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Management of Clients with Hematologic Disorders
NRS 108
Majuvy L. Sulse RN, MSN, CCRN
HEMATOLOGIC DISORDERS
Disorders associated with ErythrocytesDisorders of bleedingDisorders associated with white blood
cellsLymphomas
ANEMIA(decreased in number of RBCs)
Low Hgb
Less 02 carrying capacity
Hypoxia
H/H, Bone marrow aspiration, Peripheral smear
( Diagnosis)
Abnormal & deficient production, blood loss, destruction of RBC
( Causes)
Pallor, Fatigue, Palpitation, Low BP, SOB, DOE, MI, Renal failure
Management of Anemia
Medical: Identify cause Treat cause Relieve
symptoms Prevent
complications
Nursing: Assess Educate
Classification of Anemia
Based on the size of the RBCNormocyticMicrocyticMacrocytic
Iron Deficiency Anemia
Causes: Inadequate iron supply Chronic blood loss without iron replacement Decreased iron absorption in the intestines
Signs/ Symptoms: Hypochromic (low MCH), microcytic ( low MCV) Elevated serum binding capacity Brittle, spoon-shaped nails with longitudinal ridges Cheilosis (painful mouth cracks/ sores) Red shiny tongue Insidious development of fatigue
Diagnosis/Treatment
Treatment: Identify cause
Diagnosis: Laboratory values Gastroscopy Sigmoidoscopy Occult blood in stools Radiographic studies
of GI Iron supplement Nutritional/dietary
Megaloblastic Anemia
Predominance of megaloblasts & lack of normoblasts Includes pernicious anemia, Vit. B12 & Folic acid
deficiencies Related to surgery particularly of terminal ileum where
B12 is absorbed; vegetarian diet; prolonged exposure to nitrous oxide
Related to aging & long term gastritis Related to alcohol malnutrition & malabsorption (Folic
acid deficiency)Macrocytic, normochromic RBC
Lack of Intrinsic factor Autoimmune response
Diagnosis & Treatment: (Megaloblastic Anemia)
Schilling test- definitive dx of pernicious anemia
Gastric secretion analysis- pH, free HCL, low gastric secretion
Diagnosis & Treatment :(Megaloblastic Anemia)
Management- Lifelong tx with Vit B12 injection Iron & Folic acid supplement Nutritional/ dietary changes
Foods that are rich in folic acid and vitamin B12 include the following:
eggs meat poultry milk shellfish fortified cereals
Aplastic Anemia
Low Hb & pancytopenia Unknown etiology / autoimmune disturbance Direct injury by Myelotoxins- agents causing bone marrow
damage when received in large doses Medication induced:
Chemo, chloramphenicol, sulfonamides, mephenytoin, quinine
Exposure to environmental hazards: Benzene, insecticides, radiation & radioactive
materials Infections
Hepatitis, miliary TB, EPBV Congenital/hereditary causes
Aplastic Anemia-Manifestations & Dx
Manifestations- Exertional dyspnea, fatigue, pallor, infections Bleeding (nasal,oral, rectal,vaginal) ecchymosis,
petechiae, pupura Low platelets (less 30, 000), RBCs, WBCs Dry bone marrow on aspiration
Diagnosis- Differential count Manifestations History of exposure to myelotoxins
Aplastic Anemia
Medical Remove causative
Idiopathic cause- treat with steroids, hormone therapy
Autoimmune cause- bone marrow transplants (younger than 30 or those who have not received transfusions yet
Antithymocyte & cyclosporine therapy- skin testing & watch for allergic reactions
Blood transfusions Frequent CBCs esp for those on radiation therapy
Nursing management Infection control Client education
Anemia from Blood Loss
Types Acute-trauma, complications from surgery
Hypovolemia, hypotension, hypoxemia, weakness, tachycardia, stupor
Chronic- bleeding ulcer, hemorrhoidsGradual and vague symptoms as fatigue, pallor,
dyspnea Medical/ Nursing Interventions
Identify source of bleeding Control through medical or surgical interventions Blood transfusions
Hemolytic Anemia
Abnormal destruction of RBCs by Intrinsic-defective RBCs, enzyme deficit(G6PD) extrinsic factors- toxins, injury as in prosthetic heart
valves Failure of bone marrow to replace destroyed RBCs Sites of hemolysis:
Intravascular-circulation Extravascular- macrophages of spleen, liver & bone
marrow
Findings/ Treatment
Findings Normocytic
anemia Reticulocytosis as
compensatory mechanism
Increased RBC fragility
Short lifespan Hyperbilirubinemia
- blood, urine, stools
S/S as in Anemia
Treatment Identify/Treat
cause IV fluids to flush
kidneys NAHCO3 or Na
Lactate to alter urine pH (decrease) precipitation in renal tubules
Splenectomy
Sickle Cell Anemia and Sickle Cell Trait
Inherited, autosomal, recessive disorders of HB synthesis resulting in decrease O2 to the tissues (hypoxia) and obstruction of blood vessels
Substitution of valine for glutamic acid in B-globin gene
Primarily seen in the black population Sickle cell trait is a mild form of the disease and it is
the commonest Sickle cell trait is prevalent in Africa Resistant to the parasite that causes malaria Genetically inherited from each parent
Inheritance
Pathophysiology
Sickling of RBC-triggered by Hypoxia- low O2 tension in blood
High altitude Emotional/ physical
stress Surgery Blood loss Infection-bacterial & viral
Dehydration Acidosis Decreased plasma volume-
Increased blood viscosity Hypothermia Stress
Pathophysiology
Sickled cell-rigid & elongated causes tissue injury (as cells can’t pass through small vessels) results in local hypoxia anemia as more cells are hemolyzed by spleen
Initially reversible with re-oxygenation then becomes irreversible due to cell membrane damage from recurrent sickling
Sickle Cell crisis
Exacerbation of RBC sickling Vaso- occlusion (Vaso-occlusive crises) -
vasospasm Changes in membrane permeability plasma loss
& hemoconcentration development of thrombi, tissue ischemia, infarction, necrosis
Shock is a life threatening consequence Frequency, extent, & severity of episode is
dependent on percentage of HbS present
Causes of Sickle Cell Crisis
Vaso - OcclusionAplastic crisisHemolytic crisisSequestration crisisMixed crisis
Types of Sickle Cell Disease
Sickle cell anemiaSickle cell ThalassemiaSickle cell HbC-
Clinical Manifestations
Cardiovascular changesSkin changesAbdominal changesMusculoskeletal changesCentral nervous system changes
Clinical Manifestation
Noticed after 6 months when fetal Hb is no longer present
Improper growth related to anemia Retarded growth and delay in sexual maturity Hand- foot syndrome- edema of hands and
feet Pain from tissue ischemia-hands, feet , joints
Clinical Manifestation
Weakness and fatigue-exercise intolerance
Jaundice- development of gallstonesPallor-of mucous membrane-grayish
cast on skinPriapism-occlusion of penile veinsInfarct on the spleen - small and scarredLeg ulcers in about 75% of cases
Complications
CHF from ischemia & heart enlargement
Acute Chest syndrome-fever, chest pains, cough, dyspnea
Pulmonary infarct- pulmonary HTN, MI, Cor Pulmonale
Blindness from retinal obstruction-hemmorhage-detachment
Renal failure- hemoconcentration
Autosplenectomy-shrinking of spleen from repetitious scarring
CVA- thrombus formation
Osteoporosis/ Osteosclerosis
Leg ulcers esp. at ankles-tissue hypoxia
Infection- absence of phagocytic activity by spleen
Diagnostic findings
Peripheral SmearSickling TestDNAElevated serum bilirubin levelsSkeletal xrays-reveal bone/joint
deformities & flatteningMRI-check for clots (CVA)
Medical/ Nursing Management
No cureSupportive interventions: pain relief,Hydration, O2, rest, blood transfusionsPatient Teaching to avoid factors that cause
crisisAssessment of family understanding of the
disease & coping mechanismLinks for supportGenetic counseling
Get Involved Class!
What nursing diagnosis should receive the highest priority in a client with sickle cell crisis?
A nurse is preparing a teaching plan for a sickle cell client, what should the nurse emphasize on to prevent sickle cell crisis?
Polycythemia Vera
Excessive production of erythrocytes, leukocytes, platelets due to excessive activation of pluripotent stem cells
ManifestationsHTN- headache, vertigo, tinnitus, dizziness,
visual disturbancesCHF- angina- hypervolemia & viscosity Intermittent claudication- thrombophlebitis
Polycythemia Vera
Stroke- thrombi formationPruritus- histamine release from basophilsHemmorrhage- vessel rupture from tissue
distentionHepatomegaly & Slenomegaly from organ
engorgementPlethora- Ruddy complexionGout—hyper uric acid production from RBC
destruction
Diagnosis
RBC=8-12 millionHgb=18-25 gmIncreased platelet countHCT= >54% in men; 49% in womenElevated WBCs with basophiliaIncreased serum uric acid, B12SplenomegalyHyperplastic bone marrow
Medical/Nursing Management
Goal of treatment isReduce blood volume & viscosity
Phlebotomy to Hct levels of 45-48% (about 300-500ml/day)
Reduce bone marrow activity- myelosuppressive agents & radioactive phosphorous
Hydration therapy with I & O
DISORDERS OF BLEEDING- THROMBOCYTOPENIA
Decreased production of platelets below 150,000/uL
Manifests as bleeding- skin bruises easily Maybe
acquired –food, drugs, infections, aplastic anemia
inherited- pancytopenia, hereditary thrombocytopenia
Diagnosis/ Management
CBC shows low platelet & Hb count Assess for hx of NSAIDS Avoid injury Good oral hygiene & skin care Rectal enemas & suppository-avoid constipation Avoid IM, SC injections, rectal temperatures Apply pressure on any bleeding source Monitor signs of bleeding
Immune Thrombocytopenia Purpura- ( ITP)
Autoimmune bleeding disorder Platelets coated with antibodies Destroyed by macrophages in liver & spleen Survival is 1-3 days instead of 8-10 Gradual onset & transient remissions occur
Clinical Manifestations
Petecchiae- small, flat, pinpoint red Purpura-numerous petecchiae Ecchymosis-large purplish lesions Epistaxis- Bleeding gums Heavy menses Complication=hemorrhage
Diagnosis
Platelet Ct<100, 000Prolonged bleeding time with normal
coagulation timeIncreased capillary fragilityPositive platelet antibody testBone marrow aspirate contains normal
or increased megakaryocytes
Management
Cortecosteroids (prednisone)-suppreses the phagocytic response of Spleenic macrophages, depress antibody formation, reduce capillary permeability & bleeding time
IV immunoglobulin (IVIG) Immunosuppressive therapy Splenectomy- Platelet transfusions
Hemophilia
Characterized by prolonged bleeding after surgical/ dental or small trauma or cuts
Types Hemophilia A- factor Vlll-most common-80% cases Hemophilia B- factor lX deficiency-inherited gene Von Willebrand’s disease- deficient Vlll & platelet
dysfunction
Etiology
Sex linked genetic disorderTransmitted by females but males
express the disorderCarriers transmit the gene to half their
daughters and the disorder to half their sons
Males transmit the gene to all their daughters but none to their sons
Clinical Manifestations
Slow persistent bleeding from cuts/scratches Delayed hemorrhage-hours/days after injury Severe bleeding after dental surgery GI bleed Nosebleed Hematoma Prolonged APTT
Treatment
Goal is to stop bleedingIncrease anti-hemophilic factor-give
factor Vlll, lXPrevent complicationsSupport therapy
LEUKEMIA
DESCRIPTION Malignant exacerbation in the number of
leukocytes, usually at an immature stage, in the bone marrow
May be acute, with a sudden onset and short duration, or chronic, with a slow onset and persistent symptoms over a period of years
Affects the bone marrow, causing anemia, leukopenia, the production of immature cells, thrombocytopenia, and a decline in immunity
LEUKEMIA
DESCRIPTIONThe cause is unknown and appears to
involve gene damage of cells leading to the transformation of cells from a normal state to a malignant state
Risk factors include genetic, viral, immunological, and environmental factors and exposure to radiation, chemicals, and medications
CLASSIFICATION OF LEUKEMIA
ACUTE LYMPHOCYTIC LEUKEMIA (ALL) Mostly lymphoblasts present in bone marrow Age of onset is less than 15 years CNS manifestation common-leukemic meningitis Normally, the lymphocytes fight infection by making
antibodies that attack harmful elements. But, in ALL, the cells are immature and overabundant. They crowd out other blood cells, and may collect in the blood, bone marrow, and lymph tissue.
CLASSIFICATION OF LEUKEMIA
ACUTE MYELOGENOUS LEUKEMIA (AML) affects the young blood cells (called blasts) that
develop into a type of white blood cell (called granulocytes). The main function of granulocytes is to destroy bacteria. The blasts, which do not mature and become too numerous, remain in the bone marrow and blood.
Age of onset is between 15 and 39 years
Classification
CHRONIC MYELOGENOUS LEUKEMIA (CML)Mostly granulocytes present in bone marrowAge of onset is after 50 years of ageCML occurs mainly in adults and is rare in
children CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
Mostly lymphocytes (B cells) present in bone marrow
Age of onset is after 50 years of age,
Diagnosis
CBC-WBC may be normal, decreased or elevated
Decreased plateletDecreased HbBone marrow aspiration- increase in
marrow cells
LEUKEMIA
ASSESSMENTAnorexia, fatigue, weakness, weight lossAnemiaBleeding (nosebleeds, gum bleeding, rectal
bleeding, hematuria, increased menstrual flow)
PetechiaeProlonged bleeding after minor abrasions or
lacerations
Assessment
Elevated temperatureLymphadenopathy and splenomegalyPalpitations, tachycardia, orthostatic
hypotensionPallor and dyspnea on exertionHeadacheBone pain and joint swelling
Leukemia
ASSESSMENTNormal, elevated, or reduced white blood
cell (WBC) countDecreased hemoglobin and hematocrit
levelsDecreased platelet countPositive bone marrow biopsy identifying
leukemic blast phase cells
Leukemia
INFECTIONA major cause of death in the
immunosuppressed clientCan occur through autocontamination or
cross-contaminationCommon sites of infection are the skin,
respiratory tract, and gastrointestinal (GI) tract
Leukemia
IMPLEMENTATION: INFECTION
Initiate protective isolation procedure-also called Neutropenic Precautions or Reverse isolation
Ensure frequent and thorough hand washing Reduce exposure to environmental organisms by
eliminating raw fruits and vegetables (low-bacteria) from the diet, fresh flowers from the client’s room, and by not leaving standing water in the client’s room
Avoid invasive procedures such as injections, rectal temperatures, and urinary catheterization
Leukemia
CLIENT EDUCATION: INFECTION Avoid crowds and those with infections Consume a low-bacteria diet and to avoid drinking
water that has been standing for longer than 15 minutes
Avoid activities that expose the client to infection such as changing a pet’s litterbox or working with houseplants or in the garden
Neither the client nor their household contacts should receive immunization with a live virus
Leukemia
BLEEDING During the period of greatest bone marrow
suppression (the nadir), the platelet count may be extremely low, less than 10,000/mm3 (bleeding risk)
Examine the client for signs and symptoms of bleeding; examine all body fluids and excrement for the presence of blood
Handle the client gently; use caution when taking blood pressures to prevent skin injury
Measure abdominal girth, which can provide an indication of internal hemorrhage
Provide safety-pad side rails and sharp corners of the bed and furniture
Leukemia
IMPLEMENTATION: BLEEDING Provide soft foods that are cool to warm in
temperature Avoid injections if possible to prevent trauma to the
skin and bleeding; apply firm, gentle pressure to a needle stick site for at least 10 minutes
Avoid rectal suppositories, enemas, and thermometers
If the female client is menstruating, count the number of pads or tampons used
Administer blood products as prescribed
Leukemia
FATIGUE AND NUTRITION Assist the client in selecting a well-balanced diet Provide small, frequent meals (high calorie, high-
protein, high-carbohydrate) that require little chewing
Assist the client in self-care and mobility activities Allow adequate rest periods during care; do not
perform activities unless they are essential Administer blood products for anemia as prescribed
Leukemia
CHEMOTHERAPY Induction therapy: Aimed at achieving a rapid,
complete remission of all manifestations of the disease
Consolidation therapy: Administered early in remission with the aim of cure
Maintenance therapy: May be prescribed for months or years following successful induction and consolidation therapy; the aim is to maintain remission
Leukemia
IMPLEMENTATIONAdminister antibiotic, antibacterial, antiviral,
and antifungal medications Blood replacements as neededPrepare the client for transplantationAdminister colony-stimulating factors as
prescribedMaintain infection and bleeding precautions
Leukemia
IMPLEMENTATIONProvide an adequate dietProvide an activity schedule that will
conserve energy Instruct the client in appropriate home care
measuresProvide psychosocial support and support
services for home care
Leukemias
AcuteCombination ChemoInductionRest- 3 weeksConsolidationMaintenace follows remission- lasts 2-3 yearsBone marrow transplant
ChronicTreatment based on symptomsRadiation to spleen, nodesInterferonAutologous stem cell transplantBlood transfusions: RBCs, platelets, WBCs
HODGKIN’S DISEASE
DESCRIPTION A malignancy of the lymph nodes that originates in
a single lymph node or a single chain of nodes; metastasis occurs to other adjacent lymph structures and eventually invades nonlymphoid tissue
Usually involves lymph nodes, tonsils, spleen, and bone marrow and is characterized by the presence of the Reed-Sternberg cell in the nodes
Possible causes include viral infections and previous exposure to alkalyting chemical agents
Prognosis is dependent on the stage of the disease
Etiology & Pathophysiology
Unknown cause Linked to Epstein-Barr Virus Genetics- high among Jews Mechanism of growth & spread- unknown Cancerous cells transforms in lymph and
progresses to other nodes or direct infiltration of blood vessels
Clinical Manifestations
Enlargement of cervical, axillary, inguinal lymph nodes may be painless
B symptoms-Weight loss, Fever, night sweats Cough, stridor, dyspnea Hepatomegaly Splenomegaly SVC syndrome from intra thoracic involvement
Diagnosis & Staging
Peripheral blood analysis-microcytic, hypochromic anemia, leukocytosis
Lymph node biopsy-definitive dxBone marrow examination-staging( l-lV)CXR, CT Scan, radioisotope studies-
define sites Lymphangiography-assess nodes &
vessels
Hodgkins Disease
IMPLEMENTATIONFor stage 1 and 2 without mediastinal node
involvement, the treatment of choice is extensive external radiation of the involved lymph node regions
With more extensive disease, radiation along with multi agent chemotherapy is utilized
HODGKIN’S DISEASE
Implementation Monitor for side effects related to chemo or radiation Monitor for signs of infection & bleeding Maintain infection and bleeding precautions Discuss possibility of sterility with male client
receiving radiation & inform of options related to sperm banks
Pain management Client & family support If terminal- ensure client has dignified death
NHLNON- HODGKIN’S LYPHOMA
Group of malignancies with origins in lymphoid cells affecting all ages
Most common occurring cancer & 5th leading cancer causing death
Men are more affected than women; survival better for women than in men
Incidence higher in whites than other races Painless lymph node enlargement is primary
manifestation Symptoms will present on where disease has
spread
Diagnosis & Treatment
Diagnostic tests are same as for Hodgkin’s disease-Lymph node biopsy done
Treatment-radiation & chemotherapy Nursing assessment for nodes characteristics,
B symptoms appearance Support for client & family Manage risk for infection, bleeding, sexual &
reproductive dysfunction Nutritional management
Multiple Myeloma
Cancer of the plasma cell, an important part of the immune system that produces immunoglobulins (antibodies) to help fight infection and disease.
Hypercalcemia, anemia, renal damage, increased susceptibility to bacterial infection, and impaired production of normal immunoglobulin are common clinical manifestations of multiple myeloma. It is often also characterized by diffuse osteoporosis, usually in the pelvis, spine, ribs, and skull.
Diagnostics/ Management
Diagnostics Radiologic studies- lesions in bones,
demineralization, osteoporosis Bone marrow biopsy- immature plasma
cells Abnormal immunoglobulin Monoclonal immunoglobulin chains in
blood & urine exam
Multiple Myeloma
ManagementChemotherapy, radiation txAutologous bone marrow transplantPlasmapheresisHydration, diuretics, phosphate for treatment
of HypercalcemiaPain managementSupportive for anemia, leukopenia,
thrombocytopenia