Hemotologic DisordersBy Diana Blum RN MSN

Metropolitan Community CollegeNURS 2140

Assessment

Should be subjectiveShould be objective

Anatomy

Blood is a type of connective tissue◦Transports nutrition ◦Transports oxygen◦Transports hormones

Immunological aspects also important◦Critical to body defense

◦Maintains temperature◦Controls PH◦Removes toxins◦Regulates electrolytes

Hematopoiesis:

Bone Marrow:

Stem Cell:

Cell Types (page 1991-94)

Plasma:

Leukocyte:

Reticulocyte:

WBC:

RBC:

Erythrocyte:

Platelet:

Lymphatic system

Responsible for removing excess fluid protein and large particles from cells

Main functions: ◦fluid balance maintenance◦Defense◦Fat absorber

Spleen

Stores 20-40 ml bloodRemoves old cellsFilters and destroys antigens before they

enter systemStores platelets (1/3 of body’s store)

Hemostasis (page 1995)

Stopping of blood flow2 phases:

◦Primary◦Secondary

Critical thinking here

Be able to explain tests to ptsBe able to explain why tests being doneKnow normal from abnormalBe good communicator with physicianBe able to consider anticipated actions

◦Ex. When blood transfusion needed

Anemia (page 2008)

Hemoglobin is lowOxygen is low Not a disease but an underlying condition2 types: hypoproliferative and hemolytic

◦Hypoproliferative: marrow can’t produce adequate erythrocytes Lack of factors, chemicals, marrow damage

◦Hemolytic: premature destruction of erythrocytes. Leads to tissue hypoxia Abnormality like sickle cell, direct injury

Erythropoiesis

Stimulation of RBCs by erythropoietin

Erythropoietin: hormone produced by kidneys during periods of hypoxia to ensure growing tissues will have sufficient oxygen.

Determination between types

Based on:◦Marrow ability to respond to decreased

erythrocytes ◦Manner of maturity for young erythrocytes◦The presence or absence of end products of

erythrocyte destruction within the circulation

Clinical manifestations (pg 2010)

Rapidity with the anemia has developedDuration of anemiaMetabolic requirements of patient Other disorders

Cardio: tachycardia, hypoxia, MI symptomsRespiratory: SOB in varying degreesNeuro: headache, poor concentration, vertigo,

irritibility, confusion

Compensation also may occur

Education

Prioritize activitiesPlan rest periodsEats healthy foods rich in iron, vitamins, and

proteinsUse nutrition supplements as neededReport palpitations, dizziness, dyspneaMonitor vitalsMonitor edemaMonitor neuro statusSafety techniques

Low RBC Low HGB andHCT Low serum Iron level Low ferritin level High TIBC level Results from diet low in iron Results from body not absorbing enough iron from GI tract

◦ Not enough hemoglobin made as result s/s: sore tongue, brittle nails, fatigue, pallor, orthostatic changes (in

severe cases) Dx: bone marrow aspiration, lab work Tx: iron supplements, iron rich foods (liver, oysters, red meat, fish,

dried fruits, legumes, dark green veggies, whole grain breads and cereal)---orange juice or other vitamin C product will enhance iron absorption◦ What is the major side effect of IV iron?

Iron Deficiency Anemia

Anemia and Renal Disease

Pts don’t become severely anemic til creatinine levels exceed 3mg/100ml

Hematocrit decreasesCauses:

◦shorted RBC lifespan◦Deficiency of erythropoietin◦Long term hemodialysis

Tx: monitor serum levels of iron, hematocrit, and vitals

Anemia and chronic disease

Only inflammation, infection, and malignancy cause

It is nonprogressiveDevelops over 6-8 weeksMany don’t require treatment

Most cases are idiopathic Infection and pregnancy, medication, chemicals or radiation

can trigger◦ Airplane glue, arsenic, pestisides

Complete failure of the bone marrow Low RBC count Low WBC Low Platelet cts S/S: pallor, fatigue, tachycardia, sob, hypotension,

prolonged/spontaneous bleed, frequent infections, purpura, retinal hemorrhages are common

Tx: transfusion of RBC and Platelet, antibiotics, corticosteroids, bone marrow transplant, ICU setting, monitor for signs and symptoms of infection and bleeding, neutropenic precautions

Aplastic Anemia

Pt does not absorb vitamin B12 from stomach◦ Higher incidence of gastric cancer

Pt may lack intrinsic factor-essential for b12 absorption Assess hx: gastrectomy, crohn’s, family history,etc. s/s: weakness, sore tongue, numbness of hand and

feet Tx: B12 injections monthly, endoscopy q1-2 years---if

untreated can develop heart failure and lead to death◦ Monitor oral cavity, and skin. Monitor for jaundice, monitor

gait, provide small frequent bland diet Dx: Obtain shilling test

Pernicious Anemia

Thalassemia

Most frequent in SE Asian, African, and Mediterranean descent

Folic Acid Deficiency

Folate is in green leafy veggies and liverAlcohol increases need for folic acidS/S same as pernicious anemia except no

neuro symptomsDx: folate levelTx: replacements

RBCs normally disc shaped In sickle cell they are sickle shapedEasily ruptureObstruct blood flowGenetic in natureMost common in African AmericansSickle cell is recessive (inherit from mom

and dad)

Sickle cell anemia

Sickle Cell Crisis

3 types: ◦sickle crisis: most common---obstructive◦ aplasic crisis---from infection from parvovirus◦ sequestrian crisis---result from other organs pooling

sickled cells Spleen is most common Also in liver and lungs

S/S: tachycardia, fever, decreased HGB, infiltrates on chest x-ray

Pulomary HTN is a symptom that is not usually detected until damage is irreversible

Monitor x-rays, CT scan, Echo, HGB levelTx: treat symptoms, Bone marrow transplant,

transfusions, Hydroxyurea, Arginine

Glucose -6-Phospate Dehydrogenase Deficiency

This is a deficiency in the gene that is essential for membrane stability

Hemolysis only occurs with stressful situationsMostly affects african americans, greek or italian

decent as well as asians and jewish origins Inherited on x chromosomeS/S: asymptomatic, or jaundice, pallor,

hemoglobinuriaDx: serum G-6-PDTx: stop offending med, transfusion, educate about

meds to avoid, instruct to wear medic alert bracelet

Enough RBCs made but they are destroyed once they are released into circulation

Causes: infection, drug reaction, cancers/s: pallor, fatigue, tachycardia, sob,

hypotension, jaundice, high bilirubin levelsPositive direct coombs antiglobulin testTx: blood transfusions, corticosteroids,

spleenectomyRecovery in few days to weeks

Autoimmune Hemolytic Anemia

Too many RBCs produced◦ Blood more viscous

s/s: headache, dizziness, ringing in the ears, blurred vision, ruddy complexion, HTN, pruritis, gout problems, burning fingers and toes

DX: o2 sat, CBC with DIFF Complications: CVA, MI, BLEED issues Tx: hydroxyurea, interferon, plavix, ASA Treat symptoms Teach pt: don’t cross legs, quit smoking, stay active, minimal

alcohol, avoid excess iron phlebotomize blood

Use 18 gauge needle In antecubital vein and remove I unit of blood

Polycythemia Vera

Leukemia

Defect in the WBC stem cellAbnormal reproductionsUnknown cause, may be from chemical or

radiation exposureSymptom onset is abrupt

AMLAcute Myeloid Leukemia

Defective stem cell Incidence rises with age 60+Prognosis variableYounger pts may survive 5 yearsS/S: fever, infection, weakness, fatigue, bleed

tendency, painDx: no warning, CBCComplications: bleeding (petechiae, ecchymosis),

infection, DICTX: chemo, transfusions, infection treatment, growth

factor agents, bone marrow transplant, palliative care

CML

Uncommon in pts under 20Life expectancy 3-5 yearss/s: asymptomatic, dyspnea, mild

confusion, enlarged liver, wt loss, anorexiaTx: tyrosine kinase inhibitor, avoid

antacids and grapefruit juice, interferon, cytosine, leukopheresis, bone marrow transplant

Acute Lymphocytic LeukemiaALL

Most common in young kids 4-14Uncommons/s: decreased cbc counts, pain, enlarged

liver, enlarged spleen, headache, vomitingTx: bone marrow transplant, chemo,

corticosteroidsComplications: infection

CLL

Common in older adultsMost common form in US and Europe More frequent in mens/s: asymptomatic, lymphadenopathy,

pain, hepatomegaly, spleenomegaly, anemia, fevers, drenching sweats, wt loss, infections

Tx: treat symptoms, chemo, monoclonal antibodies

Hodgkins Disease

Characterized by reed- sternberg cells in the lymph nodes

Highest occurance is in 20s and50sMen are more likely than women to haveTx: radiation, chemo, bone marrow

transplant, stem cell transplantSurvival rates vary5 yr survival rate is 82%

Non Hodgkins Lymphoma

6th most common type of cancer and cancer death in US◦Avg age of dx is 60

Stages◦Low grade◦ Intermediate grade◦High grade

The higher the grade the more aggressiveTx: chemo, radiation, bone marrow transplant,

stem cell transplant5 year survival rate is 52%

Multiple Myeloma

Cancer of the plasma cellsMost common over the age of 60No known cause

◦Genetics and radiation exposure play a parts/s: bone pain, hyperuricemia (kidneys), anemia,

hypercalcemia, fractures, spinal cord compression, renal failure

Diagnosis: radiographs, serum and urine protein electrophoresis, bone marrow biopsy

No known cureTx: chemo and radiation to treat symptoms,

vertebroplasty, antiemetics, pain management

Bleeding disorders

Clotting Cascade

http://www.mhhe.com/biosci/esp/2002_general/Esp/folder_structure/tr/m1/s7/trm1s7_3.htm

Too few platelets◦Causes: cancer treatment

Too many platelets being destroyed◦Causes: idiopathic thrombocytopenic purpura,

thrombic thrombocytopenic purpuraDx: bone marrow bx, CBC s/s: petechiae, purpura, gingival bleed,

epistaxis, prolonged bleeding Tx: transfusions, spleenectomy, chemo, stop

med that is causing, stop ETOH, treat infection

Thrombocytopenia

Immune thrombocytopenic purpura

Most common among kids and young girlsUsually 1-6 wks after viral infectionCause unknown, h. pylori may play roles/s: asymptomatic, low platelet count, petechiae,

hemoptysisDx: bone marrow bx, platelet ctTx: stop med, immune globulin, chemo,Nsg: assess lifestyle, teach symptoms, avoid

constipation, avoid valsalva, avoid teeth flossing, use only electric razors, use only soft bristled tooth brush, monitor for osteoporosis

HIT

Example of drug induced immune mediated thrombocytopenia

RareRelated to heparin therapyTx: observation, assessment, monitor lab

values

Von Willebrand’s Disease

Most common inherited bleeding disorder1-2% international populations/s: Mucosal and cutaneous bleeding,

porlonged bleed times

Tx: DDAVP, FFP, Cryoprecipitate, bleed precautions

Thrombic Thrombocytopenic Purpura

GeneticLack of blood clotting factor1-2 cases per 20,000 personsTypes

◦A: factor VIII is missing-higher incidence◦B: factor IX is missing

Trait is carried on x chromosomeRare for women to haves/s: uncontrolled bleed especially in joints, skin, GI

tractTx: no cure. Transfusions, pain treatment (IV

morphine is common)..monitor for addiction to opiods.

Hemophilia

DIC

Not a diseaseTriggers: sepsis, trauma, cancer, shock, abruptio

placentae, allergic reactionsLife threatening conditionInitially coag time is normalS/S: are manifested in organs with either clots or

bleeding. Bleeding from all orifaces(tear ducts, gums, IV sites, rectum, urethra, etc.)

Dx: PT, PTT, D-DimerTx: treat the cause, replace fluids, o2, fix electrolyte

imbalances, administering vasopressors are vital, cryoprecipitate to replace factor 5 and 7, FFP, heparin

Universal donor= O-◦Does not contain A, B, or Rh antigens

Universal recipients= AB+◦Blood contains A, B, and RH antigens

Usually blood banks exactly match the pt blood

Blood typing for transfusion

Pt needs 18 or 20 gauge IV needle so cells are not lysed (destroyed)

Prior to administration, blood needs to be checked by 2 licensed nurses. Check the expiration date, name, medical record number, type of blood, blood band id, pt birthday◦Check vitals prior to administration

**blood must be initiated with in 30 minutes of arrival from lab to floor

Use blood tubing for administrationMonitor for blood reactionsMonitor vitals continuously during administration

adminstration on blood

Hemolytic: fever, chills, nausea, dyspnea, chest pain, back pain, hypotension◦ Antigen/antibody rx to transfusion◦ Happens shortly after initiation◦ Tx: stop the transfusion, call md, supportive therapy to maintain HR and BP

Anaphylactic: urticartia, wheezing, dyspnea, hypotension◦ Type 1 hypersensitivity rx to plasma proteins◦ Occurs within 30 minutes of initiation◦ Tx: stop transfusion, call md, be ready for epi and steroids

Febrile: fever, chills◦ Recipients antibodies rx to donor leukocyte◦ Occurs within 30-90minutes of initiation◦ Tx: stop infusion, call md

Circulatory overload: cough, frothy sputum, cyanosis, decreased BP◦ cardio system is unable to manage the additional fluid load◦ Occurs anytime during transfusion and up to several hours after completion◦ Tx: stop infusion, call for help, be prepared for code, be prepared to administer oxygen and Lasix

Blood reactions

Minimize the # of invasive procedureAvoid prolonged tourniquet useAvoid IM injections Instruct the client to use soft bristled

tooth brushNo strait edge razor shaving only use

electric razorAvoid NSAIDS

Bleeding precautions

Risk for injury r/t bleed. Goal: cessation of bleeding aeb no visual signs of bleeding and stable vs

Acute pain r/t bleeding into closed spaces(creating pressure on nerves. Goal: pain relief aeb: patient states pain is relieved and appears to be in relaxed manner.

Ineffective therapeutic regimen management r/t lack of knowledge about dx process and self care. Goal: effective management of condition aeb patient accurately describes condition and demonstrates self care measures.

Nrsg diagnosis

ANY QUESTIONS???