HEMATOLOGY

 The hematologic or hematopoietic system includes the blood, blood vessels, and blood forming organs (bone marrow,
spleen, liver, lymph nodes, and thymus gland).

Major function of blood

a. Carry necessary materials (oxygen, nutrients) to cells and to remove CO2 system and metabolic waste products.
b. It also plays a role in hormone transport, inflammatory and immune responses, temperature regulation, fluid-electrolyte
balance, and acid-base balance.
DISORDERS OF THE HEMATOLOGIC SYSTEM
1. IRON DEFICIENCY ANEMIA:
a. Iron stores are depleted, resulting in a decreased supply of iron for the manufacture of hemoglobin in RBC’s
b. Commonly results from blood loss, increased metabolic demands, syndromes of gastrointestinal malabsorption, and dietary
inadequacy

ASSESSMENT
a. Mild cases usually asymptomatic.
b. Pallor, irritability
c. Weakness and fatigue may also have dyspnea
d. Palpitations, tachycardia, headache, dizziness, and cold sensitivity.
e. Smooth sore tongue, cheilosis, koilonychias, pica
Lab. findings:
a. Serum iron is decreased
b. RBC’s small (microcytic) and (hypochromic).
c. Hgb markedly decreased
INTERVENTIONS
a. Determine possible sources of bleeding
b. Increase iron intake or administered as prescribed
oral iron preparations: route of choice.
  Give after meals or snacks to prevent gastrointestinal side effects
 (best to be taken on an empty stomach to enhance its effect)
 Dilute liquid preparations well and administer using a straw to prevent staining of teeth.
 If possible administer with orange juice as vitamin C to enhance its absorption.
 Inform client that iron preparations will change stool color and consistency (dark and tarry) and may cause constipation.
2. PERNICIOUS ANEMIA
a. Chronic progressive, macrocytic anemia caused by a deficiency of intrinsic factor; the result is abnormally large erythrocytes
and hypochlorhydria (a deficiency of hydrochloric acid in gastric secretions).
b. Lack of intrinsic factor is caused by gastric mucosal atrophy (possibly due to heredity, prolonged iron deficiency, or an
autoimmune disorder), can also result in client who have had a total gastrectomy if vitamin B12 not administered.
c. Usually occurs in men and women over age 50, with an increase in blue eyed persons.

ASSESSMENT
a. Anemia, weakness, pallor, dyspnea, palpitations, fatigue.
b. GI symptoms: sore mouth, smooth, beefy, red tongue, weight loss, constipation or diarrhea, jaundice.
c. CNS symptoms; tingling, paresthesia of hands and feet, paralysis, depression, psychosis.
Lab. tests:
a. Erythrocyte count decreased.
b. Gastric analysis: decreased free hydrochloric acid.
c. Positive schilling test.
 Measures absorption of radioactive vitamin B12 both before and after parenteral administration of intrinsic factor.
 Definitive test for pernicious anemia.
 Used to detect lack of intrinsic factor.
 Fasting client is given radioactive vitamin B12 by mouth and nonradioactive vitamin B12 IM to saturate tissue binding
sites and to permit some excretion of radioactive vitamin B12 in the urine if it is absorbed.
 24-48 hour urine collection is obtained; client is encouraged to drink fluids.
 If indicated, a second stage Schilling test will be performed 1 week after first stage. Fasting client is given radioactive
vitamin B12 combined with human intrinsic factor and the test will be repeated.
INTERVENTIONS
a. Provide a nutritious diet high in iron, protein, and vitamins (fish, meat, milk/milk products, and eggs).
b. Avoid highly seasoned, coarse or very hot foods if client has a mouth sores.
c. Provide mouth care before and after meals using a soft toothbrush and non-irritating rinses.
d. Patient teaching (importance of life long vitamin B12 therapy). Once a month IM injection.

3. SICKLE-CELL ANEMIA
 Most common inherited disease among black Americans.
 HgbS (abnormal hemoglobin), which has reduced oxygen carrying capacity, replaces all or part of the hemoglobin in the
RBC’s.
 Life span is 6-20 days instead of 120, causing hemolytic anemia.
 Death often occurs in early adulthood due to occlusion or infection.
ASSESSMENT
VASO-OCCLUSIVE CRISIS:
 Most common type of crisis; caused by stasis of blood with clumping of the cells in the microcirculation, ischemia, and
infarction.
 Signs include fever, pain, and tissue engorgement
SPLENIC SEQUESTRATION:
 A life threatening crisis caused by the pooling of blood in the spleen.
 Signs include profound anemia, hypovolemia, and shock
APLASTIC CRISIS:
 Caused by the diminished production and increased destruction of RBC’s, triggered by viral infection or the depletion of folic
acid.
 Signs includes: profound anemia and pallor
 Frequent infection esp. with H. influenzae
 Infants may have Dactylitis (hand –foot syndrome) symmetrical painful soft tissue swelling in the hands and feet in the
absence of trauma.
ASSESSMENT
 First sign in infancy may be “colic” due to abdominal pain (abdominal infarct).
 Splenomegaly: initially due to hemolysis and phagocytosis; later due to fibrosis from repeated infarct to spleen.
 Weak bones or spinal defects due to hyperplasia of marrow and osteoporosis.
 Leg ulcers, especially in adolescents, due to blockage of blood supply to skin of legs.
MEDICAL MANAGEMENT
1. Drug therapy:
a. Analgesics: acetaminophen, meperidine, morphine (avoid aspirin as it enhances acidosis, which promotes sickling).
b. Avoid anticoagulants (sludging is not due to clotting)
c. Antibiotics.
2. Blood transfusions
3. Hydration: oral and IV
4. Bed rest
5. Surgery: Splenectomy
6. Administer oxygen and blood transfusion as prescribed to increase tissue perfusion.
7. Maintain adequate hydration
8. Avoid tight clothing that could impair circulation.
9. Keep wounds clean and dry.
10. Provide bed rest to decrease energy expenditure and oxygen use. Avoid activities that require so much energy.
11. Encourage patient to eat foods high in calories, CHON, with folic acid supplementation.
12. Keep arms and legs from becoming cold.
13. Decrease emotional stress.
14. Provide good skin care, especially to legs.
4. APLASTIC ANEMIA
a. A deficiency of circulating erythrocytes resulting from the arrested development of RBC’s within the bone marrow.
b. Possible causes includes: chronic exposure to myelotoxic agents, viruses, infection, autoimmune disorders, and allergic
states.
 c. Definitive diagnosis: Bone marrow aspiration (demonstrates conversion of red bone marrow to fatty red bone marrow ).

ASSESSMENT
a. Pancytopenia (a deficiency of erythrocytes, leukocytes, and thromboctyes).
b. Petechiae, purpura, bleeding, pallor, weakness, tachycardia, and fatigue.
MANAGEMENT
a. Blood transfusion, splenectomy, corticosteroids, immunosuppressive therapy.
b. Bone marrow transplant (treatment of choice if suitable donor exists).
c. Administration of antilymphoctye globulin (ALG) or antithymoctye globulin (ATG) to suppress the autoimmune response.
IMPLEMENTATIONS
a. Administer blood transfusions as prescribed.
b. Transfusion should be discontinued as soon as the bone marrow begins to produce RBC’s.
c. Administer corticosteroids and immunosuppressive therapy as prescribed.
5. HEMOLYTIC ANEMIA
a. May be congenital or acquired.
b. Increase rate in RBC’s destruction
c. Cause often unknown, but erythrocyte life span is shortened and hemolysis occurs at rate that the bone marrow can’t
compensate for.
d. Diagnosis is based on lab evidence of an increased rate of erythrocyte destruction and a corresponding production.
Compensatory effort by bone marrow to increase in size.

ASSESSMENT
a. Pallor, jaundice.
 b. Chills, fever, irritability, and pain.
c. Abdominal pain and nausea, vomiting, diarrhea, melena.
d. Hematuria
e. Dyspnea.
f. Hepatomegaly.
g. Laboratory tests.
 A. Hgb and Hct decreased.
 B. Reticulocyte count elevated
MEDICAL MANAGEMENT
a. Identify and eliminate (if possible) causative factors.
b. Drug therapy.
c. Corticosteroids in autoimmune types of anemia.
d. Folic acid supplements.
e. Blood transfusion therapy.
INTERVENTIONS
 Monitor for signs and symptoms of hypoxia including confusion, cyanosis, shortness of breath, tachycardia, and palpitations.
 Assess for the presence of jaundice may make assessment of skin color in hypoxia unreliable.
 If jaundice and associated pruritus are present, avoid soap during bathing and use cool or tepid water.
 Frequent turning and meticulous skin care are important as skin friability is increased.
6. THALASSEMIA MAJOR (Cooley’s anemia)
 B- thalassemia refers to an inherited hemolytic anemia, characterized by reduction or absence of the B- globulin chain in hgb
synthesis
 Fragile RBC=short life span
 Autosomal recessive pattern of inheritance
 Insufficient B-globulin chain synthesis allows large amounts of unstable chains to accumulate
 Precipitates of alpha chains that form cause RBCs to be rigid & easily destroyed, leading to severe hemolytic anemia= chronic
hypoxia
 Skeletal deformities: frontal & maxillary bossing, pathologic fracture
 Hemosiderosis (a term used to describe an overload of iron in your organs or tissues .)

CLINICAL MANIFESTATIONS
 Onset is usually insidious
 Signs and symptoms are primarily r/t progressive anemia, expansion of marrow cavities of the bone & development of
hemosiderosis
 Early Symptoms often include progressive pallor, poor feeding & lethargy
 Further signs: hemorrhage, bone pain, exercise intolerance, jaundice, & protuberant abdomen
DIAGNOSTIC EVALUATION
 Decrease hemoglobin
 RBC= increase in number
 Hgb elctrophoresis= elevated levels of HgF & HgA2, limited amount of HgA

MYELOPROLIFERARIVE Disorder
Polycythemia Vera
a. Hyperplasia of all bone marrow elements
  increase RBC mass
 increase blood volume viscosity
 decrease marrow iron reserve
b. splenomegally

ASSESSMENT
a. Reddish purple hue of skin & mucosa, pruritus
b. Splenomegaly, hepatomegaly
c. Epigastric discomfort, abdominal discomfort
d. Painful fingers & toes from, paresthesias
e. H/A altered mentation
f. Weakness, fatigue, night sweats, bleeding tendency
g. Hyperuricemia
DX TESTS
a. CBC
b. BONE MARROW ASPIRATION
MANAGEMENT
1. HYPERVISCOSITY
 Phlebotomy @ intervals determined by CBC results to decrease RBC mass
 Generally 250-500ml removal @ a time
2. HYPERPLASIA
a. Myelosuppressive therapy,
b. Generally using hydroxyurea or IV radioactive phosphorus (32P), biologic response modifier, ie alpha interferon
3. HYPERURICEMIA- allopurinol
4. PRURITUS - antihistamines (cimitidine), low dose acetyl salicylic acid; certain anti-depressants (doxepin), phototherapy,
cholestyramine
INTERVENTION
a. Encourage/assist ambulation
b. Assess for early sign and symptoms of thromboembolic complications
c. Monitor CBC & assist with phlebotomy as ordered
d. Patient Education
e. Educate about risk of thrombosis; encourage patient to maintain normal activity pattern & avoid long periods of rest
f. Avoid hot showers
g. Report @ regular intervals for follow up blood
DISORDERS OF PLATELETS AND CLOTTING MECHANISM
HEMOPHILIA
a. Hereditary coagulation defect usually transmitted to affected male by female carrier through sex–linked recessive gene,
resulting in prolonged clotting time.
b. Most common type is hemophilia A- factor VIII deficiency.
c. Male inherits hemophilia from their mothers, and females inherit the carrier status from their fathers.
ASSESSMENT
a. Abnormal bleeding in response to trauma or surgery.
b. Joint bleeding causing pain, tenderness, swelling, and limited range of motion.
c. Tendency to bruise easily.
d. Prolonged PTT
e. Normal bleeding time, prothrombin time (PT) and platelet count.
IMPLEMENTATION
a. Administer factor VIII concentrate.
b. Monitor for bleeding and maintain bleeding precautions.
c. Monitor for joint pain; immobilize the affected extremity if joint pains occur.
d. Monitor urine for hematuria.
e. Instruct the parents regarding activities for the child, emphasizing the avoidance of contact sports.
f. Instruct the parents on how to control bleeding.
VON WILLEBRAND’S DISEASE
a. Inherited (autosomal dominant) or acquired bleeding disorder
b. Low level of von willebrand factor & prolonged bleeding time

ASSESSMENT
a. Mucosal & cutaneous bleeding
b. Prolonged bleeding
Diagnostic Evaluations
a. Bleeding time-prolonged
b. Von willebrand’s factor-decreased
c. Factor VIII- generally decreased
MANAGEMENT
 a. Replacement factor VIII via infusions of cryoprecipitate
b. Antifibrinolytic medication (AMICAR) to stabilize clot formation
c. Desmopressin (DDAP)= to manage mild moderate bleeding
INTERVENTIONS
a. Institute bleeding precautions
b. Monitor pad count amount of saturation during menses
c. Administer blood products as ordered
d. Patient Education
 Teach patient bleeding precaution
 Demonstrate the use of direct, steady pressure @ bleeding site if bleeding develops
DISSEMINATED INTRAVASCULAR COAGULATION (DIC)
 Grave coagulopathy resulting from overstimulation of clotting & anticlotting processes in response to disease/ injury
 Generalized intravascular clotting which in turn overstimulates fibrinolytic mechanisms
↓
hypercoagulability
↓
Hypocagullability

↓
hemorrhage

ASSESSMENT
a. Purpura on lower extremities & abdomen
b. Hemorrhagic bullae, acral cyanosis, focal gangrene in skin
Diagnostic Tests:
a. Marked decrease of blood platelets
b. Low levels of fibrinogen & other clotting factors
c. Prolonged prothrombin & partial thromboplastin times & abnormal erythrocyte morphologic characteristics
INTERVENTIONS
a. Treatment of primary disorder is essential in mgt. of DIC
b. Heparin (controversial)
c. Transfusions of whole blood, plasma, platelets, cryoprecipitate & other blood products are administered to replace depleted
factors
d. Environment that protects from trauma & bleeding
e. Side rails of bed are padded

IDIOPATHIC THROMBOCYTOPENIA PURPURA

a. Increased destruction of platelet
b. Exact cause is unknown
 c. Spleen is the site of destruction of platelets

ASSESSMENT
a. PETECHIAE: spider-web appearance of bleeding under skin d/t small size platelets
b. Echymosis
c. Blood in any secretions, bleeding from mucous membranes, nosebleeds
DX TESTS:
a. Platelet count decrease
b. Anemia
INTERVENTIONS
a. Control bleeding, prevent bruising
b. Provide support to client & be sensitive change in body image
c. Protect from infection
d. Measure normal circumference of extremities for baseline
e. Administer meds orally, rectally, or I.V. rather than I.M.
f. Avoid aspirin
MULTIPLE MYELOMA
a. Unknown etiology
b. Environmental factors = chronic exposure to low levels of ionizing radiation
c. Proliferation of neoplastic plasma cells derived from B-lymphocyte ( clone) & producing homogenous immunoglobulin ( M
protein or bence jones protein without apparent antigenic stimulation
d. Plasma cells produce (O-A-F) extensive bone loss
e. Abnormal immunoglobulin affects renal function, platelet function, resistance to infection, & may cause hyperviscosity of
blood
f. Generally affects older people & is more common among black men & women

ASSESSEMENT
 a. Constant, often severe bone pain caused by bone lesions & pathologic fractures
b. Fatigue & weakness r/t anemia caused by crowding of marow by plasma cells
c. Proteinuria & renal insufficiency
d. Electrolyte disturbances, including hypercalcemia (bone destruction), hyperuricemia (cell death), renal insufficiency.
INTERVENTIONS
a. Chemotherapy including oral melphalan ( Alkeran) or cyclophosphamide ( cytoxan), corticosteroids alone or in combination
with chemotherapy
b. Alpha interferon as maintenance therapy
c. Plasmapheresis
d. Radiation therapy for bone lesions
e. Supportive care: allopurinol & fluids
a. hemodialysis
b. surgical stabilization & fixation of fractures
f. Bone marrow or peripheral blood stem cell transplant in selected cases