hematologic disorder

7/31/2019 Hematologic disorder

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BLOOD PHYSIOLOGY/DISORDERS

N-103 MMCCOLLEGE OF NURSING 2011-2012 FIRST SEM

MAJOR FUNCTIONS1. TRANSPORT

2. DEFENSEPLASMA

Clear, straw-colored 91-92% water

PLASMA CONTENTS

WATER PROTEIN.. Albumin, globulin, fibrinogen, prothrombin, nutrients, met wastes,

electrolytesERYTHROCYTES

4.5 6.2 million/ cu mm Red bone marrow

Erythropoiesis 120 days/4 mo

hematocrit

Fraction of the blood occupied by RBC

***NORMAL : 3X the hb valueBLOOD CLASSIFICATION

MAJOR BLOOD GROUPSRh FACTOR

Present in RBC POSITIVE or NEGATIVE **O NEGATIVE no antigen, no Rh factor AB positive no antibodies in serum, Rh factor present

AGE-RELATED ASSESSMENT FINDINGS FOR HEMO DISORDERS

AREAS :

NAIL BEDS (CAPILLARY REFILL)

HAIR DISTRIBUTION

SKIN MOISTURE AND COLOR1. Nail beds

Pallor, cyanosis, and decreased capillary refill

**ELDERLY - ASSESS LIPS *nails are typically thickened and discolored2. Hair distribution

Thin or absent hair on trunk and extremities

**ELDERLY lack of hair or lower extremities and toes *loss of body hair in an evenpattern over time3. Skin moisture and color

Skin dryness, pallor, jaundice


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ELDERLY DRY SKIN IS normal

Pigment loss and skin changes like jaundice may occur

PallorANEMIAS

Excessive blood loss, inadequate production, excessive rbc

destruction ORIGINS

Defect in bone marrow production

Loss of RBC e.g. hemorrhage, chronic bleeding, hemolyticprocesses

Hereditary disorders

Inadequate nutritional intake of iron, B12, and folic acidIRON DEFICIENCY ANEMIA

inadequate intake/ excessive loss

Common in adolescents, infant on milk as primary diet, pregnancy;chronic blood loss ( gi bleed, menses, hookworm infestation);

Common in the Elderly**** poor dietary intake and decreasedabsorption in the small intestines

DIAGNOSTICS

HEMOGLOBIN; HEMATOCRIT

Serum iron and ferritin(M-18=270ng/mL, F-18-160ng/mL)


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Koilonychia

Pica

Dx sources of chronic blood lossTREATMENT

Supplemental iron for 6 months

Empty stomach; with meals if GI upset occurs

Liquid/enteric-coated

EGGS, MILK, CHEESE, ANTACIDS INHIBIT ORAL IRON

Increased dietary intake

Supplemental folic acidNURSING DIAGNOSES

Imbalanced nutrition, less than body requirements r/2inadequate intake

Ineffective tissue perfusion r/2 decreased oxygencarrying capacity of the blood

PERNICIOUS ANEMIA

loss of intrinsic factor NOT ASSOCIATED WITH INADEQUATEDIETARY INTAKE

**NEEDED FOR NORMAL DNA SYNTHESIS IN MATURING rbcDIAGNOSTIC

SCHILLING TEST

RBC MORPHOLOGY

GASTRIC ANALYSIS for free HClSCHILLING'S TEST

NPO PO RADIOACTIVE CYANOCOBALAMINE

IM RADIOACTIVE B12

URINE COLLECTION (24H)

NORMAL: at least 5%(+)RADIOACTIVE b12 IN URINE


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APLASTIC ANEMIABONE MARROW DEPRESSION

ETIOLOGY:MEDICATIONS e.g. chemotx, benzene, chloramphenicol,anticonvulsantsRadiation

Idiopathic

Congenital (Fanconis anemia)DIAGNOSTIC

BONE MARROW BIOPSY Site : posterior iliac crestPrep local anesthetic, analgesic, **feeling of pressure &painPost bleeding at site; apply pressure to site; bed restfor 30 min analgesic as indicated

CLINICAL MANIFESTATIONS

Anemia s/s

Fever, infections

Bleeding s/sNURSING DIAGNOSES

RISK FOR INFECTION

RISK FOR INJURYTREATMENT

BONE MARROW TRANSPLANTATION

ANDROGENIC HORMONE THERAPY to stimulate bonemarrow regeneration

CORTICOSTEROIDS / immunosuppressive treatmentsBONE MARROW TRANSPLANTATION

GOAL : TO RESTOR HEMATOLOGICAL AND IMMUNOLOGICALFUNCTION

BONE MARROW T - PROCEDURE ADULT CLIENT ASP OF 400 800 ML OF BONE MARROW FROMDONORS ILIAC CREST; PROCESsED AND TRANSFUSED TO RECIPIENT

PROCEDURE

TYPING

ALLOGENIC HISTOCOMPATIBLE DONOR E.G. RELATIVE

AUTOLOGOUS FROM CLIENTS OWN BONE NARROWWITHOUT DISEASE AND IS FROZEN

SYNGENEIC FROM IDENTICAL TWIN WITH PERFECTTISSUE MATCH

PREPARATION

IMMUNOABLATIVE PREP chemo and radiotx to produceimmunologically suppressed state before marrow transfusion; takes 5 7 days pre

COMPLICATIONS - BMT


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INFECTION due to immunosuppression

Bleeding from severe thrombocytopenia

GRAFT-VERSUS-HOST DISEASE / REJECTION (GVHD)GVHD

ACUTE REJECTION 7-30 days post

CHRONIC REJ occurs in 100 days

S/S erythematous rash on palms and feet, spreading to trunk;altered liver enzymes, liver tenderness and jaundice; GI s/s

Gvhd nsg implicationsPrep for immunosuppression with chemo and radiotxConfirmed by SKIN or ORAL MUCOSAL BIOPSYSUCCESSFUL ENGRAFTMENT hematopoiesis after 2 5 weeks postCare of an immunosuppressed client

POLYCYTHEMIA VERA (PRIMARY)

Proliferation/HYPERPLASIA of all red marrow cells

Occurs during middle age

s/s ruddy complexion, headache, hepatosplenomegaly, pruritus, s/spoor perfusion e.g. angina, claudication, thrombophlebitis,hypertension

NURSING DIAGNOSIS INEFFECTIVE TISSUE PERFUSION (multiple organs)

DIAGNOSTICS

CBC

BONE MARROW ASPIRATE AND BIOPSY

URIC ACIDTREATMENT

PHLEBOTOMY 2-3 TIMES PER WEEK

ANTICOAGULANTS

immunosuppressantsLEUKEMIAS

Uncontrolled proliferation of abnormal WBC and eventual cellulardestruction occurs as a result of the infiltration of the leukemic cells intobody tissue Primarily affected are high vascular organs of RES..spleen, liver,lymph nodes

Infiltration, enlargement, fibrosisLEUKEMIAS

PRIMARY CONSEQUENCES:

ANEMIA INFECTION BLEEDING TENDENCY

LEUKEMIA : types

ACUTE LYMPHOCYTIC (BLAST OR STEM CELL


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ACUTE MYELOGENOUS (AML)

CHRONIC MYELOGENOUS (CML)

CHRONIC LYMPHOCYTICTYPE: ACUTE LYMPHOCYTIC

PEAK = 4 YRS OLD; 65 YRS OLD

Favorable prognosis with chemotherapy

Can infiltrate meninges causing increased ICPTYPE: AML

ADULTS**disease of older people with median age of 67 ONSET age 15 35 yrs

TYPE : cml

Common after age of 20 Slow onset

s/s less severeTYPE : CML

COMMON OLDER ADULTS

FREQ ASYMPTOMATICDIAGNOSTICSBONE MARROW ASPIRATION ** increased number of blast cellsLumbar puncture ** presence of leukemic cellsCHRONIC MYELOCYTIC LEUKEMIA

TREATMENT PHASES:

INDUCTION - 2 destroy leukemic cells

INTENSIFICATION starts stat after induction targetingundetected leukemic cells lasting several months

CONSOLIDATION after remission to eliminate any remainingcells

MAINTENANCE lower doses to keep d body free of leukemic

cellsCHRONIC LYMPHOCYTIC LEUKEMIA (CLL)Proliferation of morphologically normal but functional inert lymphocytesLymphocytes are immunoincompetent and responds poorly to antigenicstimulationIndolent for years with gradual transformation to more malignant diseaseNURSING DIAGNOSES

ACUTE PAIN

ACTIVITY INTOLERANCEHEMOSTASIS

MECHANISMS

Extrinsic initiated by tissue damage and blood loss

Intrinsic mechanism within the blood vessel where blood loss andtissue trauma are not present

THREE PHASES


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FIRST : tissue injury..release of platelet factor..calcium andaccessory factors leads to thromboplastin formation SECOND : conversion of PROTHROMBIN to THROMBIN

THIRD : conversion of FIBRINOGEN to FIBRIN**RBCs are trapped in the fibrin and gives color to the clot

FIBRINOLYSIS

Clots are dissolved by FIBRINOLYSINBLEEDING DISORDERS

THROMBOCYTOPENIA

ATP/ITP

DIC


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CLASS. and ETIOLOGY

Decreased platelet production Increased platelet destruction

Abnormal distribution/sequestration in spleen

Dilutional

c/mNo s/sPlatelets M; asstd with pregnancy, SLE, thyroid

diseaseMGTSupportive care bleeding control, platelet transfusionHigh- dose immunotherapy


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SplenectomyDIC/disseminated intravascular coagulopathy

Acquired thrombotic and hemorrhagic syndrome

Abn activation of the clotting cascade and accelerated hemolysis

Widespread clotting in small BV; consumption of clotting factors andplatelets

Simultaneous bleeding and thrombosis

Etiology/patho

Event: overwhelming infection(bacterial sepsis), OB c/p (abrplacentae, eclampsia, amniotic fluid embolism, retention of dead fetus);massive tissue injury ; vascular and circ collapse

Hemolytic BT rx, snakebite

CAc/m

Abn clotting extremity coolness & mottling; acrocyanosis;dyspnea, abn breath sounds; altered mental status, acute renal failure,pain (ischemia, infarct) Abn bleeding -

Dx/mgt

Treat underlying disorder Replacement tx fresh frozen plasma, platelet

Supportive fluid rep, O2, maintain BP, renal perfusionCOMPLICATIONS

THROMBOEMBOLIC HEMORRHAGIC (CEREBRAL)** the most common cause of deathNURSING DIAGNOSES

RISK FOR INJURY

INEFFECTIVE TISSUE PERFUSION


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NURSING INTERVENTIONS

MINIMIZE BLEEDING PROMOTE TISSUE PERFUSION

OBJ: minimize bleeding

**avoid dislodging clots

Apply pressure to site of bleeding 20 min Cautious use of tape

Bedrest

Internal bleeding = BS, abd girth Evaluate fluid status and bleeding freq VS check, CVP, I and O

OBJ: PROMOTE TISSUE PERFUSIONKeep client warmAvoid vasoconstrictive agentsChange position frequently, perform ROMMonitor ECG and lab tests arrhythmia, ABG, BUN, creatinine

MONITOR s/s vascular occlusion, REPORT STAT

Brain, eyes, bone, pulmonary, extremities, coronary, bowel

SICKLE CELL ANEMIA Severe, chronic, hemolytic anemia

Genetically determined, inherited disease autosomal recessive(nextslide)

CLINICAL COURSE: PAIN -> occlusion of capillaries by sickled RBC

INCIDENCE: AFRICAN-AMERICAN; ARABIC/MEDITERRANEAN ANCESTRY

PARENTS HETEROZYGOUS for HbS

AUTOSOMAL RECESSIVE

A genetic condition that appears only in individuals who have receivedtwo copies of an autosomal(nonsex chromosome)gene, one copy fromeach parent

The parents are carriers who have only one copy of the gene and DONOT EXHIBIT THE TRAIT because the gene is recessive to its normalcounterpart gene.

If both parents are carriers, there is a 25% chance of a child inheritingboth abnormal genes and, consequently, developing the disease. Thereis a 50% chance of a child inheriting only one abnormal gene and ofbeing a carrier, like the parents, and there is a 25% chance of the childinheriting both normal genes.
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CAUSE

inheritance of a gene from the structurally abnormal portion of thehemoglobin chain

characteristics

Hemoglobin A(HbA) is partly/completely replaced by abnormalsickle hemoglobin S(HbS)

**sensitive to changes in the 02 content of the RBC

deficient O2 causes cells to assume the SICKLE shape,become rigid and clumps-> obstructing capillaries

PRECIPITATING FACTORS: sicklingAny condition that increases need for O2 and alters O2 transport

Dehydration

Infection

Trauma Strenuous physical exertion

Cold exposure

Hypoxia

Acidosis Surgery

pregnancy

Sickle cell crisis

1. vaso-occlusive crisis

2. splenic sequestration

3. aplastic crisis Crisis: vaso-occlusive

Blood stasis and clumping in capillaries -> ischemia, infarction

s/s fever; painful swelling of hands & feet(dactylitis), joints; abdominalpain; hepatomegaly; icteric sclera; vomiting; acute back pain; priapism


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**MOST COMMON EVENT in INFANTS and TODDLERS

Crisis: splenic sequestration

Pooling of and clumping of blood (hypersplenism)

Rapid onset

s/s profound anemia; hypovolemia, shock

***fragile rbc..rapid destruction(6-20 days)***frequent cause of death in INFANTS

APLASTIC CRISIS

BONE MARROW ceases RBC PRODUCTION INCREASED DESTRUCTION OF RBC

LOW RETICULOCYTE COUNT

MANAGEMENT:prevention of sickling

Promote adequate oxygenation and hemodilution

Increased fluid intake

AVOID HIGH ALTITUDES and low-O2 environment AVOID physical exertion

AVOID extreme heat or cold Blood transfusion

Interventions IVF normal saline, oral fluids

O2 adm, BT as prescribed

Adm analgesics (RTC) ***Meperidine is AVOIDED (risk ofmeperidine-induced seizures

POSITION: elevate head (< 30deg) extremities extended (to promotevenous return); AVOID strain to joints

Adm antibiotics as ordered to prevent infection

VACCINES H influenzae type B, menincococcusTHALLASEMIA

A group of genetic (inherited) blood disorders

COMMON FEATURE: defective production of hemoglobin

COMMON: Greek and Italian descent

BETA THALLASEMIA decreased production of normal adult hemoglobin (HbA), the

predominant type of hemoglobin from soon after birth until death

HEMOGLOBIN: heme, globin globin has 4 protein sections called polypeptide chains

Two are identical (the alpha chains)

The other two chains are also identical to one another (beta chains)

In persons with beta thalassemia, there is reduced or NOproduction of beta globin chains.

MINOR THALASSEMIA

only one copy of the beta thalassemia gene (together with one perfectlynormal beta-chain gene).


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The person is said to be HETEROZYGOUS for beta thalassemia

MAJOR THALASSEMIA/COOLEYS ANEMIA

two genes for beta thalassemia and no normal beta-chain gene

The child is homozygous for beta thalassemia.

A striking deficiency in beta chain production and in the production of

Hb a serious disease

CHARACTERISTICS

Anemia begins: within the first months after birth; becomesprogressively more and more severe

S/S failure to thrive

Feeding problems(easy fatigue from lack of oxygen, with the profoundanemia)

bouts of fever ( predisposed to infection)

diarrhea and other intestinal problems.

pale skin, irritability, growth retardation

Abdominal swelling (hepatosplenomegaly)

jaundice (hemolytic anemia)

TREATMENT

BLOOD TRANSFUSION

FOLIC ACID

GENE THERAPYHEMOPHILIA

INHERITED, congenital bleeding disorder

SEX-LINKED, RECESSIVE TRAIT caused by gene carried on their Xchromosome

Affect males; CARRIERS: FEMALES

Most common transmission: union of an unaffected male with a trait-carrier female


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PATHOPHYSIOLOGY and ETIOLOGY

LACK OF FACTORS VIII (hemophilia A/ classic hemophilia) andIX(hemophilia B/Christmas hemophilia)..defect in intrinsic phase ofcoagulation process

Unstable blood clot Platelet number and functions are normal

CLINICAL MANIFESTATIONS

Diagnosed after the child becomes active

Severity varies***depends on plasma level of the coagulation factorinvolved

Hx of prolonged bleeding after circumcision, easy bruising,spontaneous hematomas, hemarthrosis, hematuria, GI bleed

DIAGNOSTIC EVALUATION

Protime and BT normal PTT prolonged

Prothrombin consumption decreased

Thromboplastin increased

Gene analysis to detect carrier state, for prenatal diagnosis

MANAGEMENT

PROMPT, appropriate treatment**KEY

REPLACEMENT OF type-specific coagulation concentrates duringbleeding episodes

Supportive therapy : NSAIDS, PT

COMPLICATIONS

AIRWAY obstruction Repeated hemorrhage ->degenerative joint changes

Intestinal obstructions

Nerve compression - > paralysis


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Intracranial hemorrhage

NURSING PLANS AND INTERVENTIONS

Prevent hypovolemia through bleeding control

Provide protection against bleeding

Preserve mobility

Relieving pain Enhancing family coping

Bleeding control

Apply cold and pressure for 10-15 min to site ofinjection/venipuncture

NO SUTURING AND CAUTERIZATION

Immobilize and elevate above heart level Adm coagulation concentrates as ordered

AVOID RAPID adm to reduce transfusion reaction; 2-3ml/min

STOP transfusion if with hives, tingling, chills, fever, flushing

Bleeding protection

AVOID rectal temp AVOID injections if possible

NO ASA and ASA products

Safety p/c pad cribs/siderails; inspect toys; protection devices;remove environmental hazards

NO hard candy, suckers, candy canes, straw, sharp eating-utensils

Preserve mobility

Treat bleeds stat

Supportive care to bleeding joints: Immobilize(slight flexion);elevate; ice packs followed by heat

Gentle passive exercise 48 h after acute hemarthrosis

Physical therapy Pain relief

increasing pain means that bleeding continues

NSAIDs during acute phase

Opioids sparingly


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hematologic disorder

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