deoxygenation
• Sickle solubility test
• Turbid solution indicates presence of Hb S

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Diagnostic Tests

• Hemoglobin electrophoresis
• Presence of Hb S Hb A indicates sickle cell
  trait lt 40 Hb S
• Presence of Hb S only indicates sickle cell
  anemia 85-95
• Erythrocyte life span
• Decreased 10 to 20 days

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Clinical Manifestations

• Hemolytic anemia
• Pallor
• Jaundice
• Fatigue
• Irritability

• Extensive Sickling
• Vaso-occlusive or thrombotic crisis
• Aplastic Crisis
• Sequestration Crisis
• Hyperhemolytic Crisis

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Health History

• Previous crisis
• Precipitating events
• Severity
• Usual treatment
• Review of activities last 24 hours
• Food fluid intake
• Exposure to temperature extremes
• Type of clothing worn
• Meds taken, ingestion of alcohol recreational
  drugs
• Exercise, trauma, stress

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Health History

• Activity toleration
• Fatigue
• Activity intolerance
• ADLs
• Sleep rest pattern
• Climbing stairs
• SOB
• Measure on Likert scale 0-10

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Assess

• Skin
• Jaundice or Pallor, ulcerations
• Skeletal structures
• Joint swelling, disproportionably long arms
  legs, skeletal fragility
• GI function
• Enlarged liver spleen

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Assess

• Comfort
• Bone, abdominal pain
• Cardiac Function
• Systolic murmurs, tachycardia, dysrthythmias,
  cardiac enlargement, hyportension, ? pulse
  pressure
• Respiratory Function
• Dyspnea, acute respiratory distress

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Assess

• Sensory Function
• Altered LOC, abnormal pupillary response,
  abnormal reflexes
• Renal Function
• Decreased UO, edema
• Sexuality
• Delayed sexual maturity

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General Management

• Supportive Care
• Rest
• O2 therapy
• IV fluids elytes
• Analgesics
• Sedatives
• Blood transfusion
• Genetic counseling

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Drug Therapy

• Experimental use of antisickling agents
• Hydroxyurea
• Erythropoientin
• Nitric Oxide
• Flurocor
• Oral administration
• Folic acid /or Fe
• Bone Marrow Transplant
• Gene Replacement Therapy

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Immunohemolytic Anemiaor Autoimmune Hemolytic
Anemia

• ? RBC hemolysis due to attack by own immune
  system
• Attacks antigen on RBC cell membrane
• Against universal component of Rh system
• Immune system is not able to recognize self as
  normally would
• Cause may be infections, drug reactions, and
  certain cancers
• Hemolytic anemia of newborn can occur with a Rh
  neg mother and Rh positive baby
• Blood transfusions can also cause a problem

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Immunohemolytic Anemiaor Autoimmune Hemolytic
Anemia

• Signs and symptoms
• Pallor
• Fatigue
• Tachycardia
• Shortness of breath
• Hypotension

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Treatment

• Glucocorticoids for mild to moderate immune
  suppression
• Cytoxin, Imuran
• Plasma exchange therapy
• Splenectomy
• Transfusion limited or no benefit

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• PERNICIOUS ANEMIA
• or
• Vitamin B 12 Deficiency

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Vitamin B12 Deficiency

• Rare chronic progressive condition
• Abnormal formation function of RBCs
• Inadequate intake or inability to absorb Vitamin
  B12
• Fatal if not treated

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A P

• Can be results of atrophied parietal cells in the
  stomach
• Absence of intrinsic factor (IF), preventing
  absorption of B12
• IF secreted by parietal cells gastric mucosa
• B12 absorbed in distal ileum
• Digested B12 attached to IF in stomach, able to
  pass into blood stream in ileum

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A P

• If not attached to IF, B12 passed through the
  body through the stool
• Insidious onset, several months for symptoms to
  manifest

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Symptoms

• Affect 3 major body systems
• Hematopoietic system
• Fatigue
• Dizziness
• Ringing in ears
• Pale or yellowish skin
• HR, enlarged heart with murmur
• Chest pain

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Symptoms

• GI system
• Sore, beefy red tongue
• Loss of appetite, Weight loss
• Diarrhea abd cramping

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Symptoms

• Nervous system
• Numbness, tingling, burning in arms, legs
  feet
• Muscle weakness
• Difficulty loss of balance while walking
• Changes in reflexes
• Irritability, confusion depression

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Diagnostic Tests

• CBC abnormally large RBCs, may be abnormal
  shaped, retic ct. low
• WBC may be decreased
• Gastric analysis NG inserted gastric contents
  aspirated, acholohydria
• Gastorscopy with biopsy assess parietal cell
  atrophy R/O CA

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Diagnostic Tests

• Schilling test Patient given Injection to
  saturate liver
• Radioactive B12 orally
• 24 hour urine collection
• Impaired absorption radioactive B12 will not be
  excreted in urine

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Treatment

• Prognosis good if treatment started
• Up to 18 month for improvement
• Dietary replacement if absorption normal
• Red meat, liver, fish, poultry, shellfish, milk,
  dairy products, eggs, some fortified foods

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Treatment

• If absorption abnormal Cynocobalamine IM 200ug/d
  X 2 weeks, Biweekly for 2 weeks, monthly for
  life
• Stop smoking, maintain ideal weight, teaching for
  med. Action, side effects, daily skin care, mouth
  foot care, assess for breakdown, alt. Rest
  activity, promote safe environment, avoid heat
  pad etc.

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Folic Acid Deficiency

• Vitamin B complex
• Seen in alcoholism, malabsorption syndromes,
  pregnancy
• Most prevalent in infants, adolescents, pregnant
  lactating females, alcoholics elderly
• Increase incidence of drugs use during pregnancy

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Food Sources

• Found in asparagus spears, beef liver, broccoli,
  collards, mushrooms, oatmeal, peanut butter, red
  beans, wheat germ

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Clinical Manifestations

• Develop slowly over a period of months
• Symptoms related to tissue hypoxia
• Glossitis
• Jaundice
• Spleenomegaly

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Treatment

• Administer folic acid every day until deficiency
  is corrected
• High dises to patients with malabsorption
  problems
• Folvite adults 250 to 1,000 mcg/d until
  hematological responses increases
• Maintainance 400 mcg/day X 2