HematologyByJoyce Smith RN BSN

Coagulation Disorder

• Platelet Disorders– Thrombocytopenia

• Autoimmune Thrombocytopenia Purpura• Thrombotic Thrombocytopenia Purpura

• Clotting Disorders– Hemophilia– DIC

Autoimmune Thrombocytopenia Purpura

• Immune Thrombocytopenia Purpura

• Idiopathic Thrombocytopenia Purpura

• Immunologic platelet destruction causes a marked decrease in number of circulating platelets

• Most common acquired thrombocytopenia

Autoimmune Thrombocytopenia Purpura

• Acute usually affects children 2-9, postviral

• Chronic adults <50, F 20 to 40, six months

• Recovery occurs within 1 to 2 months for patients with acute 90%

• Chronic 10 to 20 % recover without treatment

• Risk of acute hemorrhage greatest during 1st & 2nd weeks, intracranial bleed fatal

Risk Factors

• Immune-related disorders• Viral infections such as rubella,

chicken pox, mumps, measles, or smallpox

• Sensitivity to drugs, allergies, & blood transfusion

• Exposure to insecticides & chemicals, vinyl chloride

Clinical Response

• Insidious onset of bleeding from the mouth, nose, and skin upon slight injury

• Spontaneous bleeding form mucous membranes

• Generalized weakness, fatigue, and lethargy, petechiae & ecchymosis

Assessment

• Spontaneous bleeding episodes, CBC with severely low platelets <20,000/mm3

• Increased bleeding time, decreased platelet survival time & possible platelet antibodies

• Question about exposure to chemicals, recent immunizations, & exposure to or contractions of viral illness

Assessment

• Assess for minor bleeding, epistaxis or bruising tendencies

• Look for petechiae, hematomas, & superficial ecchymotic areas on skin

• Note change in LOC, confusion, & lethargy

• Palpate abdomen for liver & spleen enlargement

Treatment

• Reduce & control severity of bleeding• Maintain homodynamic stability• Identify possible cause of bleeding• Plasmapheresis• Splenectomy in chronic cases• Platelet transfusion• High-does gamma globulin to elevate

platelet count & reduce turnover

Treatment

• Corticosteroids & Imuran to suppress immune response in chronic

• Antimitotic drugs & cyclophosphamide

Nursing Diagnosis

• Risk for injury d/t prolonged bleeding time– Control localized bleeding– Transfuse if nec– Teach adequate oral hygiene,

including use of soft toothbrush or sponge, frequent brushing, no floss

– Avoid drugs that decrease platelet aggregation

Interventions

• Caution patient to avoid using razors with blades

• Use normal saline nasal drops or sprays to decrease drying of mucous membranes

Thrombotic Thrombocytopenia Purpura

• Rare blood condition characterized by formation of small clots in the circulation

• Uses up platelets causing low platelet count

• 1-3 million per year• Most common 20-40 years old• F 2X > M• Develops spontaneously <20%

factors that predispose

A & P

• Clots form in circulation & temporarily disrupt local blood supply

• Affects the blood vessels of the brain & kidney– Headache– Confusion – Difficulty speaking– Transient paralysis, numbness– Hypertension

Possible Causes

• Drugs• Pregnancy• Infections• Systemic lupus erythematosus• Malignancy

Clinical Manifestations

• Malaise • Fever• Headache• Occasionally diarrhea• Bruising, rarely bleeding• Purpura• Ecchymosis

Diagnosis

• No specific test to diagnose• Symptoms• Blood count• Renal function• Other illnesses with low platelet

counts have to be excluded

Treatment

• Corticosteroids• RBC transfusions• Folic acid• Platelet transfusions• Hepatitis B Vaccinations• Aspirin may be started when

platelet count reaches about ½ normal

Treatment

• Plasma exchanges daily X 5 days to be effective, 3 hours– Plasma removed & replaced by donor

plasma– Removes circulating antibodies

against cleaving protease, plasma with normal cleaving protease activity

– Allergic reactions– Tingling of fingers or around mouth

caused by low calcium levels

Poorly Responding Disease

• Alternative plasma replacement• Vincristine• Splenectomy• Immunosuppression

– Azathioprine– Cyclophosphamide– Ciclosporin

DIC (Disseminated Intravascular Coagulation)• Hypercoagulability state• Occurs when the bodies

coagulation is overstimulated• Secondary to: sepsis, burns,

cancer, major trauma, obstetric complications, CABG

• Diagnosis is by: PTT, PT, thrombin time, fibrinogen level, and D-dimer

• May use heparin, administration of blood products

• Takes lots of time and persistence to overcome this problem

Hemophilia

• Bleeding time greatly increased d/t impaired coagulation

• 0.01 % of US population• Hemophilia A: deficient or absent

factor VIII 80% of cases• Hemophilia B: Christmas disease,

deficient factor IX 15% of cases

Risk Factors

• 30% of persons with hemophilia have on notable family history of disease

• Male• Mother who is carrier, inherited x-

linked recessive disorder, female carrier has 50% chance of transmitting X chromosome to son or daughter

A & P

• Clotting factor deficiency impairs the hemostatic response, preventing clot formation

• Severity varies with degree of clotting factor deficient, specific cause, & location of bleeding

Clinical Manifestations

• Spontaneous bleeding• Skin & musculoskeletal sites stressed or

receive direct trauma• Excessive bleeding after circumcision• Prolonged bleeding after dental, surgical

procedures, or childbirth• Subcutaneous or muscular hematomas

lead to pressure on vital organ & produce damage

Complications

• Significant # of individuals with hemophilia received blood transfusions before 1984 became infected with HIV

• Bleeding into joints; knees, ankles, & elbows most common

• Repeated episodes lead to destruction of joint & loss of motion

Clinical Management

• Immediate halt bleeding• Avoid aspirin for pain• Immobilize joint & apply ice• Surgical correction of

musculoskeletal complications• Genetic counseling

Interventions

• May be necessary to give transfusion prior to dental or surgical procedure

• Synovectomy, joint debridement, or arthroplasty to treat hemarthrosis complications

Interventions

• Transfuse with cryoprecipitate, VIII & freeze-dried concentrates VIII or IX

• Genetically engineered synthetic factor VIII or recombinant factor VIII

• Desmopressin for mild hemophilia

Multiple Myeloma

• Neoplastic disease: bone & bone marrow infiltrated by defective plasma cells that form multiple tumors

• >50, M 2X >F, AA>C 2:1• Possible viral , hypersensitivity

reaction, & chronic inflammation, maybe genetic

• Excessive # neoplastic plasma cells

Clinical Manifestations

• Develop insidious & slow• Severe skeletal pain: usually

pelvis, spine, & ribs• Excessive accumulations of

abnormal plasma cells in bone marrow

• Osteoporotic lesions in skull, vertebrae & ribs

Clinical Manifestations

• Degeneration of bones leads to calcium loss into serum & cause hypercalcemia

• Precipitates renal dysfunction• Anorexia• Confusion • Hyperuricemia

Clinical Manifestations

• Thrombocytopenia• Anemia• Granulocytosis• Fatigue• Weakness • Weight loss• Tingling or myalgia of extremities

Diagnostics

• Pancytopenia• Elevated serum protein,

hypocalcaemia, hyperuricemias & creatinine

• Bence Jones protein in urine• X-ray bone scan, MRI:

osteoporosis, demineralization, tumors

• Bone marrow aspirate & biopsy

Clinical Management

• Long-term; symptoms management of chronic disease– Control pain– Palliative radiation– Drugs to prevent complications

• Hypocalcaemia, hyperuricemia, dehydration

Clinical Management

• Orthopedic support• Plasmapheresis• Chemotherapy• BMT• Fluids, diuretics, anti gout agents• NSAID• Skin care for RT

Clinical Management

• Long-term prognosis is poor• Final stages do not respond to

treatment • Hospice care