Disord Heme Sys

Disorders of the Hematological
Blood and Lymph
• Two of the body’s most important functions
– Transportation
– Protection-from foreign invaders
• Achieved through the circulatory and lymph
• Blood is the primary transportation fluid
Blood Cells
• RBC’s, WBC’s, and Platelets
• Blood cells are made in bone marrow
– Other structures involved in the blood cell
production are:
• Kidneys (Erythropoietin)
• Liver (stores iron)
• Lymph system (breaks down blood cells, produces
Red Blood Cell Production (RBC)
• Erythropoiesis is the process of making red
blood cells
– Stimulated by decreased PO2
– The kidneys releases Erythropoietin
• Reticulocytes are immature RBC’s
• Iron, folic acid, and Vitamin B12 are needed
for RBC production
• Iron is needed for Hemoglobin production
Red Blood Cells (RBC)
• Normal Values
– Males 4.7-6.1
– Females 4.2-5.4
• Increased: Dehydration, Polycythemia
• Decreased: Anemia, Leukemia, Posthemorrhage
Hemoglobin (Hgb)
• Contained within RBC’s – red pigment
Oxygen carrying compound of the RBC
• Normal Values
– Males 14-18 g/dL
– Females 12-16 g/dL
• Increased: Dehydration, COPD, Polycythemia
• Decreased: Anemia, leukemia, Post-hemorrhage
– To measure Hgb, RBC’s are broken and Hgb is
released and counted
– Hemoglobin content is expressed as
normochromic or hypochromic anemia
• Erythrocytes are classified according to:
– Size, shape and color
– RBCs size is usually expressed as: macrocytic,
microcytic, or normocytic
– Average life span of an RBC = 120 days
• A measure of the packed cell volume of RBCs
• Expressed as a % of the total blood volume
• Normal Values
– Males 42-52%
– Females 37-47%
• Increased: Polycythemia, dehydration
• Decreased: Anemia, leukemia, hemorrhage,
fluid overload
White Blood Cells
• Have nuclei, are colorless, live from a few days
 several years
• Primarily involved in body defenses such as
destruction of bacteria and viruses
• “Differential” – blood count in which the
different kinds of WBCs are counted and
reported  can discriminate between virus
and backteria
• Also called “thrombocytes
• Normal Values 150,000-400,000
cells/microliter – involved with clotting
• 5-9 day life span
• Increased- Polycythemia, granulocytic
• Decreased- Bleeding, thrombocytopenia,
Erythrocyte Indices
• Will help determine type of anemia
– Iron Deficiency
– Folic Acid Deficiency
– B12 Deficiency
• Definition of Corpuscular: A living cell
Mean Corpuscular Volume (MCV)
• Volume (size) of the RBC
– Hematocrit divided by RBC Count
– Low
• RBC = Microcytic (small)
• Iron Deficiency Anemia
– High
• RBC = Macrocytic (large)
• B12
• Folic Acid Deficiency Anemia
Mean Corpuscular Hemoglobin (MCH)
• Weight of hemoglobin in a RBC
– Hemoglobin divided by the total RBC count
– Low
• Hypochromic (Deficient of hemoglobin)
– Iron Deficient Anemia
– High
• Hyperchromic- Lab error
Mean Corpuscular Hemoglobin
Concentration (MCHC)
• Concentration of hemoglobin in the RBC
– The hemoglobin is divided by the RBC
– Low
• Hypochromic (deficient of hemoglobin)
– Iron Deficiency Anemia
– High
• Hyperchromic
– Lab Error
Blood Types
• Genetically determined
• Determined by the presence or absence of
specific antigens on the outer surface of the
• In certain blood types, the antigens on the
RBC are accompanied by antibodies in the
Blood Types
• O – Good Donor, Bad Receiver
– Contains A and B antibodies at birth
• A- Very selective- receives A only
– Contains B antibodies at birth
• B- Very selective- receives B only
– Contains A antibodies at birth
• AB – Good Receiver, Bad Donor
– Does not contain A or B antibodies
Rh Factor
• Located on the surface of RBCs
• “positive” = have the factor
• “negative” = do not have the factor
• When an Rh- person has been exposed to
Rh+ blood, antibodies develop within 2 weeks
No reaction with this first exposure.
Rh Factor
• On any exposure thereafter, the Antibodies
will attack Rh positive blood.
• “Rh incompatibility” = mother’s antibodies
hemolyze the fetal RBCs  rupture and loss of
contents of cells
Rh Factor
• RhoGAM – intramuscular (or IV) injection
– Action: desensitization antibodies
– Next baby’s intrautero development without the
potential complications associated with Rh
• Who (include blood type and Rh factor) will
need to receive Rhogam?
– A women who is Rh Negative and is pregnant
(prophylaxis) or has just given birth (baby is Rh
Rh Factor
• When is RhoGAM given?
– 2 injections
• 1. between 20-28wks pregnancy
• 2. within 72 hrs. post partum
Who’s the Best?
• Which blood type (including Rh factor) is the
universal donor?
– O negative
• Which blood type is the universal recipient?
– AB positive
Lymphatic System
• A subdivision of the cardiovascular system
• Consists of:
– Lymph vessels
– Lymph fluid
– Lymph tissue
• Basic Functions:
– Maintenance of fluid balance
– Production of lymphocytes
– Absorption and transportation of lipids from intestine
to bloodstream
• Lymph is a specialized fluid formed in the tissue
spaces and transported by way of lymphatic
vessels to eventually  reenter the circulatory
• Lymphatic Tissue
Lymph nodes
• Characterized by low RBC count, ↓ hematocrit and
• Look at CBC indices to determine RBC health
Anemia causes delivery of an insufficient supply of
oxygen AND nutrients to the cells
• Loss of the O2-carrying element in the blood results in a
supply/demand imbalance in vital organs.
• Causes
– Hemorrhage
– Impaired production of RBC’s
– Increased destruction of RBC’s (hemolysis)
– Nutritional deficiencies
Diagnostic Tests:
Coombs’ Test
Hemoglobin Electrophoresis
Gastric Analysis
Coombs Test
• Direct Antiglobulin test – identifies
autoantibodies against RBCs
– Coombs + = autoimmune hemolytic anemia,
hemolytic disease of the newborn, lymphomas,
LE, mycoplasma infection, and infectious
General Signs and Symptoms of Anemia
• Big five
Chest pain
Dizziness (Vertigo)
Hypovolemic Shock
• May be caused by severe dehydration or extreme blood loss
• May require 1000 mL loss before severe s/s
• Initial s/s:
– Thirst
– Weakness
– Restlessness
• Later s/s
Tachycardia and weak, thready pulse
Pallor and cold, clammy skin
Hypovolemic Shock
• H&H will not decrease until ~6-8 hours
• Treatment
– If bleeding source obvious: direct pressure
• If not, intervention ASAP
– IV fluids (Isotonic/Volume expanders: NS)
– O2
– O negative blood transfusion
– Platelets
– Plasma
– Factor 7
Nursing Interventions
Monitor blood and fluid restoration
Identify blood loss sites to control bleeding
VS frequently
I/O – particularly careful monitoring of urine
• Patient teaching
Pernicious Anemia
• Caused by autoimmune disease: There is an
absence of glycoprotein intrinsic factor that is
usually secreted by the stomach and assists with
the absorption of Vitamin B12.
• Without this factor, Vitamin B12 is unavailable
for the maturation of the Red Blood Cell
Signs and Symptoms
• Subjective
Soreness and burning of
the tongue
– Tingling of the hands
and feet
– Impaired proprioception
• Objective
– Smooth and
erythematous tongue
– Gum infection
– Mental disorientation
– Personality changes
– Behavior problems
– Severe neurological
impair from destroyed
Spinal Cord Tracts
Diagnostic Tests
• Schilling test
– Subject will get two doses of vitamin B-12
(cobalamin). The first dose is taken by mouth. The
second dose is radioactive and is given as a shot 26 hours later. Urine is tested for radioactive B12
– Reveals malabsorption of Vit B12
• Serum Megaloblastic Anemia Profile (serum level of
B12) soon to replace Schilling test
– Will reveal serum levels of Vit B12 + more
• Gastric Analysis – determine pH
Medical Management
• Medication
– B 12 injections
– Folic acid supplement
– Iron replacement
• Transfused with Packed RBC if severe anemia
• CBC every 3-6 months
Iron-deficiency Anemia
• Plenty of RBC, normal H &H sometimes, but
• Causes
– Diet
– Usually chronic bleeding
– Body’s demand exceeds its absorption
• Pregnancy
• Infants
• Young adolescents
– Malabsorption of iron as in celiac disease or sprue
– Subtotal gastrectomy because iron needs an acid environment
to be absorbed.
Iron Deficiency Anemia
• Signs and Symptoms
– Sore, Swollen Tongue
– Cracks in the side of the mouth
– Brittle nails
– Increased frequency of infections
– PICA- Ice, dirt, paint etc.
– Restless Leg Syndrome
Iron Deficiency Anemia
• Medical Management
• Because less than 10% of iron is absorbed in
the duodenum, high doses of Iron supplement
are needed
• Additional Vitamin C has shown to enhance
iron absorption
• See p. 277 Box 7-2 for food sources of
nutrients needed for Erythropoiesis
Because some patients cannot tolerate oral
preparations of iron, Iron can be given IV or IM.
The z-track method of giving iron dextran is
preferred to prevent staining
Sickle Cell Anemia
• Genetic --Recessive homozygous
• Predominately found in the African
and African-American Population
• First crisis usually occurs at 10-12
weeks of age
• Pathophysiology: After the
hemoglobin molecules give up their
oxygen, some of them may cluster
together and form long, rod-like
Sickle Cell Anemia
• A sickle cell is an abnormal, crescent-shaped
RBC containing hemoglobin S (a defective
hemoglobin molecule)
• Sickle Cell crisis – an episode of acute
“sickling” of RBCs which causes occlusion and
ischemia in distal blood vessels
– Sickling  clumping or aggregation of these
misshapen RBCs which lodge in small vessels
Health maintenance for
patients with sickle cell
disease starts with early
diagnosis, preferably in
the newborn period and
includes penicillin
prophylaxis, vaccination
against pneumococcus
bacteria and folic acid
Sickle Cell Maintenance
• Health maintenance for patients with sickle
cell disease
– Starts with early diagnosis, preferably in the
newborn period
– Vaccination against pneumococcus bacteria
(infections are a major complication of Sickle-Cell
– Folic acid supplementation
– Prevention of infection and dehydration
Medical Management
• Supportive
– Pain is biggest problem
• PCA pump is indicated
• Long-term MS therapy with breakthrough pain meds
– Rehydration
– Packed cells for anemia but clients have problems
with iron overload
– Probable treatment/cure is stem cell transplant
Aplastic Anemia
aka Hypoplastic Anemia
• Congenital Aplastic Anemia is caused by a
chromosomal alteration
• Acquired Aplastic Anemia related to chemical
• Cause of 70% of acquired aplastic anemias remains
unknown (idiopathic)
Aplastic Anemia
• People are pancytopenic when:
– Reduced or absent red blood cells
– Reduced or absent white blood cells
– Reduced or absent platelets
Aplastic Anemia
• Signs/Symptoms:
– Pallor
– Bleeding tendencies
– Dyspnea
– Palpitations
– Repeated infection with high fevers
– Fatigue, weakness
– General malaise
Diagnostic Tests
• Diagnostic tests
– Bone Marrow Aspiration
• Hypoplastic or aplastic fatty deposits
– CBC may show normocytic and
normochromic blood cells
Medical Management
• Need to know cause!
– Blood transfusion with caution (need to preserve
immune system for bone marrow transplant)
– Spleenectomy if spleen is enlarged
– Steroids and/or androgens used to stimulate bone
– Immunosuppressive therapy or cyclosporine
Bone Marrow Transplant
• Involves taking cells that are normally found in the bone
marrow (stem cells), filtering those cells, and giving them
back either to the patient or to another person.
• The goal of BMT is to transfuse healthy bone marrow
cells into a person after their own unhealthy bone
marrow has been eliminated.
• Stem cells, when transplanted, find their way to the
recipient's marrow and begin to differentiate and
produce all types of blood cells that are needed by the
• Abnormal ↑ in RBC’s
• Two types
– Primary
• Myeloproliferative disease: overproduction by the bone
• Related to a Stem Cell abnormalityIncreased WBC, RBC
and platelets
– Secondary
• Usually related to chronic hypoxia – a physiologic response
to compensate for hypoxia Increased RBC
– Chronic hypoxia maybe d/t: pulmonary disease, CV
disease, high altitude, tissue hypoxia
Polycythemia Vera
• Signs and Symptoms:
– ↑ blood volume and viscosity  HTN, angina, HF,
– Venous distention
– GI bleeding
– Hepatomegaly and splenomegaly from organ
Polycythemia Vera
• Diagnostic Tests:
– H and H (Plasma and RBC volume ↑)
– Reticulocyte and Erythrocyte counts
– Platelets and RBC’s
– Bone Marrow exam
Polycythemia Vera
Polycythemia Vera
• Medical Management
– Repeated plebotomy
– Myelosuppressive agents
– Allopurinol for gout
• True Chronic Disease requires lifetime treatment
• Population can be non-compliant until very ill
• Death by thrombosis
Disorders of Leukocytes
• Severe reduction of Granulocytes
– Basophils
– Eosinophils
– Neutrophils
• Low WBC count (leukopenia)
• Usually caused by medication and/or toxic
substances(includes herbal substances!)
• Signs and Symptoms:
– Fever and chills
– Headache and fatigue
– Infections
– Ulceration of the mucous membranes
– Pneumonia and UTIs
Medical Management
Need to know the cause
Transfusion of Packed RBC
G-CSF (filgrastim or Neupogen)
Neutropenic precautions (Reverse Isolation)
Death by Infection
Nursing Interventions
• A patient with a compromised WBC system is
highly susceptible to life-threatening
List nursing interventions directed toward
protecting the patient from potential sources of
• Malignancy involving
the hematopoetic
• Characterized by rapidly
developing white cells
that overwhelm the
bone marrow, lymph
system, spleen and liver
• Complications include
infiltration and damage
to organs as well as the
central nervous system
• Increased susceptibility
to infections
Four Major Types:
Acute lymphocytic leukemia (ALL)
Acute Myelogenous leukemia (AML)
Chronic Myelogenous leukemia (CML)
Chronic Lymphocytic leukemia (CLL)
• S/SX
– Irrelevant complaints at first
• Malaise
• Fatigue
• Irritability
Mucous membrane bleeding
Bone pain
Meningial irritation
Enlarged lymph nodes
• Diagnostic Tests
– WBC ↓, ↑, or excessively ↑
– Bone Marrow Bx.
– Lymph Node Bx.
– LP, CT scan
Medical Management
• Chemotherapy – interrupt cell growth at multiple
points in the cell cycle
• Bone marrow transplant and stem cell therapy
• Death by infection, hemorrhage and/or GraftVerses-Host Disease if BMT treated
• More to follow in Oncolgy Nursing
Nursing Interventions
Prevention of Infection
Patient education
Pain Control
Psycho-social support for coping
Coagulation Disorders
Disorders of Coagulation
• Release of blood from the vascular sys. results
– trauma or vessel damage
– vessel inadequacy
– disturbance of the function of platelets or clotting
factors, or liver disease ( impairment of clotting
• Disorders can be congenital, acquired, or 2nd
to disease or medication toxicity
“Who Ate my Platelets?”
Idiopathic Thrombocytopenia (auto-immune disease)
Aplastic anemia
• Platelets < 150,000 (nml = 150,000-400,000/mmᶟ)
• S/SX
– Petechiae and eccymosis on the skin
– Frank Bleeding
– Low Platelet count on CBC
– With extremely low platelet counts, fatal central
nervous system or gastrointestinal hemorrhage
can occur
• Management
Corticosteroid therapy
Splenectomy if needed
Immunosuppresive drug
– Platelets to increase count to
– Plasmapheresis
• Plasmapheresis involves
selective removal of the
patient's plasma, which is
then replaced by albumin
• Time time needed to
complete a treatment
usually ranges from 1 1/2 - 3
• Disease of the royal
family (don’t marry
your cousin!)
• X-linked hereditary trait
• Clinical Manifestations:
– Internal or external hemorrhage with large
ecchymoses into tissue
– Hemarthrosis = bleeding into a joint
• Swelling, fever, pain
• Diagnostic Tests
– Blood work for Factors VIII or IX
• absent or deficient
Medical Management
• Replacement therapy—giving or replacing the
clotting factor that’s too low or missing.
– Most common: antihemophilic factor VIII is
– Hemophilia B is missing factor IX
• Check out the National Hemophilia Society
After an IM injection
Hemophilia Crisis
• Usually involves trauma
– Ice and elevate above heart
– Apply pressure to bleeding
– Immobilization of limb
– Pain Management
• Can be treated with cryoprecipitate (FFP)
• Medic alert bracelet should be worn at all
HIV and Hemophilia
• Usually associated with infusions prior to 1984
• Heat inactivation of the virus has significantly
reduced the client’s exposure to HIV
vonWillebrand’s Disease
• Mild form of Hemophilia
• S/Sx
– Slow coagulation of blood (as opposed to none)
– Spontaneous GI Bleeds
– Epistaxis
– Gingival Bleeding
• Common occurrences: post partum,
menorrhagia, post op
vonWillebrand’s Disease
• Medical Management:
– Usually treated with Cyroprecipitate (containing
Factor VIII)
– Fibrinogen
– Fresh plasma
– Desmopressin (DDAVP): Synthetic ADH
Disseminated Intravascular
• A disorder in which the proteins that control
blood clotting become abnormally active 
• Small blood clots form in blood vessels
• Plasma clotting factors are depleted during
widespread clotting within small vessels 
bleeding disorder and thrombosis.
• Primary disorder initiates a generalized
intravascular clotting
High Risk Diagnosis
pg. 290, AHN
Medical Management
• Supportive in nature
• Heparin therapy to calm
the hypercoagulopathy
Lab tests confirm dx
Serum fibrinogen - low
(PT) - high
(PTT) - high
Platelets: depressed
breakdown): elevated
Multiple Myeloma
• A malignant neoplastic immunodeficiency disease of
the bone marrow
• Neoplastic plasma cells infiltrate the bone marrow 
destruction of osseous tissue, esp. flat bones  pain,
fractures, and skeletal deformities
• “Monoclonal Protein” – the specific immunoglobulin
produced by the myeloma cells
• Gradual onset so it slips
right by practitioners
• Could present itself as a
spinal fracture with cord
• Disrupts erythrocyte,
platelet and leukocyte
• Electrolyte disturbance
from release of Calcium and
Phosphorus from
deteriorating bone cells
Signs and Symptoms
Bone pain
Feeling ill
Night sweats
Weight loss is not common in the early stages.
Multiple Myeloma
• Physically, patients are pale with diffuse bone
tenderness, especially around the sternum
(breastbone) and pelvis (hips).
• Pathologic fractures (fractures caused by tumors)
occur frequently.
– The spine is the most common location for a pathological
fracture. It can also happen in the ribs and pelvis.
• Diagnostics
– Radiographic skeletal
– Bone marrow aspiration
– Labs
Pancytopenia (low cells)
Elevated creatinine
– Urinalysis
• High protein
Multiple Myeloma
• Medical Management
– Supportive
– Radiation and chemotherapy to reduce or impede
tumor growth
– Hypercalcemia tx.
– Pain management
Multiple Myeloma
• Nursing Interventions:
– Pain management
– Prevention infection
– Prevention bone injury
– Administration Chemo
– Maintain hydration
– I/O
– Weight-bearing
– Patient Education
Hodgkin’s Disease
Look! It’s
’m so
• Enlargement of lymphoid
• Affects men 2:1
• Bimodal peak (2 separate
– Early 20-30
– Late 60-70
– Accounts for <1% of all cancer
– Differentiated by Lymphocyte
cytology, Reed-Sternberg cell
Hodgkin’s Lymphoma
• Hodgkin's disease may occur in a single lymph
node, a group of lymph nodes, or, sometimes,
in other parts of the lymphatic system such as
the bone marrow and spleen.
• This type of cancer tends to spread in a fairly
orderly way from one group of lymph nodes to
the next group.
Hodgkin’s Lymphoma
• Risk factors associated with this disease:
– Age/Sex -- Hodgkin's disease occurs most often in people
between 15 and 34 and in people over the age of 55. It is
more common in men than in women.
– Family History -- Brothers and sisters of those with
Hodgkin's disease have a higher-than-average chance of
developing this disease.
– Viruses – Epstein-Barr virus is an infectious agent that may
be associated with an increased chance of getting
Hodgkin's disease.
Hodgkin’s Lymphoma
• Signs/Symptoms
– A painless swelling in the lymph nodes in the
neck, underarm, or groin
– Unexplained recurrent fevers
– Night sweats
– Unexplained weight loss
– Itchy skin
Hodgkin’s Lymphoma
• Diagnostic studies
CBC: elevated WBC
Presence of Reed-Sternberg
• Reed-Sternberg cell: characterized by mirror
image nuclei
Medical Management
• Radiation against localized
• Chemotherapy for early
and late stages
• Candidates for Neupogen
because of suppressed
neutrophil production
• High risk for secondary
malignancies especially
Non-Hodgkins Lymphoma
Absence of the Reed-Sternberg Cell
Usually described as “all other lymphomas”
More likely to spread beyond the lymph nodes
Comprised of approximately 10 different
subtypes and 20 different disease entities
• These subtypes are grouped into 3 biologic
states- low grade, intermediate grade, and high
grade lymphomas
Non-Hodgkins Lymphoma
• S/Sx
– Fatigue
– Malaise
– Anorexia
Non-Hodgkins Lymphoma
• Diagnostic Tests:
– Anemia
– Increased platelets
– Leukocytosis(increased and abnormal WBCs)
– ESR-elevated
– Hypercalcemia and elevated Alkaline Phosphatase
– Positive Coombs test
– Chest Xray
– CT scan
Non-Hodgkins Lymphoma
• Medical Management
– Therapy is determined by several factors:
• biologic state of the lymphoma
• stage of lymphoma
• presence or absence of symptoms (e.g., weight
loss, night sweats, organ dysfunction)
• overall general health of the patient
– Chemo and radiation
Non-Hodgkin’s Lymphoma
• Nursing Interventions
– Supportive care during radiation and chemo
– Observation for complications
– Patient education
– Participate in care planning

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