Interferences

with
Diffusion

Anemia

Leukemia

Hemophilia
Interferences with Diffusion
 Describe clinical manifestations, causes, therapeutic interventions,
& nursing management of patients with the following Hematologic
Problems:
 Anemias –
 Decreased Erthrocyte Production
 Iron Deficiency Anemia; Thalassemia;
 Megaloblastic Anemias: Cobalamin deficiency, Folic Acid Deficiency; Aplastic Anemia
 Anemia Caused by Blood Loss
 Anemia Caused by Erythrocyte Destruction
 Sickle Cell Disease
 Acquired Hemolytic Anemia
 Hemochromatosis
 Polycythemia
 Problems of Hemostasis:
 Thrombocytopenia
 Hemophilia and Von Willebrand’s Disease
 Leukemias –
 Acute myelogenous leukemia
 Acute lymphocytic leukemia
 Chronic myelogenous leukemia
 Chronic lymphocytic leukemia
 Lymphomas
 Hodgkin’s Disease / non-Hodgkin’s lymphomas
 Multiple Myeloma
Mature Normal Erythrocytes
Nutrients Needed for Erythrocytes
 Interferences with Diffusion
Hematologic System Review
 Complete Blood Count Studies
 Hgb
 Hct
 Total RBC Count
 Red Cell Indices
 MCV – mean corpuscular volume (size of RBC)
 MCH – mean corpuscular hemoglobin (weight of Hb/RBC)
 MCHC – mean corpuscular hemoglobin concentration (saturation of
RBC with Hb)
 WBC
 WBC Differential
 Platelet Count
 Erythrocyte Sedimentation Rate (ESR or Sed Rate)
Development of Blood Cells
Blood Components
What are the functions of blood components?
Functions of Blood
Drugs Affecting Hematologic
Function
 Interferences with Diffusion
Anemias Caused by Decreased Erythrocyte Production

Anemia

 Deficiency in the number of erythrocytes (RBCs)

 The quantity of hemoglobin
 Volume of packed RBCs (hematocrit)

 Clinical Manifestations: caused by the body’s response

to hypoxia
 Mild (Hb 10 -14) no symptoms or minor changes
 Moderate – (Hg 6 – 10) CV Changes: palpitations, dyspnea,
diaphoresis
 Severe – (Hg<6) multiple body system CV, Cerebral, Major
Organs
 Anemia
Clinical Manifestations
 Interferences with Diffusion
Anemias Caused by Decreased Erythrocyte Production
Iron-Deficiency Anemia
 Common hematologic disorder

 Etiology: Inadequate dietary intake, malabsorption, blood loss, or hemolysis

 Clinical Manifestations:
 Pallor
 Glossitis – inflammation of the tongue
 Cheilitis – inflammation of the lips
 Headache, paresthesia, burning sensation of the tongue

 Diagnostic Studies: Lab Studies

Endoscopy to identify GI bleed

 Treatment: Drug Therapy – oral Iron replacement

 Iron absorbed best in duodenum
 Ferrous sulfate – take about one hour prior to meal
 Gastric side effects: nausea / constipation

 Nursing Management – Diet & Medication Instruction

 Interferences with Diffusion
Anemias Caused by Decreased Erythrocyte Production
Thalassemia

 Autosomal recessive genetic disorder of inadequate production of

normal hemoglobin
 Hemolysis occurs
 Abnormal Hb synthesis
 Ethnic groups of Mediterranean Sea & near equatorial regions of Asia
and Africa
 Clinical Manifestation: mild – moderate anemia with hypochromia
(pale cells) or microcytosis (small cells)
 Minor: one thalassemic gene – mild
 Major: two thalassemic genes – severe – physical & mental growth
retarded - cardiac failure is fatal
 Medical Management:
 Medication: Chelation Therapy IV deferoxamine (Desferal) – iron
binding agent to reduce iron overload
 Transfusions to maintain Hg >10g/dl
 Nursing Management: Supportive
 Interferences with Diffusion
Anemias Caused by Decreased Erythrocyte Production
Megaloblastic Anemias
Caused by impaired DNA synthesis & characterized
by the presence of large RBCs
Cobalamin Deficiency (Vitamin B12)–
 Intrinsic factor (IF) is secreted by the parietal cells of the gastric mucosa
 Cobalamin is not absorbed if IF is not present
 Causes: Pernicious anemia, nutritional deficiency; heredity enzyme
defect
 Clinical Manifestations: GI—sore tongue, anorexia, N&V, abdominal
pain; muscle weakness, paresthesias of feet and hands; confusion
 Diagnostic Testing: Serum cobalamin levels; gastroscopy; Schilling
Test – assesses parietal cell function
 Medical Management: Parenteral administration of cobalamin – daily
for 2 weeks, then weekly until >HCT, then monthly for life;
intranasal form
 Nursing Management: Health Promotion; protection from sensory
injury—burns, trauma; pt compliance with replacement therapy
 Interferences with Diffusion
Anemias Caused by Decreased Erythrocyte Production

Megaloblastic Anemias
Folic Acid Deficiency – Folic Acid is required for DNA
synthesis leading to RBC formation & maturation
 Causes: Poor nutrition green leafy vegetables, citrus fruits, &
beans, nuts, grains; malabsorption syndromes; drugs that
impede absorption (Dilantin); Alcohol abuse; anorexia;
hemodialysis patients
 Clinical Manifestations:
Manifestations similar to cobalamin deficiency –
dyspepsia, smooth, beefy red tongue; absence of neurologic
problems
 Diagnostic Testing: < Folate Level (norm: 3-25mg/ml)
 Medical Management: Replacement Therapy Folic Acid
1mg/day
 Nursing Management: Medication & dietary compliance
 Interferences with Diffusion
Anemias Caused by Decreased Erythrocyte Production

Anemia of Chronic Disease

 Associated with underproduction of RBCs and
decreased RBC survival
 Causes: Renal failure; advanced liver
cirrhosis; chronic inflammation; malignancy;
immunosuppression
 Medical Management:
 Correct underlying disorder
 Erythropoietin Therapy – Epogen, Procrit
 Nursing Management: Care of the debilitated
patient – dietary & medication compliance
 Interferences with Diffusion
Anemias Caused by Decreased Erythrocyte Production

Aplastic Anemia
 Pancytopenia – decrease of all blood cell types ---
RBCs, WBCs, platelets & hypocellular bone
marrow
 Congenital
 Acquired – exposure to radiation & chemicals, post viral
& bacterial infections
 Idiopathic – 70%

 Medical Management:
 Immunosuppressive therapy
 Bone Marrow Transplantation
 Interferences with Diffusion
Anemia Caused by Blood Loss

 Acute Blood Loss

 Hemorrhage
 Decreased oxygen-carrying capacity

 Chronic Blood Loss

 Body maintains its blood volume by slowly increasing plasma
volume < RBCs
 Clinical Manifestations:
 Range from fatigue with melena to orthostatic BP changes to
shock
 Medical Management:
 Treat underlying cause –
 Blood replacement – packed RBCs
 Supplemental Iron
Lab Study Findings in Anemias
 PAIR-SHARE

Name one thing you learned thus far

 Interferences with Diffusion
Anemia Caused by Increased Erythrocyte Destruction
Sickle Cell Disease
 Group of inherited autosomal recessive disorders
characterized by the presence of abnormal Hgb in the
erythrocyte
 Causes the erythrocyte to stiffen & elongate
 Sickle shape in response to lack of oxygen
 Occurrence: 50,000 Americans
 1 in 350-500 African Americans; Mediterranean, So Am; East
Indian, Arabian ancestry
 Types:
 Sickle Cell Anemia: most severe – inherited homozygous for
hemoglobin S (HbSS) from both parents
 Sickle Cell Trait: mild - inherited from one parent + one
normal
 Interferences with Diffusion
Anemia Caused by Increased Erythrocyte Destruction

Sickle Cell Disease

 Sickling Episodes:

 Hypoxemia – triggered by stress, surgery, blood

loss, viral or bacterial infection*(most common),
dehydration, acidosis
 Low oxygen tension in the blood
 Sickled cells cannot easily pass through capillaries
 Hemolyzed in the spleen
 Initially reversible – then becomes irreversible due to chronic
sickling
 Interferences with Diffusion
Anemia Caused by Increased Erythrocyte Destruction
Sickle Cell Crisis

 Severe, painful, acute exacerbation of RBC sickling causing a

vasoocclusive crisis
 Impaired blood flow, vasospasm, severe capillary hypoxia
 RBC Cell membrane permeability changes – plasma loss, & thrombi;
tissue ischemia & infarction
 Sudden & persists for days
 Clinical Manifestation: PAIN – tissue ischemia
 Aching joints—hands & feet
 Organs that have a high need for oxygen are most affected: heart, lungs,
eyes, kidneys, brain
 Spleen scarring & small – auto splenectomy
 Bones – osteoporosis
 Chronic leg ulcers
 Prone to infection – pneumococcal pneumonia
 Medical Management: Hospitalization--Oxygen, rest, fluids &
electrolytes, treat infection, transfusion therapy, Chelation therapy,
pain management
 Bone Marrow Transplant & Gene therapy technology
Sickle Cell Disease Manifestations
 PAIR-SHARE

Priority Nursing Actions

for the client
in
Sickle Cell Crisis
 Interferences with Diffusion
Anemia Caused by Increased Erythrocyte Destruction

Acquired Hemolytic Anemia

 Extrinsic causes of hemolysis:

 Physical – trauma – renal dialysis; CP bypass
 Autoimmune Reactions – medications; systemic
lupus erythematosus; leukemia; lymphoma
 Infectious agents and toxins – parasites; antigen-
antibody reactions; splenomegaly
 Medical Management:
 Corticosteroids; Blood product administration;
splenectomy
 Interferences with Diffusion
Anemia Caused by Increased Erythrocyte Destruction
Polycythemia
 Production o& presence of increased number of RBCs
 Increased blood viscosity – hyperviscosity
 Increased circulating volume – hypervolemia

 Types:
 Primary – polycythemia vera / chromosomal mutation – insidious -- >50 years of age
 Secondary – chronic hypoxia stimulates erythropoietin production in the kidney >
erythrocyte production
 High altitude, COPD, CV disease

 Diagnosis: Elevated RBC, WBC, Platelets; bone marrow aspiration –

hypercellularity of RBCs; splenomegaly

 Medical Management:
 Phlebotomy to maintain HCT 45-48%
 300-500 ml removed every other day until <HCT
 Hydration
 Myelosuppressive therapy: busulfan (Myleran); hydroxyurea (Hydrea) = inhibits bone
marrow production
 Gout – Allopurinol
 Antiplatelet medication: Persantine, Plavix, ASA – prevent thrombotic complicatins
Primary & Secondary
Polycythemia
 Interferences with Diffusion
Problems of Hemostasis
Thrombocytopenia
 Reduction of platelets below 150,000/ul
 ITP: Immune Thrombocytopenic Purpura: autoimmune
 platelets are coated with antibodies; destroyed in spleen
 Women 20-40 years
 Normal survival 8-10 days; ITP: 1-3 days
 TTP: Thrombotic Thrombocytopenic Purpura
 Uncommon syndrome; adults 20-50 years
 Characterized by hemolytic anemia, thrombocytopenia,
neurologic abnormalities, fever, renal abnormalities
 HITT: Heparin-Induced Thrombocytopenia & Thrombosis
Syndrome
 Immune-mediated response to Heparin
 Triggers platelet aggregation
 Decreased platelets & increased thrombosis
 Interferences with Diffusion
Problems of Hemostasis
Thrombocytopenia
 Clinical Manifestations:
 Bleeding:
 Mucosal — epistaxis, gingival, large bullous hemorrhages on
buccal mucosa
 Skin -- superficial ecchymoses; petechiae — flat pin-pointed
red brown microhemorrhages; purpura — numerous petechiae
resulting reddish skin bruising
 Prolonged bleeding – after injections & venipuncture
 Internal bleeding – hemorrhage – any major organ --
orthostatic hypotension -- Cerebral hemorrhage fatal
 Medical Management:
 ITP: Corticosteroid; immunosuppressive therapy;
splenectomy; platelet transfusion
 TTP: Corticosteroids; plasma exchange; plasmapheresis;
splenectomy
 HITT: Discontinue Heparin – Protamine Sulfate
 Interferences with Diffusion
Anemia caused by Blood Loss or
Erythrocyte Destruction
Nursing Management
 Assess: Assess oral cavity, skin, nasal cavity, urine, stool –
occult and overt bleeding; Lab values—CBC, platelet count;
vital signs; signs & symptoms for each blood dyscrasia

 Nsg Action: Ice, packing, direct pressure to control bleeding;

administer medications; No ASA or platelet-acting meds; oral
hygiene, skin care, IV carefully—blood product transfusion
care; Avoid IM/SQ meds; pad rails & firm surfaces; pain
management as needed; post splenectomy care;

 Pt Education: Interpret lab values; Rationale for no sharps –

electric razor; disease process; medications; avoid valsalva;
blow nose gently—one side at a time; signs & symptoms of
bleeding; lab values; health promotion—rest, oxygenation,
 Interferences with Diffusion
Problems of Hemostasis
Hemophilia
Sex-linked recessive genetic disorder caused by
defective or deficient coagulation
Hemophilia Factor VIII Recessive – female carrier;
A Occurs primarily in male

Hemophilia Factor IX Recessive – female carrier;

B Occurs primarily in male

Von vWF and Autosomal dominant; seen in

Willebrand’s platelet both sexes
disease dysfunction
 Interferences with Diffusion
Problems of Hemostasis
Hemophilia – Disease of Childhood
 Clinical Manifestations:
 Slow, persistent, prolonged bleeding from minor trauma and small
cuts
 Delayed bleeding after minor injuries
 Uncontrollable hemorrhage after dental extractions
 Epistaxis, especially after a blow to the face
 GI bleed – ulcers & gastritis
 Hematuria from GU trauma
 Splenic rupture from abdominal trauma
 Ecchymosis & subcutaneous hematomas
 Neurologic signs – pain, anesthesia, & paralysis – from nerve
compression caused by hematoma formation
 Hemarthrosis – bleeding into joints – joint deformity – may cause
crippling

SYMPTOMS OFTEN LEAD TO THE DIAGNOSIS

 Interferences with Diffusion
Problems of Hemostasis

Hemophilia

Time Life Expectancy

 Early 1900’s 11 years
 1970’s 60 years
 1980’s late 40’s

 90% of older people are HIV+

 Many are Hepatitis C+
 Improved blood product testing & screening has decreased
the number of younger people with blood borne sequellae
Coagulation Mechanism
 Clotting Cascade
 XII + Surface: intrinsic path: heparin/ PT
         |
                XIIa
                  |             VIIa + TF: extrinsic path: (warfarin/PT)
                XI---XIa <----/    
         |      
                IX-----IXa                          
             |     
                    VIIIa |      
                          |                                 Thrombomodulin
                      X----------Xa                 --------> Prot. C/S --
                      /           | Va                                 |
          /      |                  |
      Antithrombin III ----->   II-------IIa ----------------------> VIIIa & Va
                  |
                  |
                          Fibrinogen----Fibrin
 Specific Clotting Factors
    fibrinogen (factor I);
- prothrombin (factor II)
    - converts finbrinogen to fibrin
    - activates V, VIII & XIII (when bound to thrombomodulin) ; - activates protein C; - Vit K dependent;
  - factor V:   when activated, serves as enzyme co-factor
  - factor Xa: part of Xa/Va complex which activates prothrombin;
- factor VII:
- part of factor VII/tissue factor complex-activates factor X & IX; - is activated by Xa; - Vit K dependen
- factor VIII: serves as enzyme cofactor to help activate factor X;
- factor IX: - acts w/ IXa/VIIIa/phos complex that activates factor X; - Vit K dependent;
- factor X: - acts as Xa/Va phos complex that actives prothrombin; - Vit K dependent;
- factor XII:
  - protein C:
    - when activated to Ca by thrombin bound to thrombomodulin, inhibits by proteolysis factors VIIIa and
Va in
reactions requiring prot S and phospholipids as cofactors; Vit K dependent;
  - antithrombin III:
    - is a plasma protease inhibitor that serves as a protease scavenger;
    - any of the blood-clotting enzymes that move away from the growing clot rapidly form a complex, and their
activities are neutralized
    - formation of complexes is accelerated by heparin, forms of which are located in the
microvasculature on the surfaces of endothelial cells;
    - inhibitor of the enzymes thrombin, Xa, IXa; Is activated by   heparin;
 Interferences with Diffusion
Problems of Hemostasis
Hemophilia
 Diagnostic Studies:
 Partial thromboplastin time: prolonged
 Bleeding time:
 Prolonged in von Willebrand’s because of structural
defective platelets;
 Normal in Hemophilia A & B
 Factor Assays:
 Factor VIII – A
 Factor IX – B
 vWF – von Willebrand
 Interferences with Diffusion
Problems of Hemostasis
Hemophilia
 Medical Management Goals:
 Preventive Care
 Replacement Therapy during acute bleeding
 May also be given prior to surgery or dental care
 Factor VIII: Examples: Alpohanate, Bicolate, Koate
 Factor IX: Examples: Alphanine, Benefix, Konyne
 Mild Hemophilia A & von Willebrand’s:
 Desmospressin acetate (DDAVP) – causes a release of vWF,
which binds with factor VIII, increasing concentration
 Short-lived; IV/SQ/Intranasal
 Treatment of complications
 Hemophilia
Hematoma of the Ear
 Interferences with Diffusion
Problems of Hemostasis
Hemophilia
 Nursing Management:
 Health Promotion:
 Acute Intervention:
 Stop the topical bleeding – direct pressure, ice, Gelfoam or fibrin foam
packing; topical hemostatic agents – thrombin
 Administer the specific anticoagulant factor
 Joint bleeding: rest; pack in ice; analgesia
 Mobilization when bleeding subsides – P & AROM exercise
 Manage life-threatening complications:
 Airway management – Head and neck injuries
 Neuro signs – Head trauma; spine trauma
 Home Care:
 Patient Education – Age-specific: how to live with illness: increased
oral hygiene; injury prevention; Medic Alert; routine medical follow-up;
non-contact sports; self-administration of replacement factors
 Local chapters of National Hemophilia Society
 Daily oral hygiene
Acute Hemarthrosis – Right Knee
 PAIR-SHARE

What coagulation studies

are affected by hemophilia?
 Interferences with Diffusion
Transfusion Therapy - Indications
 Packed red blood cells – anemia; Hgb < 6-9g/dL depending on
symptoms

 Washed red blood cells – hx allergic rx; bone marrow transplant

patients

 Platelets – thrombocytopenia; active bleeding with platelet count

<80,000

 Fresh frozen plasma – deficiency in plasma coagulation factors

 Cryoprecipitate – Hemophilia VII or von Willebrand’s disease

 WBC’s – Sepsis, neutropenic infection

 Interferences with Diffusion
Transfusion Therapy
 Responsibilities
 Before Transfusion: assess lab values; verify order;
assess pt’s VS, UO, hx of transfusion reactions;
CONSENT; patent venous access; pt ID / Blood
verification RN-RN - document.
 During Transfusion: Appropriate tubing & filter;
normal saline infusion; prescribed rate of
administration; Remain with pt for the first 20 mins of
transfusion; monitor VS and assess for transfusion
rx. – document
 After transfusion: Assess VS; discontinue infusion
& dispose of bag & tubing according to policy;
document; reassess blood work
 Interferences with Diffusion
Transfusion Therapy
 Transfusion reactions
 Hemolytic: blood type or Rh incompatibility – antigen-antibody reaction –
fever, chills, DIC, circulatory collapse (back pain, tachypnea, tachycardiac,
hypotension, chest pain, hemoglobinuria, apprehension

 Allergic: Urticaria, bronchospasm, anaphylaxis – hx of allergy

 Febrile: occurs in pts with anti-WBC antibodies – chills, tachycardiaa, fevere,

hypotension, tachypnea

 Bacterial: Rapid onset: occurs as a result of contamination – tachycardia;

hypotension, fever, chills, shock

 Circulatory Overload: Infused too quickly; Hx CHF – Hypertension, bounding

pulse, distended jugular veins, dyspnea, restlessness, confusion