HEMATOLOGICAL DISORDERS

HEMATOLOGICAL DISORDERS

• The blood and the blood forming sites, including the bone marrow and the
reticuloendothelial system

• Blood

– Plasma

– Blood cell

Blood Cells

• Erythrocyte: RBC

• Leukocyte: WBC

– Neutrophil

– Eosinophil

– Basophil

– Monocyte

– Lymphocyte:

• T lymphocyte

• B lymphocyte

• Thrombocyte: platelet

ANEMIA

• - Decrease availability of oxygen to tissues due to insufficient RBC

Etiology:

 Decrease production of healthy RBC

 Increased RBC destruction

 Loss of blood

 Inadequate dietary intake of vitamins & minerals

 Infections

 Drugs and chemicals

renzN405 1
Types of Anemia according morphologic characteristics of RBC

1. Normocytic/Normochromic

 Acute blood loss

 Hemolysis

 Chronic renal disease

 Cancer

 Diseases of endocrine dysfunction

 Aplastic anemia

 Pregnancy

2. Macrocytic/Normochromic

 Vitamin B12 deficiency

 Folic acid deficiency

 Liver disorders

 Alcoholism

 Splenectomy

3. Microcytic/hypochromic

 Fe deficiency anemia

 Thalassemia

 Lead poisoning

4. Abnormal shape of RBC

 Sickle shape anemia

 Hereditary spherocytosis

Manifestations

• Pallor

• Easy fatigability

• Weakness

• Weight loss and Anorexia

• Shortness of breath

• Headache/dizziness

• Amenorrhea

• Cold sensitivity

• Tachycardia

• Paresthesia

renzN405 2
 1. Mild anemia (hgb 10-12 g/dl)

- Usually asymptomatic

- S/sx occur after strenuous exertion

2. Moderate anemia (hgb 6-10g/dl)

- Dyspnea, palpitations, diaphoresis, chronic fatigue

3. Severe anemia (<6 g/dl)

- Significant multi-organ dysfunction

Management

• Correct or control the cause

• Provide transfusion of packed RBCs

• Treatment is specific to the type of anemia:

– Dietary therapy

– Iron or vitamin supplementation: iron, folate, B12

– BMT or PBSCT

– Immunosuppressive therapy

– Other (Surgery: splenectomy)

ANEMIA CAUSED BY DECREASED ERYTHOCYTE PRODUCTION

A. Fe Deficiency Anemia

- Chronic, hypochromic, microcytic

- Most prevalent hematologic disorder

Risk factors:

 Gender and Age

 Insufficient dietary intake

 Blood loss (e.g. menstruation)

 Impaired absorption of Fe

 Excessive demands of RBC production 2◦ to hemolysis

Clinical manifestations

• General manifestations of anemia

• Cheilosis

• Koilonychias

• Pica

• Tinnitus

• Plummer-Vinson Syndrome

renzN405 3
Diagnostics

• Peripheral blood smear

• CBC and Fe profile

 Decrease hgb (6 to 9g/dl)

 Decrease serum Fe level

 Increase total Fe-binding capacity

 Absence of hemosiderin

• Determination of source of blood loss

 sigmoidoscopy, colonoscopy

 Upper & lower GI series

 Stool & urine occult blood exam

Management

 Correction of chronic blood loss

 Diet & supplemental Fe preparations

Oral FeSO4

Preferred & least expensive

E.g. Ferrous sulfate (Feosol)

Ferrous gluconate (Fergon)

 Should be given p.c.

 Use straw for oral liquid Fe

 Take Vitamin C

 Do not administer with milk, antacid

Parenteral Fe

Administered as deep IM or IV

Imferon (IM)

 Use Z tract

 Do not massage site of injection

 Fe Dextran

 Should be infuse not >1ml/min

 Antidote: deferoxamine mesylate

renzN405 4
Indications for parenteral Fe

 Malabsorption syndrome

 Intolerance to oral iron

 Persistent blood loss

 Compliance of patient

 Rapid response is needed

 Supportive nursing care

- Promote rest

- Provide good oral and skin care

B. Folic Acid Deficiency Anemia

Megaloblastic anemia with slow and insidious onset

- Amount of folic acid is insufficient to synthesize DNA, RNA & proteins

• Serum folate level: < 4mg/ml

Etiology and risk factors:

 Inadequate dietary intake

 Chronic alcoholism

 Eating disorders

 Long term use of anticonvulsants

 Cancer & leukemic patients

 Pregnancy

 Use of certain oral contraceptives

 Malabsorption condition

Clinical manifestations:

 General s/sx of anemia

 No neurological manifestations

Diagnostics:

 BME and PBS – confirmatory test

 Schilling’s test

Management:

 Therapeutic trial:

 50-100mg of folic acid/IM daily x 10 days

 Oral folic acid replacement

 Nutritional support and proper food preparation

 Vitamin C is sometimes prescribed

renzN405 5
C. Pernicious Anemia

 Megaloblastic or macrocytic caused by failure of absorption of Vitamin B12 (cobalamin)

 Autoimmune disorder characterized by absence of intrinsic factor in gastric secretions

 Common between 40 -70 years old

 Peak incidence: 70 years old

 Prevalent in Celtic & Scandinavian ancestry

Pathophysiology

• Intrinsic factor production (by the parietal cells of the stomach)

• Vit B12 absorption

• RBC production

• DNA synthesis

• Impairment of integrity of cells

Causes:

 Insufficient dietary intake

 Certain medications (neomycin, OCP)

 Genetic disorders ( tanscobalamin II def.)

 Gastric surgery

Clinical Manifestations:

- General s/sx of anemia

- GI manifestations

- neurologic disorders

Diagnostics:

 CBC

 PBS

 Serum Fe profile

 Gastric secretion analysis - to check for the presence of free HCL

 Schilling test

 Definitive test

 Oral radioactive Vit B12 is adm.after parenteral infusion of intrinsic factor

 Collect 24-hour urine specimen

 (+) Result: excretion of Vit B12

renzN405 6
Management:

 Cobalamin Therapy

 Parenteral cobalamin – standard treatment

 Cyanocobalamin or hydroxycobalamin

 1000ug daily for 2 weeks

 Then weekly (10 weeks), then monthly tx for life

 Fe and folic acid supplements

 Monitor blood counts

D. Aplastic Anemia

- Hypoplasia of the bone marrow leading to pancytopenia

- Fat replaces bone marrow

- insidious or rapid onset

Causes:

 Hereditary (Fanconi syndrome)

 Acquired – 80%

 High dose of radiation and chemotx

 Certain drugs

 Autoimmune disorder

 Infectious agents

 Pregnancy

 Idiopathic

 Most common in adolescents and young adults

Clinical manifestations

• General s/sx of anemia

• Freq. infections

• Unexplained bruising

• Prolonged bleeding

Severe manifestations

 Pancytopenia

 Normocytic anemia

 Neutropenia

 Thrombocytopenia

renzN405 7
Diagnostic test

 Bone marrow aspiration/biopsy

Adult: post. Iliac crest – most common site

Prone position during the procedure

Child: tibia – most common site

Side-lying position during the procedure

Management:

 Withdrawal of offending agent

 Blood transfusion

- Mainstay therapy if 2 to myelotoxic agents

 Treatment of infection

 Immunosuppressive therapy (Antithymocyte Globulin- ATG)

 Bone marrow transplant

- Tx of choice for <60 years old

 Diet adjustments

 Client education

ANEMIA CAUSED BY INCREASED ERYTHROCYTE DESTRUCTION

A. Hemolytic Anemia

- Excessive or premature destruction of RBC

- RBC destruction > production

Causes:

 Hereditary (G6-PD enzyme def., sickle cell anemia, thallasemia)

 Acquired

- Exposure to toxic chemicals, drugs

- Trauma

- Infections & immune reactions

Clinical manifestations: general

Diagnostics:

1. CBC

2. Blood smear

3. RBC fragility

4. Shortened RBC lifespan

5. Fecal and urinary urobilinogen

Management:

renzN405 8
  Withdrawal of offending agent

 Transfusion therapy

 Folic acid & Fe supplement

 Splenectomy

- tx of choice for hereditary

 Erythropoietin injection

B. Sickle Cell Anemia

-autosomal recessive disorder affecting hemoglobin

Etiology & Risk factors:

 Prevalent in areas where malaria is endemic

 Children are rarely symptomatic until late 1st year (r/t increase amt of fetal hgb (HgbF)

Classification:

 Sickle cell anemia: homozygous for sickle cell gene

 Sickle cell trait:

- Heterozygous

- Milder form

- Carrier state of HbS

- 8% of African Americans are carriers

- Malaria resistant

Clinical manifestations:

1. Vasoocclusive crisis

- aka “ pain crisis”

- Most common & non-life-threatening

- Results from sickled cells obstructing blood vessels, causing occlusion, ischemia
&potential necrosis

S/sx:

Fever

Acute abdominal pain

Hand- foot syndrome (dactylitis)

Priapism

Arthralgia

renzN405 9
2. Sequestration crisis

- Results from sudden & massive trapping of destroyed RBC by visceral organs especially
spleen

- Most commonly occurs between 8 months & 5 years old

- Death is due to anemia or cardiovascular collapse

- Functional asplenia = termed for chronic manifestation

3. Aplastic crisis

4. Hyperhemolytic crisis

5. Acute chest syndrome

- Most common cause of mortality

- Char. by chest pain, fever, cough, tachypnea, pulmonary infiltrates

- Fat emboli – major etiology

6. CNS involvement

– Most prevalent in childhood & adolescence

– Stroke (thrombotic) – most severe symptom

7. Overwhelming infection

 Streptococcus pneumoniae

 Haemophilus influenza type B

8. Chronic symptoms

• Jaundice

• Gallstones

• Delayed sexual maturity

• Growth retardation

Diagnostics:

 Blood smear

 Sickle turbidity tube test

– For mass screening of HbS

 Hgb electrophoresis – diagnostic test

- Use to diff. sickle cell disease to SC trait

renzN405 10
Management:

 Prevention of sickling phenomenon

- Adequate oxygenation

- Adequate hydration

- Administration of hydroxyurea to fetal hgb

- Monthly blood transfusion

 Treatment of crisis

- Hydration/electrolyte replacement

- Antibiotic therapy

- Pain management: rest

- Blood products

 Genetic counseling

C. Polycythemia Vera

 Hyperplasia of bone marrow

 Erythrocytosis, leucocytosis,thrombocytosis

 Caused by unregulated neoplastic proliferation

 Peak incidence: 50-70 y/o with Jewish descent

Pathophysiology:

Clinical manifestations:

 Ruddy complexion

 Cardiovascular HPN (dizziness, headache)

 Fatigue

 Shortness of breath

 Increased clotting leading to stroke & MI

 Hepatosplenomegaly

 Skeletal gout

renzN405 11
Diagnostics:

 CBC

 RBC count - inc. (8-12 M)

 Hgb, hct level – inc.(18-25g/dl)

 Platelet count

 Bone marrow biopsy

 ABG

 Serum uric acid level determination

Management:

 No permanent cure

 Increase fluid intake

 Monitor s/sx of bleeding & thromboembolism

 Administer analgesic & antihistamine as ordered

 Therapeutic phlebotomy

 Chemotherapy

 Radiation therapy (Na phosphate/IV)

D. Hemochromatosis (HH)

- aka “iron overload disease”

- Inherited metabolic disorder that causes increased absorption of Fe that is deposited in

the body tissues, organs

- HH seldom manifest until adulthood

- Defective gene: HFE

Risk factors: M=F (Males dev. symptoms at younger age)

Clinical manifestations:

 Joint pain – most common

 Depression

 Bronzing of the skin

 Fatigue

 Impotence

 Arthritis

 Loss of body hair

 Liver, pancreas, heart problem

renzN405 12
Diagnostics:

- Transferring saturation test (TS)

- Serum ferritin test

- HFE mutation determination - definitive test

Management:

 Phlebotomy

 Avoid alcoholic beverages

 Limit dietary intake of food rich in Fe

E. Thalassemia Major ( Cooley’s Anemia)

- Autosomal recessive disorder

- Basic defects: Deficiency in the synthesis of beta chain polypeptides

- Mediterranean, Africa, SouthEast Asian origin

Classification:

1. Alpha Thalassemia

- Most common

- Heterozygous

- Benign & asymptomatic

2. Beta Thalassemia minor

- Mild to moderate Microcytic anemia

- Rate of production of globin molecule

- Insidious onset

3.BetaThalassemiamajor

- Homozygous

- Incompatible with life

- Severe microcytic ,hypochromic anemia

Clinical manifestations: (s/sx noted toward the end of the 1st year)

 Severe anemia

 Unexplained fever; headache

 Anorexia, poor feeding

 Enlarged abdomen; splenomegaly, hepatomegaly

 Impaired physical growth

 Listlessness; exercise intolerance

renzN405 13
  Failure to thrive

Diagnostics:

1. Amniocentesis

2. Peripheral blood smear

3. Hgb electrophoresis

Management:

 Chronic blood transfusion

 Iron chelation with deferoxamine

 Splenectomy

 Bone marrow transplantation

 Dietary intervention

- Vitamin C intake

- Increase tea consumption

 Genetic counseling

DISORDERS OF PLATELETS AND CLOTTING FACTORS

Idiopathic Thrombocytopenic Purpura

- Most common thrombocytopenic disorder

- Hemorrhagic autoimmune disease that results in platelet destruction upon reaching liver &
spleen

Clinical manifestations:

 Petechiae

 epistaxis

 Ecchymosis

 Easy bruising

 Bleeding gums

Diagnostics:

Bone marrow aspirate – megakaryocytes

Platelet count < 100,000/mm3

Prolonged bleeding time

Normal coagulation time

Increased capillary permeability

(+) platelet Ab screening

renzN405 14
Complications:

1. Spontaneous cerebral hemorrhage

2. Severe nose, GIT and urinary bleeding

3. Bleeding into the diaphragm

4. Nerve pain or paralysis

Management:

 Administer high dose corticosteroids

 Plasmapheresis - short term therapy

 Surgery

 IV gamma globulin

 Splenectomy

 Immunosuppressive therapy

(eg. Vincristine, cyclophosphamide)

COAGULATION DISORDERS

A. Hemophilia

 Defect in clotting mechanism of the blood

 Genetic disorder; X-linked recessive transmission

 Usually occurs in males

 Females – carriers

Classification:

1. Hemophilia A “classic hemophilia”

 Factor VIII (antihemophilic globulin) deficiency

 Lab Fx: prolonged coagulation time

 Normal bleeding time

2. Hemophilia B “Christmas disease”

 Factor IX def. ( plasma thromboplastin component)

 Lab fx: similar with hemophilia A

3. Von Willebrand’s disease

 Factor VIII deficiency & defective platelet dysfunction

 Most common congenital bleeding disorder

 Autosomal dominant

 Lab fx: prolonged coagulation & bleeding time

 Low factor VIII levels

 Decrease platelet adhesiveness

renzN405 15
Clinical manifestations:

 Prolonged bleeding

 Hemarthrosis – hallmark

 Intracranial hemorrhage

 Recurrent hematoma formation

Severity of Bleeding:

Mild – bleeding with severe trauma or surgery

Moderate – bleeding with mild to mod. Trauma

Severe – spontaneous bleeding without trauma

Diagnostics:

1. Platelet count

2. Bleeding time

3. PT

4. PTT – prolonged

Management:

1. Control of bleeding

2. Prevention of bleeding with use of factor replacement

Drugs that replace deficient coagulation factors:

 Factor VIII concentrate from recombinant DNA

 Factor IX concentrate

Adjunctive measures

 DDAVP (deamino-D-arginine vasopressin)

- tx of choice for mild hemophilia & Von Willebrand

 NSAIDs - avoid use of aspirin

 Corticosteroids

 Amicar (aminocaproic acid)

- Oral or local application

- Prevents clot destruction

 Regular program of exercise and physical therapy

B. Disseminated Intravascular Coagulation

 Body’s response to overstimulation of clotting and anti-clotting processes in response to

injury

 Loss of balance between the clotting and lysing systems in the body

 Excessive thrombosis & excessive lysis occur simultaneously

renzN405 16
Causes:

1. Infection

2. Introduction of tissue coagulation factors into the circulation

3. Damage to vascular endothelium

4. Hemolytic transfusion reaction

5. Obstetric complications ( abruption placenta, IUFD)

Pathophysiology:

Clinical manifestations:

 Restlessness

 Anxiety

 Dyspnea

 Coolness of extremities

 Acute renal failure

 Altered mental status

 Signs of abnormal bleeding

Diagnostic findings:

 Low fibrinogen

 Prolonged PT

 Pronged PTT

 Reduced platelets

 Elevated fibrin split products

Management:

 Treat the underlying cause

 Replacement therapy for serious hemorrhagic complications

- Fresh frozen plasma

- Platelet transfusion

- Cryoprecipitate

 Supportive measures

 Heparin (controversial)

 Prevent injury

renzN405 17
Leukemia

• Hematopoietic malignancy with unregulated proliferation of leukocytes

Types:

1. Acute myeloid leukemia

2. Chronic myeloid leukemia

3. Acute lymphocytic leukemia

4. Chronic lymphocytic leukemia

A. Acute Myeloid Leukemia (AML)

 Defect in the stem cells that differentiate into all myeloid cells:

a. Monocytes, granulocytes, erythrocytes, and platelets

- Most common nonlymphocytic leukemia

- Affects all ages

b. Peak incidence at age 60

- Prognosis is variable

Manifestations:

 Fever and infection

 Weakness and fatigue

 Bleeding tendencies

 Pain from enlarged liver or spleen

 Hyperplasia of gums

 Bone pain

Treatment:

- Aggressive chemotherapy

- Induction therapy

- BMT

B. Chronic Myeloid Leukemia (CML)

 Mutation in myeloid stem cell with uncontrolled proliferation of cells: Philadelphia

chromosome

Stages

A. Chronic phase

B. Transformational phase

C. Blast crisis

renzN405 18
 CML

 Uncommon in people under 20

 Incidence increases with age;

 Mean age is 55 to 60 years

 Life expectancy is 3 to 5 years

Manifestations: (initially may be asymptomatic):

 Malaise

 Anorexia

 Weight loss

 Confusion

 Shortness of breath due to leukostasis

 Enlarged, tender spleen

 Enlarged liver

Treatment:

 Imatinib mesylate (Gleevec)

- Blocks signals in leukemic cells that express BCR-ABL protein

 Chemotherapy, BMT, and PBSCT

C. Acute Lymphocytic Leukemia

 Uncontrolled proliferation of immature cells from lymphoid stem cell

 Most common in young children, boys more often than girls

 Prognosis is good for children

- 80% event-free after 5 years,

- Survival drops with increased age

Manifestations:

 Leukemic cell infiltration is more common with this leukemia

 Symptoms of meningeal involvement

 Liver, spleen, and bone marrow pain

Treatment:

 Chemotherapy,

 Imatinib mesylate – if (+) Philadelphia chromosome

 BMT and PBSCT

 Monoclonal antibody therapy

D. Chronic Lymphocytic Leukemia

 Malignant B lymphocytes, most of which are mature, may escape apoptosis, resulting in
excessive accumulation of cells
renzN405 19
  Most common form of leukemia

 More common in older adults and affects men more often

 Survival varies from 2 to 14 years depending upon stage

Manifestations:

 Lymphadenopathy

 Hepatosplenomegaly

 Fever and weight loss

 Anemia & thrombocytopenia

Treatment: early stage may require no treatment,

 Chemotherapy

 Monoclonal antibody therapy

Laboratory tests

o Leukocyte count, ANC, hematocrit, platelets, electrolytes, and cultures reports

Nursing Diagnosis of the Patient With Leukemia

• Risk for bleeding

• Risk for impaired skin integrity

• Impaired gas exchange

• Impaired mucous membrane

• Imbalanced nutrition

• Acute pain

• Hyperthermia

• Fatigue and activity intolerance

• Impaired physical mobility

• Risk for excess fluid volume

• Diarrhea

• Risk for deficient fluid volume

• Self-care deficit

• Anxiety

• Disturbed body image

• Potential for spiritual distress

• Grieving diagnoses

• Deficient knowledge

renzN405 20
Potential Complications:

• Infection

• Bleeding

• Renal dysfunction

• Tumor lysis syndrome

• Nutritional depletion

• Mucositis

• Depression

Planning the Care of the Patient With Leukemia

Major goals include

- Absence of complications

- Attainment and maintenance of adequate nutrition

- Activity tolerance

- Ability for self-care

- To cope with the diagnosis and prognosis,

- Positive body image

- An understanding of the disease process and its treatment

Interventions

1. Mucositis

– Frequent, gentle oral hygiene

– Soft toothbrush, or if counts are low, sponge-tipped applicators

– Rinse only with NS, NS and baking soda, or prescribed solutions

– Perineal and rectal care

2. Improving Nutrition

- Provide oral care before and after meals

- Administer analgesics before meals

- Provide appropriate treatment of nausea

- Provide small, frequent feedings with soft foods that are moderate in temperature

- Provide a low-microbial diet

- Provide nutritional supplements

renzN405 21
Lymphoma

 Neoplasm of lymph origin

 Hodgkin’s lymphoma

 Non-Hodgkin’s lymphoma

 Hodgkin’s Disease

 Unicentric origin

 Reed–Sternberg cell

 Suspected viral etiology

 Familial pattern

 Incidence occurs in early 20s and again after age 50

 Excellent cure rate with treatment

Manifestations:

1. Painless lymph node enlargement

2. Pruritus

3. Fever

4. Sweats

5. Weight loss

Treatment: Determined by stage of the disease

 Chemotherapy

 Radiation therapy

Non-Hodgkin's Lymphoma (NHL)

 Lymphoid tissues become infiltrated with malignant cells that spread unpredictably;
localized disease is rare

 Incidence increases with age - the average age of onset is 50 to 60

 Prognosis varies with the type of NHL

Treatment: determined by type and stage of disease

 Interferon

 Chemotherapy

 Radiation therapy

renzN405 22
Multiple Myeloma

 Malignant disease of plasma cells in the bone marrow with destruction of bone

 M protein and Bence-Jones protein

 Median survival is 3 to 5 years; there is no cure

Manifestations:

1. Bone pain

2. Osteoporosis

3. Fractures

4. Elevated serum CHON

5. hypocalcemia

6. Renal damage

7. Renal failure

8. Symptoms of anemia

9. Fatigue

10. Weakness

11. Increased serum viscosity

12. Increased risk for bleeding and infection

Treatment:

 Chemotherapy

 Corticosteroids

 Radiation therapy

 Biphosphonates

Prepared by:

NCM104 Instructors

HAUCON

renzN405 23