BLOOD DISORDERS

Minerva A. Cobus

Overview of Hematological System

Bone Marrow

Contained inside of the bone, occupies interior of spongy and center of long bones 4-5% of total body weight Primary function is Hematopoiesisformation of blood cells

Bone Marrow

2 kinds:
a. Red marrow (functioning)
a. b.

Carries out hematopoiesis Found in ribs, vertebral column, other flat bones Red marrow that has changed to fat; found in long bones Does not contribute to hematopoiesis

b. Yellow marrow
a.

b.

Blood

Composed of plasma (55%) and cellular components (45%) Hematocrit

a. reflects portion of blood composed of RBC

Plasma

Liquid part of blood; yellow in color Consist of serum (liquid portion of plasma) and fibrinogen Contains plasma proteins such as:
a. Albumin: largest plasma proteins, involve in regulation of intravascular plasma volume and maintenance of osmotic pressure

Plasma
b. Serum globulins:
a.

b. c.

Alpha role in transport of steroids, lipids, bilirubin Beta: role in transport of iron and copper Gamma: role in immune response, function of antibodies

c. Fibrinogen, prothrombin, plasminogenfor coagulation

Cellular Components
Erythrocytes

RBCs responsible for oxygen transport

Normal 12-18 g Hgb/100cc blood; men- 14-18 g; women 12-14g Average life span- 120 days

Leukocytes-

protection from bacteria and other foreign substances

Cellular Components
Thrombocytes

essential factor in coagulation via adhesion, aggregation, and plug formation

Diagnostic Test

 Blood

Complete CBC: WBC; Hgb and Hct; platelet and reticulocyte count Coagulation studies: PT; PTT; (FSP) fibrin split products; Lee-white clotting (whole blood clotting time) Blood Chemistry: BUN; creatinine; uric acid
Urine

and stool

Urinalysis Hematest Bence-Jones protein assay (urine)

 Radiologic

Chest x-ray Radionuclide scans (bone scan)

Bone

marrow aspiration and biopsy

Puncture of the iliac crest (preferred site), vertebrae body, sternum, or tibia (in infants) to collect tissue from bone marrow Purpose: study cells involved in blood production

 Nursing care: Confirm consent Allay anxiety Position client and assist physician to maintain sterile field Immediately after aspiration, apply pressure to the site for at least 5 minutes and longer, if necessary Send specimen to the laboratory.

Sickle Cell test

Sickledex

No

hemoglobin S present Routine screening test for cell, trait or disorder; does not distinguish between them

Hemoglobin Electrophoresis
Separates

various hemoglobins and allows for identification of specific problem Differentiates between traitor disorder in sickle cell anemia Diagnosis of thalassemia and hemolytic anemia

Fibrin Split Products

Fibrinolysis

coagulation Abnormally high levels in DIC

occurs in intravascular

Schilling Test
Normal

excretion is 10-40% of oral dose of the radioactive B12 Stage 1-is a 24-hour urine test after a dose of radio labeled B12 Stage 2-is a second 24-hour urine to determine response to intrinsic factor; distinguishes between pernicious anemia and malabsorption.

BLOOD COMPONENT THERAPY

Plasma Components and Products

Consist of: Fresh frozen plasma Cryoprecipitate

Fresh Frozen Plasma

Plasma which is separated from whole blood and frozen within 6 hours of phlebotomy. Content: -plasma protein -all coagulation factors ( include labile F V + F VIII) Storage and shelf life: - storage at -18C - deep freeze at -40C can be kept for 2 year

FFP
Properties: -1 unit volume: 200 ml - pediatric : 50-100 ml Administration : - thawed in 37C water bath - administer within half an hour after thawing ( activity of labile F V, V III is rapidly lost)

FFP

Definite indications: 1) Replacement for isolated factor deficiencies (specific factor not available eg: F V) 2) Immediate reversal warfarin effect. 3) Replacement of coagulation factors in: DIVC. 4) Inherited def of inhibitors of coagulation eg: Anti thrombin III, protein C, protein S.

FFP

Conditional uses (FFP only indicated in the presence of bleeding and disturbed coagulation): Massive transfusion Liver disease Cardiopulmonary bypass surgery Special pediatric indications eg: severe sepsis

FFP

Contraindication: estimated as many 73% are used without justification 1) volume expander 2) nutritional source of protein + immunoglobulin 3) Urticaria + anaphylactoid reaction or previous severe reaction to plasma

FFP

Dose + Administration: - ABO compatible with recipient blood cell + without regard to Rh type - Cross matching not required - Amount used depend on clinical situation eg: DIVC case: 2 units for adult paediatric: 10-20 mls /kg - efficacy assessed with PT, aPTT

Platelets
Will

raise recipient's platelet count by 10,000 u/liter Pooled from 4-8 units of whole blood

Albumin Preparation
Protein

solution containing predominantly albumin prepared by fractionation of large pools of plasma. Properties: 1) Albumin solution 2) Plasma protein fraction

Albumin solution
Available in 5% and 25% solution. Contain 96% albumin and 4% globulin + other plasma protein. Pooled plasma product and is heat treated to inactivate viral activity. 5% solution: osmotically + oncotically equivalent to plasma 25% solution; markedly hyperoncotic. Shelf life: 5 years at 2-10C.

Plasma Protein Fraction

Prepared

albumin. Differences: albumin content 80 to 85% Stored at room temperature for 3 years or at 1-6C for 5 years. Administration: - albumin + PPF do not contain ABO antigen or Ab compatibility not required

the same manner as

Albumin preparation
Indication (both): Volume expansion and colloid replacement for patients in hypovolemic shock, burn, undergoing retroperitoneal surgery in which large volume of protein-rich fluid may pool in the bowel. Indication for PPF: parallel those for 5% albumin. Albumin solution not indicated to correct nutritional def (enteric, parenteral, hyperalimentation > appropriate)

Factor VIII Concentrate

FVIII

concentrate or antihemophilic Factor (AHF) specific treatment for F VIII def (hemophilia A) Prepared fr large donor pools of plasma Pooled plasma is fractionated , lyophilized + stored at 1-6C Heat treated to reduce risk of transfusion transmitted disease.

Factor IX Concentrate
1) 2)

2 heat treated , lyophilized concentrates of F IX: Factor IX complex Pure Factor IX concentrate F IX complex (Prothrombin complex) Contain factor II, VII, IX and X F IX concentrate: contain 20-30 % F IX, trace amount of F II, VII, X.

Factor IX concentrate

1)
2)

Indication: Hemophilia B Haemophilia A with inhibitors Contraindication: -Fac IX complex a/w thromboses and episode of DIC. - Adverse effect also seen in Haemophilia A with inhibitors

Autologous Transfusion
Therapeutic benefit: 1) Safe (risk of TTD eliminated) 2) No alloimmunization Conserves blood banks limited homologous blood supply Either be: 1) Perioperative Hemodilution 2) Intraoperative Salvage 3) Postoperative Blood Salvage

Perioperative Hemodilution
Eg: Cardiopulmonary bypass surgery Withdraw 1/> units of blood preoperatively + replace with crystalloid / colloid solution normovolemic, hemodiluted state Results in improved blood flow in pt circulation + loss of < red cell Labelled properly (pt name, RN,date/time phlebotomy) Reinfuse within 8 hr: reliable source of plt, coag factors.

Intraoperative Salvage
Blood-containing fluid aspirated fr op site, centrifuge or washed and reinfused through a filter during op/post op. Vascular procedure large amount of blood pool in body cavities. Collection device washes with 0.9% saline + transfuse immediately or stored at room temperature for 6 hrs or at 1-6C for 24 hrs. Complication: hemolysis, DIVC, air

Postoperative Blood Salvage

Within first 24-48 hrs after surgery in pt actively bleeding in closed site (chest, joint cavity) Contraindicated if evidence of infection or malignant tumor cell at site Blood collected into sterile container, labelled and filtered. Reinfuse within 6 hours, to minimize proliferation of bacteria

Nursing Care during Blood Transfusion

1.

2. 3. 4. 5. 6. 7.

Assess client for history of previous blood transfusion and any adverse reactions. Ensure that pt. has an 19 or 19 gauge IV catheter in place Use .0% sodium chloride At least 2 nurses should verify the blood component Take baseline v/s Start transfusion at slow rate Stay w/ the client for the 1st 15 mins; continue monitor v/s

8.

Maintain prescribe transfusion rate:

1. 2. 3. 4. Whole blood: approx 3-4 hours RBCs: approx 2-4 hours FFP: as quickly as possible Cryoprecipitate: rapid infusion

9. 10.

Monitor for adverse reactions Document the following:

1. Blood component unit number 2. Date infusion starts and ends 3. Type of component and amount transfused 4. Client reaction and v/s

Transfusion Reaction
1.

Hemolytic Reaction with in 15 min

a. S/sx headache, lumbar or sternal pain, dyspnea, restlessness, fever, chills, heat along veins, renal shutdown b. Intervention stop transfusion, obtain 1st voided urine specimen, send blood unit and client blood sample to lab, continue saline IV

Transfusion Reaction
2.

Allergic Reaction with in 30 min

a. S/sx uticaria [hives], laryngeal edema, dyspnea, sever-shortness of breath b. Intervention stop transfusion, administer antihistamine and/or epinephrine.

3.

Febrile Reaction with in 15 90 min

a. S/sx fever, chills, flushing, headache, N & V, palpitations b. Intervention treat temperature, keep client covered and warm during transfusion, administer antipyretic medication.

ANEMIAS
Decreased

availability of oxygen to the tissues Kinds:

Iron Deficiency Anemia Aplastic Anemia Folate Deficiency Anemia Pernicious Anemia Hemolytic Anemia Sickle Cell Anemia

ANEMIAS
Causes

Acute / chronic blood loss Inadequate dietary intake of vitamins / minerals needed for rbc production Decreased rbc production by the bone marrow Increased destruction of rbc Increased demands of vitamins / minerals needed for rbc production

COMMON CLINICAL MANIFESTATIONS

Pallor Easy fatigability Weakness Anorexia Weight loss SOB Headache / Dizziness Tachycardia / palpitations

Syncope Brittle hair and nails Paresthesia Cold sensitivity Amenorrhea

Iron-deficiency Anemia

Chronic microcytic, hypochromic anemia caused by either inadequate absorption or excessive loss of iron. Risk Factors:
a. Women between 15-45 y/o b. Common in infants whose primary diet is milk c. Heavy flow during menses

Assessment:
a. Mild cases usually asymptomatic b. Palpitations, dizziness, and cold sensitivity c. Dyspnea, weakness d. Laboratory findings:
a.
b. c. d.

RBCs small and pale HGB markedly decreased Hct decreased Serum iron decreased

Nursing Interventions
Promote rest Provide good oral care Provide good skin care. Diet: Iron rich foods

Organ meals Beans Green, leafy vegetables Raisins

a. b. c. d.

Monitor for s/sx of bleeding Provide adequate rest Explain all diagnostic procedure Administer iron preparation

COLLABORATIVE MANAGEMENT

Medical Management
Iron Supplement
Oral

Ferrous Sulfate Ferrous Gluconate Ferrous Fumarate

Parenteral:

Iron Dextran (Imferon)

Oral iron should be given p.c. Oral liquid iron to be administered with straw Vitamin C increases iron absorption X administer with milk, antacid Imferon : Z track (deep IM) Do not massage site of injection Iron salts change color of stool to dark green or black

Oxygen

therapy Blood transfusion as needed

Causes

FOLATE DEFICIENCY ANEMIA (MEGALOBLASTIC ANEMIA) Assessment

Cracked lips, sore tongue Same as Pernicious Anemia

Poor dietary intake Rarely eat uncooked (raw) fruits and vegetables Alcoholism Chronic malnutrition Pregnancy Anorexia nervosa Malabsorption Malignancy Prolonged TPN Chronic hemodialysis

Collaborative Management
Well balanced diet Folic acid 1mg. / day

Macrocytic, hyperchromic anemia Causes

Gastric surgery Crohns disease

PERNICIOUS ANEMIA

Pathophysiology INTRINSIC FACTOR production VITAMIN B12 absorption RBC production DNA synthesis in maturing RBC (megaloblastic cells) Impairment of integrity of cells (mouth, stomach, anus, vagina, axon of neurons)

DIAGNOSTIC TESTS

Tubeless Gastric Analysis

Diagnex blue / Azuressin tablet

Collect urine specimen

blue HCl is present (-) Pernicious Anemia X blue

(-) HCL Achlorhydria

(+) Pernicious Anemia

DIAGNOSTIC TEST
Schillings

Test

Most definitive diagnostic test Oral radioactive Vitamin B12 IM nonradioactive Vitamin 12 Collect 24 hour urine specimen Decreased excretion of Vitamin B12 in the urine (+) Pernicious Anemia

COLLABORATIVE MANAGEMENT

Vitamin B12 IM
Daily: Weekly: Monthly: 7 days 10 weeks LIFETIME (100mg/Mo)

Hydrochloric acid p.o. for 1 week Iron therapy Blood transfusion as needed Physical examination every six months *At risk for Gastric Cancer

APLASTIC ANEMIA

Hypoplasia of the bone marrow Diagnostic tests Fat replaces bone Bone marrow marrow aspiration / PANCYTOPENIA biopsy
Anemia Leukopenia Thrombocytopenia Congenital Acquired Idiopathic Infections Medications Heavy metals Hepatitis
Adult

Causes

Posterior Iliac Crest Prone Position Brief , sharp pain

Child

Tibia Side lying position

COLLABORATIVE MANAGEMENT
Medical

Management

Bone marrow transplantation Immunosuppressive therapy (Antithymocyte Globulin ATG)

Central

line 7 10 days

Blood transfusion as necessary Stool softeners; high fiber diet

NURSING INTERVENTIONS
Assess for signs and symptoms of infection, tissue hypoxia and bleeding Frequent rest periods Good oral care Use soft bristled toothbrush / soft swabs Male clients

Use electric razor

NURSING INTERVENTIONS
X ASA administration Reverse / Protective Isolation Practice asepsis X fresh fruits / fresh flowers in the patients unit Limit visitors X allow persons with signs and symptoms of infection to visit

Hemolytic Anemia
Cause

often unknown Characterize by increased rate of RBC destruction

Assessment:
Pallor, slight jaundice Hematuria Melena

Intervention
Monitor for s/sx of hypoxia If jaundice and associated pruritus are present , avoid soap. Frequent turning

Medical Mngt:
Folic acid supplements BT Corticosteroids in autoimmune types of anema

Sickle Cell Anemia

Characterized

by the sickling effect of the erythrocytes Predominantly a problem of children. Asymptomatic until 4-6 mons of age. Autosomal Recessive

Pathologic changes results from:

Increased blood viscosity Increased red cell destruction 6 20 days life span

Types of Crisis
1.

2.

3.

Vaso-occlusive severe pain, thrombotic; caused by stasis of blood w/ clumping of cells in the microcirculation Splenic sequestration life threatening crisis, death can occur w/in hours; pooling of large amount of blood in the spleen and liver Aplastic diminished production and increased destruction of RBC.

 Intervention

Pain control Promote hydration 4 6 liters/day, folic acid supplement Control anxiety Bedrest BT Genetic counseling Prevent infection Discharge planning and home care.

DISORDERS AFFECTING THE RBCS

Polycythemia
rbc and hgb production Compensatory response to chronic hypoxia

Hypoxia Erythropoietin production by the kidneys Stimulation of the bone marrow to rbc production

DISORDERS AFFECTING THE RBCS

Polycythemia Vera
Hyperplasia of the bone marrow rbc (erythrocytosis); wbc (leukocytosis); platelets (thrombocytosis) Cause is unknown; associated with genetics Pathophysiology rbc, wbc, platelets

blood viscosity thromboembolism

organ

infiltration
Hepatomegaly Splenomegaly Arthralgia Cerebral hypoxia

capillary overdistention Rupture Hemorrhage

Hypovolemia

POLYCYTHEMIA VERA

Signs and Symptoms

Headache Dizziness Blurred vision Fatigue Hepato-splenomegaly

COLLABORATIVE MANAGEMENT

fluid intake Monitor for signs and symptoms of bleeding Monitor for signs and symptoms of thromboembolism
Angina Claudication Thrombophlebitis Pruritus

Administer antihistamine to decrease pruritus Administer analgesic as ordered Therapeutic phlebotomy Chemotherapy Radiation therapy (Na Phosphate / IV) Patient teaching : Avoid high altitude

Disseminated Intravascular Coagulation

Hallmarks:

Excessive clotting, followed by dissolution

Presentation:

Cutaneous oozing, petechiae, cyanosis, N/V, oliguria, bleeding from GI tract

Causes:

Damage to endothelium Release of tissue thromboplastin Activation of Factor X

Disseminated Intravascular Coagulation

Tests/Therapies

TESTS:

THERAPIES

Platlets Prothrombin Time Fibrin

Supportive Fresh-frozen plasma, platelets, or packed red cells Heparin

 Intervention:

Watch out for bleeding problems Provide emotional support