Heme Disorders

Front 1

decreased WBC count

Back 1

leukopenia

Front 2

decreased granulocyte count.

Back 2

granulocytopenia

Front 3

decreased neutrophil count.
is determined clinically by the absolute neutrophil count (ANC).
ANC is calculated from the WBC and Diff
ANC = WBC x (% segs + % bands)
Diagnosis – WBC count
Neutrophil count < 1000 – 1500/ųl

Back 3

neutropenia

Front 4

leukocytes

Back 4

White blood cells

Front 5

lymphocytes 20-25% and monocytes 3-8%

Back 5

agranular

Front 6

basophils- .5-1%
neutrophils 60-70%
eosinophils 2-4%

Back 6

granular

Front 7

inflammatory response not so responsive!

Watch for c/o
Sore throat
Dysphagia
Nonproductive cough
Lesions
Diarrhea
Perirectal discomfort

Back 7

neutropenia s/s

Front 8

Why & What
Antibiotic therapy
Growth factor improvement
G-CSF (Neupogen, Neulasta)
GM-CSF (Leukine, Prokine)
Infection control
Monitor for s/s infection
Cultures
Avoid invasive procedures
Consider thrombocytopenia

Back 8

neutropenia nursing mgmt

Front 9

monitor visits and handwashing!!
from themselves
From everything else
Strict hand washing
Private rooms
The gifts that keep on giving
Equipment
Air
Food
Water

Back 9

neutropenia care plan

Front 10

A group of malignant disorders affecting the blood and blood-forming tissues of
Bone marrow
Lymph system
Spleen
Occurs in all age groups.
Accumulation of dysfunctional cells due to loss of regulation in cell division.
Fatal if untreated

Back 10

leukemia

Front 11

No single causative agent

Associated factors include
Chemical agents
Chemotherapeutic agents
Viruses
Radiation
Immunologic deficiencies

Back 11

leukemia pathophys.

Front 12

Most common type of leukemia in children.
15% of acute leukemia in adults.
Immature lymphocytes proliferate in the bone marrow (most are B-cell)
Signs and symptoms may appear abruptly
Fever
Bleeding
CNS manifestations are common.

Back 12

Acute Lymphocytic Leukemia (ALL

Front 13

Excessive mature neoplastic granulocytes in bone marrow
Move into peripheral blood in massive numbers
Ultimately infiltrate liver and spleen
Philadelphia chromosome
Disease specific marker
Chronic, stable phase
Followed by acute, aggressive (blastic) phase

Back 13

Chronic Myelogenous Leukemia (CML)

Front 14

Production and accumulation of functionally inactive but long-lived, mature-appearing B-lymphocytes .
Lymph node enlargement is present throughout body
Increased incidence of infection
Complications from early-stage CLL are rare
May develop as the disease advances
Pain, paralysis from pressure caused by enlarged lymph nodes

Back 14

Chronic Lymphocytic Leukemia (CLL)

Front 15

25% of all leukemias
85% of the acute leukemias in adults
Abrupt, dramatic onset
Serious infections or abnormal bleeding
Uncontrolled proliferation of myeloblasts

Back 15

Acute Myelogenous Leukemia (AML)

Front 16

Varied but usually relate to
Bone marrow failure
Overcrowding by abnormal cells
Inadequate production of normal marrow elements
Inadequate marrow elements cause
Anemia
Thrombocytopenia
↓ Number and function of WBCs

Back 16

leukemia clinical manifestations

Front 17

Splenomegaly
Hepatomegaly
Lymphadenopathy
Bone pain
Meningeal irritation
Oral lesions
Solid masses (chloromas)

Back 17

leukemia cells cause

Front 18

To diagnose and classify
Peripheral blood evaluation
Bone marrow evaluation
To identify cell subtype and stage
Morphologic, histochemical, immunologic, and cytogenic methods

Back 18

leukemia diagnostic studies

Front 19

Chemo is mainstay of treatment
Monoclonal antibodies
Uses genetic technology to target and kill cancer cells.
Antibody binds to a known antigen on the surface of the lymphocyte.
Antibody-antigen complex is destroyed by the immune system.
Stem cell transplantation. Must eradicate pt’s own stem cells first, then replace with HLA matched stem cells.

Back 19

leukemia treatment

Front 20

cancer originates in lymphocytes

Back 20

Lymphoma hogdkins

Front 21

EBV, genetics, exposure to toxins
↑ in HIV

Back 21

lymphoma hogdkins etiology

Front 22

CM
Enlarged lymph nodes
Weight loss
Fatigue
Weakness
Tachycardia
Night sweats
Pruritis – no lesions
Alcohol – causes pain
Cough, dyspnea, stridor
Liver & spleen enlargement

Back 22

lymphoma hogdkins c.m.

Front 23

Blood analysis
Biopsy
definitive
Bone marrow exams
staging
Radiologic exam
CT
MRI
PET
For staging

Back 23

lymphoma hogdkins diagnostic studies

Front 24

Leukopenia & thrombocytopenia
Hypoferremia
Hypercalcemia
hypoalbuminenia

Back 24

noted labs for hodgkins lymphoma

Front 25

Indolent vs. rapidly developing
Unknown cause
More common in immunosupressed
No hallmark cell

Back 25

lymphoma non-hodgkins

Front 26

Unpredictable
Painless lymph node enlargement
s/s relative to location

Back 26

lymphoma non-hodgkins cm's

Front 27

similar to Hodgkin’s
More diagnostics
MRI
BE

Back 27

lymphoma non-hodgkins diagnostics

Front 28

Chemotherapy care
Radiation therapy care
Pancytopenia
Pain control
Aware of type/involvement – specifically for NHL
Psychosocial & spiritual support

Back 28

lymphoma care

Front 29

Cancerous plasma cells invade bone marrow and destroy bone.
Cancer cells produce abnormal and excessive amounts of antibodies and cytokines.
Insufficient normal plasma cells reduces immune function.
Skeletal pain is most common presenting symptom.

Back 29

multiple myeloma

Front 30

Bone pain, exacerbated by movement.
Osteoporosis.
Pathologic bone fractures.
Hypercalcemia.
Myeloma protein can cause renal failure.
Anemia, thrombocytopenia, neutropenia.

Back 30

multiple lyeloma clinical manifestations

Front 31

Proteins secreted from malignant plasma cells can be detected in urine.
M antibody protein
Bence-Jones protein
Radiologic studies reveal lytic bone lesions.
Bone marrow analysis reveals large numbers of plasma cells.

Back 31

mult. myeloma diagnostic studies

Front 32

Rarely cured, goal is remission and relief of symptoms.
Chemotherapy
Corticosteriods
Biological therapy
Hematological Stem Cell Transplant
Management of hypercalcemia and prevention of pathologic bone fractures are important components of therapy.

Back 32

mult. myeloma treatment

Front 33

Thrombocytopenia has many causes: immune and nonimmune

Immune thrombocytopenic purpura (ITP)
Platelets become coated with antibodies
Macrophages in spleen destroy platelets

Back 33

thrombocytopenia

Front 34

Bleeding
Mucosal (epistaxis, gingival bleeding)
Cutaneous (ecchymoses, petechiae, purpura)
Ecchymoses: “black and blue” bruising.
Petechiae are caused by RBCs leaking out of capillaries into the skin. Look like small, flat red dots on skin.
Purpura are consolidated petechiae.
Prolonged bleeding time
Risk is high for catastrophic hemorrhage (into the brain, retina, joints).

Back 34

thrombocytopenia c.m.'s

Front 35

Platelet count < 150,000 per microliter (μl)
< 50,000/μl prolongs bleeding time
< 20,000/μl at risk for spontaneous hemorrhage
Coagulation studies may indicate which part of coagulation cascade (if any) is involved.
Example: PT, aPTT
Bone marrow studies

Back 35

thrombocytopenia diagnostic studies

Front 36

To restore intravascular volume
To restore the oxygen carrying capacity of blood
To replace clotting factors and/or platelets
To replace WBC’s

Back 36

blood therapy

Front 37

Replaces RBC’s while preventing febrile, non-hemolytic transfusion reaction

Back 37

Leuko-poor
RBC’s

Front 38

Replaces RBC’s while preventing graft vs. host disease
Used in immunocompromised clients

Back 38

Irradiated RBC’s

Front 39

Replaces plasma without RBC’s or platelets. Contains most coagulation factors. Used in the control of bleeding

Back 39

Fresh Frozen Plasma

Front 40

Stop the transfusion
Maintain IV with NS
Notify physician and blood bank
Obtain blood & urine samples (if ordered)
Monitor VS and urine output
Administer medications as indicated
Return blood bag and tubing to blood bank
In some reactions, the transfusion may be restarted after medication administration
Document transfusion reaction

Back 40

interventions for blood reactio

Front 41

Fever
Chills
Tachycardia
Tachypnea, Dyspnea
Heat/pain along vein
Hives, Rash, erythema
Flushing
N/V/D, abdominal cramping
Hypotension
Wheezing
Chest/back pain
Headache
Myalgia with fever
Loss of consciousness
Cardiac arrest

Back 41

transfusion reactions

Front 42

Deficiency in RBCs, Hgb, Hct
s/s relative to hypoxia
See table 31-3 pg 686
Primary or secondary problem

Back 42

anemia

Front 43

Normal RBC lifespan is 120 days.

Erythropoietin (EPO) is a glycoprotein primarily produced in the kidneys (10% in the liver) that regulates RBC production and maturation.
↑ Number of stem cells committed to RBC production.
Shortens the time to mature RBC’s

Back 43

erythrocyte production

Front 44

Three alterations in erythropoiesis that decrease RBC production:
Decreased hemoglobin production.

Defective DNA synthesis.

Decreased number of erythrocyte precursors.

Back 44

erythrocyte production

Front 45

Dietary intake
Malabsorption
Blood loss or hemolysis
Most susceptible
Very young
Poor diets
Women in reproductive years

Back 45

Iron-deficiency anemia etiology

Front 46

Early – symptom free
Pallor
Glossitis-infl. On tongue
Cheilitis-infla. lips
c/o headache, paresthesia-itching
Burning sensation (tongue)

Back 46

iron def. anemia cm's

Front 47

Tachycardia, palpitations
Dyspnea
Fatigue
Severity based on Hb level.
Hb 10-14 g/dl is mild
Hb 6-10 g/dl is moderate
Hb < 6 g/dl is severe

Back 47

anemia

Front 48

Dx
CBC
Cause-
Guiac test
Endoscopy
Colonoscopy
Collaborative care
Treat the underlying cause

Back 48

iron def. anemia diagnostics and care

Front 49

1) Enteric coated or sustained release should not be used
150-200 mg (daily dose) of elemental iron QID or TID
1 hour before meals (give with OJ if possible)
Liquid iron (watch the teeth!) dilute & straw
GI side effects
Heartburn, constipation, diarrhea

Back 49

iron administration

Front 50

Can be given parenterally (IM or IV)
Stain skin – change out prep and admin needles
0.5 ml air bubble after solution for IM
IM – deep muscle – Z track method
Do not massage injection site
IV – do not mix with other meds and no faster than 1 ml/min

Back 50

nursing considerations for iron administration

Front 51

Antacids
Tetracycline
Ascorbic acid
Other oral iron salts
Ferrous gluconate and ferrous fumarate

Back 51

iron salts drug interatcions

Front 52

Person may be heterozygous for defective gene or homozygous.
Heterozygous: one copy of defective gene. Has thalassemia trait or thalassemia minor. May be asymptomatic or have mild disease.
Homozygous: two copies of defective gene. Will have thalassemia major:

Back 52

Thalessemias

Front 53

An autosomal recessive genetic disorder of inadequate production of normal hemoglobin
Problem is a genetic defect in the globin chain
Alpha chain defective in α-thalassemia
Beta chain defective in β-thalassemia (AKA Cooley’s anemia)
Most often occurs in persons of Mediterranean descent. Middle Eastern, African, and Asian also.
Leads to increased destruction of RBC’s (hemolytic anemia).

Back 53

Hb Production Disorder:Thalassemia

Front 54

Microcytic (small), hypochromic(pale) anemia with decreased RBC indices.
Pallor (r/t severe anemia; Hb < 6g/dl)
FTT (inadequate weight gain)
Hepatosplenomegaly
Facial deformities & skeletal abnormalities from hyperplasia of bone marrow.
Darkening of skin
Jaundice

Back 54

Clinical Signs of Thalassemia

Front 55

No specific drug or diet is effective in treating thalassemia
Thalassemia minor
Body adapts to decreased Hb
Thalassemia major
Blood transfusions to keep Hb around 10 g/dl.
Chelating agents (Desferal) may be needed to reduce iron overloading.
Splenectomy.
No Vit C or Iron Supplementation!

Back 55

Thalassemia Treatment

Front 56

Decreased intrinsic factor (IF) is usually the cause of Vit B12 deficiency.
IF is secreted by the parietal cells of the stomach. Is necessary for B12 absorption.
May be autoimmune, or caused by anything that decreases acid production in the stomach.
Most common cause: Pernicious anemia.

Back 56

Cobalamin (Vit B12) Deficiency

Front 57

due to ineffective hematopoiesis
& autohemolysis of megaloblasts.
WBC and platelet production also affected (pancytopenia).
Neurologic symptoms
Vit B12 deficiency causes demyelination of CNS neurons.
Paresthesias, decreased DTR’s, ataxia, irritability, & memory impairment result .

Back 57

Pernicious Anemia

Front 58

Increase in dietary cobalamin by itself does not correct the anemia.
Still important to emphasize adequate dietary intake.
Drug of choice: Cyancobalamin (Lehne, p. 630-631).
Can be given PO if pt can absorb some B12.
IM or SC injection
Nasal Spray
Regular evaluation for gastric carcinoma.

Back 58

Pernicious Anemia Treatment

Front 59

Also a cause of megaloblastic anemia
Folic acid is required for DNA synthesis
RBC formation and maturation affected.
Common causes
Poor nutrition
Malabsorption syndromes
Drugs
Alcohol abuse and anorexia
Lost during hemodialysis

Back 59

Folic Acid Deficiency

Front 60

Types
Congenital
Chromosomal alteration inherited from parents.
Acquired
Majority are idiopathic.
Result from exposure to ionizing radiation, chemical agents, viral and bacterial infections .
Relatively rare (4 cases per million)
May be chronic (managed with transfusions, EPO) or acute (risk of hemorrhage & sepsis).

Back 60

Aplastic AnemiaEtiology

Front 61

Destruction or hemolysis of RBCs at a rate that exceeds production.
Third major cause of anemia.

Back 61

Hemolytic Anemias

Front 62

RBC defective.
Abnormal hemoglobin (sickle cell disease)
Enzyme deficiencies (G6PD)
RBC membrane abnormalities

Back 62

Intrinsic hemolytic anemia –

Front 63

caused by factor other than the erythrocytes.

Back 63

Extrinsic hemolytic anemia –

Front 64

Group of inherited, autosomal recessive disorders.
Presence of an abnormal form of hemoglobin in the erythrocyte.
Hemoglobin S (HbS)
Substitution of valine for glutamic acid on the β-globin chain of hemoglobin
HbS causes the RBC to stiffen and elongate.
Assumes sickle shape in response to ↓ O2 levels
Incurable disease, often fatal .

Back 64

Sickle Cell Disease (SCD)

Front 65

Typical patient is asymptomatic except during sickling episodes.
Sickle cell crisis is a vasoocclusive crisis. See Lewis, p. 696
Symptoms can be
Pain and swelling of joints.
Pallor of mucous membranes
Fatigue

Back 65

Sickle Cell Disease (SCD) c.m.s

Front 66

Supportive care!
Pain management
O2 for hypoxia and to control sickling
IV hydration
Antibiotics
Hydroxyurea to prevent sickling (off label use).
Prevention of crisis.
Transfusion if necessary.
Stem cell transplantation.

Back 66

treatment of sickle cell anemia

Front 67

Types
Vera: neoplastic transformation of hematopoietic stem cells.
Secondary: chronic hypoxemia.
Results in increased blood viscosity and risk of thrombi.
Treatment is removal of cause, phlebotomy, bone marrow suppressants.

Back 67

Polycythemia

Front 68

Hypervolemia and hyperviscosity cause symptoms.
HTN: Headache, vertigo, dizziness, tinnitus, visual disturbances.
Blood vessel distention, impaired blood flow, thrombosis and tissue hypoxia cause paresthesias and cardiovascular symptoms.
Plethora
Hyperuricemia
Generalized pruritus, exacerbated by heat.
Pt. at risk for stroke & hemorrhage.

Back 68

Polycythemia clinical presentations

Front 69

Goal is to reduce blood volume and viscosity.
Phlebotomy to reduce HCT to < 48%
IV hydration

Back 69

Polycythemia Treatment

Front 70

Polycythemia Treatment suppress bone marrow

Back 70

hydroxyurea

Front 71

Polycythemia Treatment
(lowers blood counts)

Back 71

Interferon

Front 72

Polycythemia Treatment
decreases platelets

Back 72

Anegrelide

Front 73

vit b-12 defficiency, pernicious anemia, part of the schiling test. Necessary coenzyme needed for metabolic processes including fat and carbohydrate metabolism and protein synthesis, required for RBC formation. Monitor for hypokalemia, monitor for signs of b-12 def. pallor, neuropathy, red tongue, psychosis.

Back 73

Cyancobalamin

Front 74

antianemics, iron. SE: seizures, hypotension, nausea, constipation, dark stools, diarrhea, epigastric pain, skin staining, anaphylaxis. ASSESS for anaphylaxis

Back 74

Ferrous sulfate