Chapters 33 and 33 : Hematological System

Iron Deficiency Anemia

Etiology - inadequate dietary intake
(Cause) - malaborption, 5-10% ingested iron absorbed in duodenum
- blood loss : loss of 50-75 ml of blood from upper GI,
( black tarry), peptic ulcers, gastritis, esophagitis,
hemorrhoids, GU, menstrual, pregnancy.
- Hemolysis: from immune reactions, medications, toxiins
and poisons, hemodialysis or use of heart-lung by pass
machine
Susceptible
- v young
- poor diet intake
- women in preductive years
- ** pregnant and menstruating woman need more iron
Clinical
Manifestatio FATIGUE
ns - Pallor
- glossitis: tongue infmaled
- inflame depapilation
- reduced saliva : norm flora infection
- chellitis: inflammation of lip
- PICA: craving for any unusal substance, search for occult
blood loss secondary iron deficiency
-
Diagnostic Labs
Studies - cbc: hgb, ht, mcv ,mch, mchch, platelets
- reituclocytes : rbc reenagers
- serum iron, tibc, ferritin, iron studies
- bili ruibin
Stool guacic test/ hemoccult, endoscopy, colonoscopy

Collaborativ  treat underlying disease

e Care  incrased intake of iron
 oral or occasional parental iron supplements
 transfusions of packed RBCS
Drug Therapy
 Oral iron
 Inexpensive and convenient
 Factors to consider
○ Enteric-coated or sustained-release capsules
are counterproductive
○ Best absorbed as ferrous sulfate in an acidic
environment
- Translation: Take before
 meals on empty stomach
○ Liquid iron should be diluted and ingested
through a straw
○ Side effects: Heartburn, constipation,
diarrhea STOMACH CRAMPS,
CONSTIPATION, use stool softener .iron
turns stools black, educate
 Ferrous Sulfate
 MORE ACTUAL IRON!
○ (…but more GI side effects)
 Ferrous Gluconate
 Less actual iron
 (..but easier on the stomach)

Nursing  At-risk groups

Managemen  Premenopausal women
t  Pregnant women
 Persons from low socioeconomic backgrounds
 Older adults
 Individuals experiencing blood loss
 Diet teaching
 Supplemental iron
 Discuss diagnostic studies
 Emphasize compliance
 Iron therapy for 2 to 3 months after the hemoglobin levels
return to normal

 One of the most common chronic hematological disorders

○ Iron is present in all RBCs as heme in hemoglobin and in a
stored form
○ Heme accounts for two thirds of the body’s iron

Red blood cells normally live for 110 - 120 days. After that, they naturally break
down and are usually removed from the circulation by the spleen.
Some diseases and processes cause red blood cells to break down too soon.
This requires the bone marrow to make more red blood cells than normal. The
balance between red blood cell breakdown and production determines how low
the red blood cell count becomes.

A client with a history of iron-deficiency anemia who has not taken iron
supplements for several years is experiencing increased fatigue and
dizziness.
What would the nurse expect the client’s laboratory findings to include?
1 Hematocrit (Hct) 0.38 (38%)
 2 Red blood cell (RBC) count 4.5 X 1012
3 Hemoglobin (HBc) 86 g/L ANSWER
4. Normal RBC indices

To prevent a common side effect of oral iron supplements, the nurse

teaches the patient to:
A. Take the iron preparation with meals
B. Increase fluid and dietary fiber intake
C. Report the presence of black stools to the physician
D. Use enteric-coated preparations take with orange juice

If a reticulocyte count were done on a patient with iron deficiency

anemia because of chronic bleeding it would be: High
A. Low
B. high
C. Normal
D. Reticulocyte count is not related
Thalassemia
Etiology - autosomal recessive genetic disorder : INADEQUATE
production of normal hemoglobin
- heme molecule okay but GLOBULIN protein is abnormal
- hemolysis also occurs
Alpha thalassemia: 2 HBA genes, p arm of chrm 16
Beta Thalassemia : 1 HBB gene, p arm of chrm 11
In either :
Heterozygosity: thalassemia minor
Homozygosity= thalassemia major

Clinical  Thalassemia minor

Manifestations  Asymptomatic frequently
 Moderate anemia (splenomegaly, mild jaundice)
 Thalassemia major: HOMOZYGOSITY
 Life-threatening
 Physical and mental growth often retarded
 Symptoms develop in childhood
 Splenomegaly, hepatomegaly, jaundice

Collaborative  Collaborative Care

Care  No specific drug or diet is effective in treating
thalassemia
 Thalassemia minor
 Body adapts to decreased hemoglobin
 Thalassemia major
 Blood transfusions with IV deferoxamine
 Deferoxamine chelating substance that prevents iron
intoxication and chronic iron overload due to transfusion-
dependent anemia

Megaloblastic Anemias
Anemias  Group of disorders caused by impaired DNA synthesis
 Majority result from deficiency in
 Cobalamin (vitamin B12)
 Folic acid
 Characterized by the presence of large RBCs
(megaloblasts)
B12 folic acid deficit> impaired dna synthesis > delayed in cell
cycle, longer time in G phase > large RBCs

Anemia of  Causes
chronic  End-stage renal disease
disease  Chronic liver disease
causes  Chronic inflammation
 Malignant tumors
 Chronic endocrine diseases
 Leading to...
 Underproduction of RBCs
 Mild shortening of RBC survival

Aplastic (“without growth, without blood”)

Anemia Etiology/Clinical Manifestations
Idiopathic….perhaps autoimmune?
 Symptoms caused by suppression all bone marrow
elements:
○ White blood cells, Red blood cells, and
Platelets (“pancytopenia”)
 General manifestations of anemia
 Fatigue, dyspnea
Nursing Management
 Preventing complications from infection and hemorrhage
 Untreated prognosis is poor
 75% fatal
 Treatment options
 Bone marrow transplantation
 Immunosuppressive therapy

UT treatment, it may progress and become fatal

Hemolytic Destruction or hemolysis of RBCs at a rate that exceeds
Anemia production
 Intrinsic hemolytic anemia
  Abnormal hemoglobin
 Enzyme deficiencies
 RBC membrane abnormalities
 Jaundice
 Destroyed RBCs cause increased bilirubin
 Enlarged spleen and liver
 Hyperactive with macrophage phagocytosis of the
defective RBCs
 Accumulation of hemoglobin molecules can obstruct renal
tubules
 Tubular necrosis

Sickle Cell Disease

Etiology  Autosomal recessive
 HBB gene (p arm of chrmsme 11)
○ Heterozygosity = > “carrier”
○ Homozygosity = > disease
 Presence of an abnormal form of hemoglobin-B in the
erythrocyte
 Hemoglobin S (HbS), abnormal
 HbS causes the RBC to stiffen and elongate
 Sickle shape in response to ↓ O2 levels
 Substitution of valine for glutamic acid on the β-
globin chain of hemoglobin
 Genetic disorder
 Incurable disease, often fatal

Clinical  Typical client is asymptomatic except during sickling

Manifestatio episodes (sickle cell crisis)
ns  Symptoms can be
 Pain and swelling (obstruction to circulation)
 Pallor of mucous membranes
 Fatigue

Nursing  Alleviate symptoms of disease complications

Managemen  Minimize end target-organ damage
t  No specific treatment for SCD
 Client teaching
 Avoid high altitudes, maintain fluid intake, treat
infections, pain control
 Folic acid daily supplements
 Blood transfusions in crisis
 Hydroxyurea: Antisickling agent (see next slide)
 Erythropoietin shots for clients unresponsive to
 hydroxyurea
 Bone marrow transplant
 Can cure some clients with SCD
Interventions
 Maintain adequate hydration
 Administer oxygen & blood transfusions
 Administer analgesics
 Monitor for infections
 Ensure adequate vaccines
Hydroxyurea: hydrea
 Chemotherapeutic Agent
 Decreases the formation of sickled cells
 Many SE
 Toxicity
 More research is needed on proper identification of who
should get this drug
CAUTION:
Hydroxyurea can cause a severe decrease in the number of
blood
cells in your bone marrow. This may cause certain symptoms and
may increase the risk that you will develop a serious infection or
bleeding. If you experience any of the following symptoms, call
your doctor immediately: fever, chills, sore throat, ongoing cough
and congestion, or other signs of infection; unusual bleeding or
bruising; bloody or black, tarry stools; bloody vomit; or vomiting
blood or brown material that resembles coffee grounds.

REVIEW

Hemochromatosis
Hemophilia &
Thrombocytopenia

please review in your text

page 801 - 812

Hemochromatosis
Etiology/Causes  Hereditary- Genetic Condition where excessive Iron
is deposited in organs
 No evidence of disease until middle age
 Men have more issues then Women
 Treatment phlebotomy
 Can have an acquired form- mainly in alcoholics
Disseminated Intravascular Coagulation (DIC)

Bleeding Manifestations Thrombotic Consequences

 Integumentary  Cyanosis
Pallor  Ischemic tissue necrosis
Petechiae  Pulmonary emboli
Purpura  Cardiovascular changes
Oozing blood (IV sites)  GI changes
 GI manifestations  Oliguria
 Hematuria
 Changes in mental status

ACUTE DIC CHRONIC DIC

 Acute develops suddenly  Chronic DIC (reflects a
Severe consumptive coagulopathy compensated state)
leading to hemorrhage. More frequently observed in solid
Organ failure frequently occurs with tumors and in large aortic aneurysms
acute DIC Clinical manifestations may only be
easy bruising to hemorrhage and from
hypercoagulability to thrombosis

DIAGNOSTIC STUDIES FOR DIC  PT/PTT/INR <- starting test for

any blood clotting
 Thrombin time
 Fibrinogen
 Platelets
 Fibrin split products
 D-dimer (byproducts of
fibrinogen breakdown)
 Antithrombin III
 Protein S and Protein C (anti-
thrombotic proteins)

DIC MANAGEMNET Principle of treatment:

Stop the coagulation to stop the
bleeding
  Treat cause!!
 Heparin
 Blood Products
PRBC’s
Platelet transfusions
FFP

LEUKEMIA
Malignancy involving the abnormal overproduction of leukocytes (immature) in
the bone marrow
 A group of malignant disorders affecting the blood and blood-forming
tissues of
Bone marrow
Lymph system
Spleen
 Often thought of as a childhood disease
 The number of adults affected is 10 times that of children

Classification
Acute Myelogenous Leukemia
Acute Lymphocytic Leukemia
Chronic Myelogenous Leukemia
Chronic Lymphocytic Leukemia
Hairy Cell Leukemia
Unclassified Leukemias
Categorires
 Type of white blood cell (WBC)
Acute lymphocytic leukemia (ALL)
Acute myelogenous leukemia (AML)
Also called acute non-lymphoblastic leukemia (ANLL)
Chronic myelogenous leukemia (CML)
Chronic lymphocytic leukemia (CLL)

Clinical Related to problems caused by

Manifestations Leukemic cells infiltrate client’s organs (including bone
marrow)
Splenomegaly
Hepatomegaly
Lymphadenopathy
Bone pain, meningeal irritation, oral lesions (chloromas)
Marrow-suppression sx (bleeding, infection, fatigue,
nutrition)

Diagnostic  To diagnose and classify

Studies Peripheral blood evaluation
 Bone marrow evaluation (biopsy)
 To identify cell subtype and stage
Morphological, histochemical, immunological, and
cytogenic methods

Collaborative  Combination chemotherapy

Care: leukemia Mainstay treatment
3 purposes
↓ drug resistance
↓ drug toxicity to the client by using multiple drugs with
varying toxicities
Interrupt cell growth at multiple points in the cell cycle
Wk 1 chemo cycle, wk 2 cbc check, wk 3 cbc chek and
repeat till wk 12
Collab care:
Chemo regimens

Side and adverse effects result from the effects of

antineoplastic medication on normal cells
Antineoplastic medications cause the rapid destruction of
cells, Resutling in Uric acid

Adverse Reactions
 Mucositis
 Alopecia
 Anorexia, nausea, and vomiting
 Diarrhea
 Anemia
 Neutropenia
 Thrombocytopenia
 Neuropathy

Collab care: bone  Goal

marrow and stem Totally eliminate leukemic cells from the body using
cell transplant combinations of chemotherapy with or without total body
irradiation
 Eradicates client’s hematopoietic stem cells
 Replaced with those of an HLA-matched
 Sibling
 Volunteer
 Identical twin
 Client’s own stem cells removed
before

Hodgkins Lymphoma
 Hodgkin’s disease
 Non-Hodgkin’s Lymphoma (NHL)

HODGKINS DISEASE
Etiology Malignancy of the Lymph Nodes
Risks: Viral infections;

Characterized by:
- proliferation of abnormal giant, multinucleated cells
- reed-sternberg cells
- located in the lymph nodes

Clinical Stage one: one group of lymph node, same side of diaphragm
Manifestations Stage two: two or more groups on the same side of the
diaphragm
Stage three: on both sides of the diaphragm , occasionally with
involvement of another organ( spleen st.3)
Stage four: liver, lung , bone marrow involvement
Treatment • Radiation therapy
– 95% of stage I or stage II clients cured with 4 to 6 weeks
of radiation therapy
• Combination chemotherapy
– Resistant disease or high risk of relapse
– MOPP and ABVD
 Wk 1 -> Chemo cycle 1
 Wk 2 -> CBC check
 Wk 3 -> CBC check
Repeat till wk 12

NON HODGKINS LYMPHOMA

Etiology – Varies from slowly developing to rapidly progressive
disease
– Type of NHLs
– 90% B cells
 – 10% T cells
Types of NHLs
– Burkitt’s lymphoma
– Diffuse large B cell lymphoma
– Follicular lymphoma
– …and many more!
– No hallmark feature, but…
– All NHLs involve lymphocytes arrested in various
stages of development

Clinical  Widely disseminated disease usually present at time of

Manifestations diagnosis
 Painless lymph node enlargement
Primary clinical manifestation
 Clients with high-grade lymphomas
Lymphadenopathy and B symptoms
 Peripheral blood is usually normal
Some lymphomas manifest in “leukemic” phase

Diagnostics  Similar to Hodgkin’s lymphoma

 Lymph node biopsy
Establishes cell type and pattern
 Staging guides therapy
 Prognosis for NHL is generally not as good as that for
Hodgkin’s lymphoma

Collaborative  Treatment consists of chemotherapy +/- radiation

Care  More aggressive lymphomas are more responsive to
treatment
Chemotherapy regimen
 CHOP
 CVP
 COPP
Biological therapy
 Alpha interferon (Intron)
 Rituximab (Rituxan)
 Ibritumomab tiuxetan (Zevalin)
 Complete remissions are uncommon
 Most respond with improvement in adenopathy
and symptoms
 Radiation alone may be treatment enough for localized
stage I or stage II disease
 Asymptomatic clients with advanced disease followed
with “watchful waiting” to assess the disease progress
Multiple myeloma
 Myeloma is malignant tumor of plasma cells within the bone (B cell
subtype) arising from a single clone

Etiology  Multiple myeloma accounts for > 40% of primary

malignant tumors of bone.

Clinical  Bone pain/fracture

Manifestations  Kidney damage
Byproduct of plasma cells causing damage to nephron
 M protein
immunoglobulin made by abnormal plasma cells

 Bone pain especially ribs, spine & pelvis

 Weakness & fatigue
 Recurrent infections
 Urinalysis shows Bence Jones proteinuria
 Osteoporosis
 Elevated calcium and uric acid levels
 Kidney failure
 Spinal cord compression

Disorders of the Spleen

1. Enlargement causes sequestration of blood products
2. Removal predisposes to infection potential
3. Rupture may cause radiating pain...

Blood Component Therapy

Administration Procedure: double check
ACUTE DELAYED
- acute hemolytic reactions - viral transmission: Hep
- febrile reactions B,C,HIV,HTLV, west nile, others
-allergic reactions ( minor or severe) - other infections e,g, prion,
- circulatory overlaod transmission: variant CJD
-bacterial sepsis - alloantibody formation
-transfusion related acute lung injury - post-transfusion Purpura ( PTP)
( TRALI)

Transfusion Reactions
Acute Hemolytic Reaction Destruction of Donor RBCs
Acute Renal Failure may occur
ABO incompatibility:
Circulatory Overload May occur in cardiac and renal
 insufficiency
Massive Blood Transfusion Reaction Hypocalcemia and HyperKalemia:
Allergic Reaction May restart transfusion with
anhistamine therapy in mild cases:
Destruction of Donor RBCs

What to do after a reaction?

 Stop the transfusion
 Change the IV tubing
 Notify the HCP
 Stay with the patient – observing vital signs
 Prepare to administer medications as prescribed
 Obtain urine specimen and other lab studies
 Return the blood & tubing to lab
 Document the occurrence

Aphresis
 AKA Plasma pheresis
 Sends a patients blood through a centrifuge and the components are
removed
 Can get extra platelets
 WBC
 Harvest stem cells for stem cell transplant

A patient with hemophilia has experienced recent trauma

 What should the nurse monitor with this patient?
Monitor for bleeding
Monitor vital signs
Control joint bleeding with immobilization, elevation and application of ice.

Hemophilia Von willebrand’s disease

 Two inherited disorders A & B  Common bleeding disorder but

 A has lack of factor VIII effects males and females
 B (aka Christmas disease) lack of equally
factor IX  vWF is needed for factor VIII
 Men get this disease Women are activity
gene carriers  Bleeding especially nose bleeds,
 Bleeding is the major issue excessive bleeding from cuts,
 Bleeding into the joints major heavy menses, postoperative
symptom is swelling and pain bleeding
 Can bleed even without trauma  Prolonged bleeding times
 Treatment for both (Similar to
Hemophilia)
DDAVP (increases Factor VIII
Cryoprecipitate needed prior to
major surgeries
Platelets adhere to damaged
endothelium
Apply pressure
Replacement of clotting factors