word-- hema

HEMATOLOGIC DISORDERS HEMATOLOGIC SYSTEM Refers to the blood and blood forming organs.  BLOOD - transports cellular components and products from one part of the body to another.  It is composed of plasma(55%) and cellular component(45%)- RBC, WBC, Platelets.  Slightly alkaline 7.35-7.45  5-6 L or 70-75 ml/kg BW or 7 to 10% of BW The formation of the RBC,WBC and platelets begins in the bone marrow  Stem cells are produced in the bone marrow  Initially stem cells are not differentiated ELEMENTS OF BLOOD  PLASMA – liquid component. Serum – plasma without the clotting factor.  90% water and the rest are plasma proteins (albumin and globulin), clotting factors etc.  FORMED ELEMENTS – RBC,WBC and Platelets FORMED COMPONENTS  RED BLOOD CELLS (RBC)  Erythrocytes- life span is 120 days.  Hemoglobin – made from Heme (Fe+) and globin  Carries hemoglobin to provide O2 to tissues  Erythropoeisis Need – erythropoietin factor(kidney), Iron, Vit.B 12, Folic Acid, Vit.B6(Pyridoxine) and Protein.  Fe deficiency ( from bleeding in the GI / etc., or heavy menstrual flow )  Folic acid – diet. Absorbed in proximal small intestine  Vit. B 12- meat/products. Combines with intrinsic factor ( found in stomach). Problem for gastrectomy –partial or total  HEMOLYSIS  Aged erythrocytes loose their elasticity and become trapped in small blood vessels.  Removed from the blood by the RES particularly liver and spleen.  Hemoglobin – recycled others breaks down to form bilirubin and secreted in the bile.  Fe- recycled others lost in feces / urine or menstrual flow WHITE BLOOD CELLS (WBC)  Leukocytes – fights infection  GRANULOCYTES 1.Neutrophils – essential in preventing or limiting bacterial infection via phagocytosis (polymorphonuclear neutrophils / lecukocytes ) (1st step phagocytosis) 2.Easinophils – involved in allergic reactions; digests foreign proteins 3. Basophils- contains histamines integral part of hypersensitivity reactions.  AGRANULOCYTES  1.Monocytes – phagocytosis esp against fungus( 2nd step)  2.Lymphocytes – integral component of immune system.  a. T lymphocytes – kills foreign cells directly/ enhance phagocytosis. Responsible for delayed allergic reactions, rejection of foreign tissues(transplanted organs) and destruction of tumor cells.( Cellular Immunity )  b. B Lymphocytes – capable of differentiating into plasma cells. This produce antibodies called Ig which are protein molecules that destroy foreign materials. ( Humoral Immunity)  PLATELETS- play a role in controlling bleeding. During vascular injury they collect at the site of the injury and to each other forming a platelet plug that stops bleeding.—forming fibrin. ASSESSMENT  PHYSICAL S/S 

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Pallor – conjunctiva Jaundice –(hemolytic) Petecchiae – small pinpoint hemorrhage Ecchymosis- small bruises Hematoma –large bruise Lymph node enlargement Spleenomegaly or hepatomegaly

DIAGNOSTIC PROCEDURE  BLOOD CBC with differential a. Hemoglobin – Males13-16 gm/dl - Females 12-14 gm/dl b. Hematocrit – Males 42-50% - Females 40-48% c. RBC – Males 4,600,000 – 6,200,000 mm3 Females 4,200,000 – 5,400,000 mm3 d. WBC – N=5,000 – 10,000 cu.mm *neutrophils – N=60-70% *eosinophils – N=1-4% *basophils – N=0–0.5% *monocytes – N=2-6% *lymphocytes – N=20-30% e. Platelets – N=200,000-350,000 per cu.mm COAGULATION SCREEN  Prothrombin time-11-16 secs. (time for clotting after thromboplastin and Ca are added to plasma)  Coumadin tx Warfarin INR less 2 2.5-3.5  Partial Thromboplastin Time-60-70secs(after Partial T reagent is+plasma)  Best single screening test for coagulation d/o  Activated Partial Thromboplastin Time -30-45 secs . Same as ptt.  Most specific for heparin tx  Clotting Time – N= 5 to 10 minutes  minutes

Bleeding Time – N=30 sec – 6

BLOOD CHEMISTRY NPO 6-8 HRS.  a. Blood Urea Nitrogen (BUN) – N=10-20mgs/dl  b. Creatinine – N=0.71.4mgs/dl  c. Uric acid – N= 2.5-8.0 mg/dl  d. Cholesterol – N=150300 mg/dl  e. Bilirubin – Total N=0.1-1.0 mg/dl  direct (N=0.10.2mg/dl)  indirect (N=0.10.8mg/dl) SPECIAL BLOOD TEST A. ESR – N=0 to 20 mm/hr (inflamm process) B. SCHILLING’S TEST – for pernicious anemia (lack intrinsic factor needed for the absorption of Vit B 12 in GI) 1. prep NPO x 8 hours 2. radioactive oral Vit.B12 given first then 3. Vit.B12 nonradioactive given IM-flush oral B12 in urine 2 hours after 24hr. urine collection for radioactive Vit.B12; N = 15-40% of oral dose excreted (inc. means B12 not absorbed secondary to lack intrinsic factor) OTHER TESTS  Urine and Stool  Urinalysis

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 Hematest  Hemoccult – prep; -no dark colored food x 24 hours prior to test Radiologic  CXR  Scan  Lymphangiography Bone Marrow aspiration and biopsy Bone Marrow Aspiration and Biopsy  Assess the quantity and quality of each type of cell produced in the bone marrow  Preferred site – iliac crest, sternum or tibia  Before: consent, position exposing the site  After: pressure to site x5miuntes

ERYTHROCYTE DISORDERS  Anemia – reduction below normal level in number of erythrocytes, quantity of hemoglobin and volume of packed RBC’s.  Basic underlying – tissue hypoxia  Related = blood loss, nutritional deficiency (Fe,Vit.B12, folic acid), lack of RBC production or bone marrow failure.  Hx of anemia secondary to genetics – thalassemia and sickle cell. ANEMIA

Treatment for Fe def. 2 *Parenteral – avoid tissue staining by using separate aspiration injection needles; Z-tract method and deep IM; do not massage but encourage ambulation. Ex. Fe dextran(imferon) , Fe Sorbitex ( Jectofer) *Dietary – increased in iron and roughage *Blood transfusion Megaloblastic Anemia  Folic acid deficiency – decrease in folic acid  Causes  poor dietary intake (green vegetables, organ meats, meats, fresh fruits)  impaired absorption in upper jejunum  increased requirement –pregnancy  impaired utilization – folic acid antagonists like  S/s – fatigue,weakness,pallor etc.  DX – low serum folate  TX- Oral Folic acid, increase diet, treat cause

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Signs and Symptoms – depends upon severity and chronicity and age.  a. Mild – asymptomatic; palpitations, dyspnea and diaphoresis following strenuous exertion.  b. Moderate – increased palpitations, dyspnea, and diaphoresis; fatigue at rest or during activity.  c. Severe – pale and exhausted all the time, severe palpitations, sensitivity to cold, loss of appetite, profound weakness, angina. Iron Deficiency Anemia Causes:  Inadequate absorption – increased requirement  Inadequate intake of iron rich foods  Physiologic need – more in children and pregnant women  Physiologic loss – menstruation  Blood loss – trauma, GI bleeding

Fe Deficiency Anemia  Signs and Symptoms:  Palpitations, dizziness, easy fatigability  Cold sensitivity, pallor  Brittle nails, and hair  Plummer-vinsons syndrome – soreness and inflammation of mouth and tongue (stomatitis and glossitis)  Hypochromic,microcytic RBC  Koilonychia – thin concave nails raised at edges (spoon nails) DX Fe Def. Anemia  Low Hemoglobin  Normal then Microcytic, hypochromic cells  Serum fe low  Peripheral blood smear – poikilocytosis (different shapes of RBC)  Nursing management: *Oral iron – route of choice; given after meals (taken 3 x a day) liquid iron intake with straw because it stains; mixed with 1 glass cold H2O, best absorbed with Vitamin C; stool becomes tarry and constipation may occur. Eg. Fe So4, Fe Gluconate, Fe Fumarate

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Pernicious anemia – Vitamin B12 (cyanocobalamine) deficiency of intrinsic factor in the gastric mucosa which is necessary for absorption of Vit.B12. Signs and Symptoms:  Hemolytic jaundice – macrocytic hypochromic  Tingling sensations, paresthesias  Beefy red tongue  Deficiency or absence of hydrochloric acid in the stomach  +Schillings test Diagnostic assessment:  Schilling’s test  Gastric analysis Nursing management:  Drug therapy – Vit B12 injections (monthly) for life  Folic acid – reverses anemia, decreases neurological symptoms  Transfusion therapy  Avoid activities that could lead to injury/changes in balance

APLASTIC ANEMIA  Depressed bone marrow activity secondary to antineoplastics, radiation, insecticide, drugs and chemical toxins. Laboratory Assessment: pancytopenia  Erythrocytopenia (anemia)  Leukocytopenia(infection -URTI -sepsis)  Thrombocytopenia (bleeding gums, nose, GI etc.)

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Nursing management:  Blood transfusion  Prevent and treat infections  Bone marrow transplant  Drug – corticosteroids; estrogen  Identify and withdraw offending agent

POLYCYTHEMIA  Polycythemia ( compensatory response to hypoxia - inc. RBC, hemoglobin)  POLYCYTHEMIA VERA-unknown cause  Proliferative disorder of the bone marrrow with increse in RBC,WBC & platelets  S/S - increase blood volume and viscosity  Reddish skin,head-ache, painful fingers and toes,hepato/splenomegaly, HPN, prone to CVA and MI.

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Leukocyte Disorder  Leukopenia is condition of fewer WBCs than normal, results from neutropenia or lymphopenia  Neutropenia results from decreased production or increased destruction of neutrophils  Medical management varies depending on cause  Nursing management is towards preventing and managing infections   

 A. HODGKIN’s – malignant neoplasms of lymphatic tissue originating in lymph nodes proliferating to spleen and liver  Signs and Symptoms – enlarged non tender nodes, abdominal pain (spleenomagaly), cough,dyspnea,dysphagia (enlarged mediastinal nodes)  DX lymph node biopsy -prescence of Reed Sternberg cells(malignant cells-Hodgkins  Management – chemotherapyMOPP (Nitrogen Mustard Oncovin Procarbazine Prednisone ), radiation

Treatment Phlebotomy to decrease blood Increase fluid to reduce blood viscosity Monitor for signs of bleeding Chemotherapy to inhibit hyperactivity of the bone marrow Administer analgesics, antihistamines(pruritus)

Leukocytosis is an increased level of WBCs Leukemia is a neoplastic proliferation of one particular cell Leukemia – most common of childhood (3-5 y/o) cancer; abnormal proliferation of WBC in blast form. Predisposing factors:  Radiation – Xrays. CV lab  Survivors of Hiroshima  Chemical induced - fertilizers

Leukemia  Proliferation of the WBC in the bone marrow, spleen, liver, lymph nodes.  Classification  1. Cell type - lymphocytic, granulocytic , monocytic  2. Maturity of Malignant cells  Acute - immature cells  Chronic - differentiated cells Types of Leukemia *Acute lymphocytic leukemia (ALL)  80-85%of childhood leukemia  95% chance of obtaining remission with diagnostic assessment  75% chance of surviving over 5 years *Acute non-lymphocytic anemia (ANLL)  granulocytic and monocytic  60-80% will obtain remission with treatment  30-40% cure rate S/S OF LEUKEMIA      

Anemia – weakness, pallor, dyspnea Petechiae, spontaneous bleeding Infection, - fever, malaise Enlarged lymph nodes, liver and spleen Abdominal pain, weight loss, anorexia Bone pain due to expansion of marrow

MANAGEMENT : Leukemia *Supportive therapy – rest, blood transfusion, prevent infection, promote nutrition, oral hygiene, skin care *Drug therapy – antileukemia – oncovin, prednisone, methotrexate (2-3 yrs.) *Radiation *Bone marrow transplant LYMPHOMA  Lymphatic tissue (lymphocytes)

b. NON HODGKIN’S – tumor originating in lymphatic tissue characterized by diffuse, undifferentiated cell; prognosis is poorer than Hodgkin’s.( no Reed Sternberg cells) Management:  *chemotherapy  *radiotherapy and  *surgery for diagnosis and staging THROMBOCYTOPENIA  Decrease platelet production(bone marow supression, chemo/radiotherapy  Increase platelet destruction(infection)  S/s - petechiae,ecchymosis(eg ,site of venipuncture), hematoma, bleeding-nose,GI,GU  TX - treat underlying disease, Platelet transfusion PLASMA DISORDERS Hemophilia  Congenital blood dyscrasia characterized by disturbance in blood clotting.Appears in males but is transmitted by females.  Hemophilia A- classic - Factor 8 anti hemophilia factor  Hemophilia B- Factor 9 - Christmas factor  X-linked traits affect males  Dx - PTT prolonged  S/s - easily bruised, prolonged bleeding from nose, mouth or laceration, hematuria  Tx- Pressure on bleeding site, plasma concentrate/ cryoprecipitate, Factor 8/9 concentrate BLOOD TRANSFUSION  Whole blood  Packed RBC  Platelet Concentrates  Fresh Frozen Plasma - plasma+ clotting factors  Plasma deriv.- clotting factors +10ml plasma eg Cryoprecipitate  Albumin BLOOD TRANSFUSION Procedure   

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1. Consent for blood transfusion 2. Identification of patient and blood- pts hospital no., name, type of infusion, expiration date etc. ; 3.Obtain vitals signs - immediately before starting, 15 minutes after then every 30 minutes for the next 2-3 hrs and end. Also 1 hr after transfusion 4. Start slowly at 2ml/ minute . Monitor for adverse reactions. 5. Record in BT chart.

BLOOD TRANSFUSION ADVERSE REACTION

1. ALLERGIC - sensitive to plasma protein s/s - flushing, itchiness, rash, wheezing Tx- stop BT, KVO with saline, antihistamine 2. FEBRILE - sensitive to donor wbc, platelets & plasma proteins s/s - sudden chills, fever, headache, flushing Tx - Stop BT, send blood samples and blood bags to blood bank. Monitor temp. Antipyretics as prescribed. 3.SEPTIC - transfusion of blood with bacteria s/s - chills, high fever, hypotension TX - stop BT., get blood culture fr. Pt and return blood bags 4.CIRCULATORY OVERLOAD - fast administration s/s - distended neck veins, dyspnea, crackles at base of lungs TX - stop BT, semi fowlers position, diuretics as ordered,O2 5.HEMOLYTIC - incompatible blood products S/s - chills, fever, low back pain, inc HR & RR, Hypotension TX- Stop BT, notify MD and blood bank, treat shock, antihistamine