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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
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