Hematologic Disorders

Jan Leynard Camposagrado, RN, USRN, MN

Hematologic System
- Consist of blood, bone marrow and reticuloendothelial system

- Blood is also considered an ORGAN

- Plasma is the fluid portion of the blood (55%)

Blood
- Composed of: RBC, WBC and Platelets

- 45% of blood volume

- Carries oxygen, nutrients and wastes from system - Also carries hormones and antibodies

Bone Marrow
- Site of hematopoiesis

- Marrow activity is limited to pelvis, ribs, vertebrae and sternum in adult

- Liver and spleen resume blood production in marrow destruction - Within the bone marrow are stem cells capable of differentiation

myeloid

lymphoid

Slide 002

Myeloid Stem Cell

Precursor for:

neutrophils monocyte eosinophils erythrocyte basophils mast cells megakaryocyte

Lymphoid Stem Cell

Precursor B: B-lymphocyte Precursor T: T-lymphocyte

Red Blood Cells

- Disk shape provides large surface for ABSORPTION and RELEASE of oxygen molecules

- Primary function - 120 days and removed by the reticuloendothelial cells - Most of the iron is recycled to form new hemoglobin
- 5-day process of erythropoiesis

White Blood Cells

- Protect the body from invasion by bacteria and other foreign entities
- Neutrophils arrive at the site within 1 hour of inflammation - Initiate phagocytosis but short-lived - Monocytes follow and continue phagocytosis for longer periods - Macrophages are effective against fungi and viruses

Lymphocytes
T-Lymphocytes
- Releases lymphokines for enhanced phagocytic activity - Responsible for delayed allergic reaction and foreign tissue rejection - Responsible for destruction of tumor cells

3 Main Groups of T-Lymphocytes 1. Killer T-cells

- Sensitized and stimulated in the presence of antigen - Destroy abnormal cells

2. Helper T-cells
- Enhance activities of T-cells & B-cells - Reduced in patients with HIV

3. Suppressor T-cells
- Have the effect of switching off the immune system
- Controls too much T-cells

B-Lymphocytes
- Releases immunoglobulin or antibodies

Eosinophils & Basophils

- Function in hypersensitivity reaction - Eosinophils are impt in the phagocytosis of PARASITES

T cells on the right, platelet on the center and rbc at the left

Platelets
- For clotting mechanism - Circulate freely in an inactive state in the endothelium - Normal life span of 7-10 days

Diagnostic Findings
Test Red Blood Cells Hemoglobin Normal Range M: 4.7 6.1 x 106 F: 4.2 5.4 x 106 M: 13.5 17.5 g/dL F: 11.5 15.5 g/dL Delivers oxygen through circulation to body tissues and returns CO2 from tissues to lungs Description

Hematocrit Platelets

M: 40 52% F: 36 48% 150, 000 400, 000/mm3

Indicates proportion of plasma and RBCs 20, 000/mm3 serious; 10, 000/mm3 life-threatening

Test
White blood cells

Normal Range
5,000 10,000/mm3

Description

Neutrophils

40 75%

Indicates bacterial infection, inflammation, stress and steroids. Increased after bacterial and viral infection

Lymphocytes

20 50%

Monocytes

1 10%

Increased in acute and chronic infection, and inflammation Increased in allergic states, parasites and metastatic tumors Increase is very rare

Eosinophils

0 6%

Basophils

0 2%

Anemia
- Hemoglobin concentration is lower than normal
- Lower RBC than normal lower amount of oxygen delivered

Three Broad Etiologic Categories:

1. Loss of RBC due to bleeding

2. Decreased production of RBC - Deficiency in co-factors

- Lack of erythropoietin

3. Increased destruction of RBC - Overactive RES

- Abnormal RBC

Classifications of Anemia
1. Hypoproliferative - marrow cannot produce adequately - due to medications - lack of co-factors

II. Hemolytic Anemia - premature destruction of RBC

Nursing Diagnosis
1. Activity Intolerance r/t weakness, fatigue & general malaise 2. Imbalanced Nutrition, less than body requirement r/t inadequate intake of essential nutrients 3. Ineffective tissue perfusion r/t inadequate blood volume

Hypoproliferative Anemia
A. Iron Deficiency Anemia
- Inadequate intake of Iron resulting to low manufacture of hgb - Most common anemia - Most common cause in male adult & post menopausal women is bleeding - Most common cause in pre-menopausal women is menstruation - Alcoholism causes GIT blood loss

Assessment
1. Pallor

2. Weakness and fatigue

3. Irritability

Diagnostic Finding
Bone Marrow Aspiration - Most definitive - Low ferritin level - Seldom used

Medical Management
1. Iron Preparation - Ferrous sulfate, Ferrous gluconate 6 month therapy 2. Iron Dextran - IM for poor iron absorption

- ANST before giving the full dose

Nursing Interventions
1. Give iron supplements between meals 2. Given with vit. C or multivitamins or fruit juice 3. Dont give iron with milk or antacid 4. Use a straw in liquid iron forms 5. Instruct pt. of S/E: constipation, black stool, foul aftertaste 6. Instruct high iron foods: Liver, bread and cereals, dark green leafy, meat, raisins, egg yolk, beans

B. Aplastic Anemia
- Rare disease where there is decrease or damage to marrow stem cells FAT - Significant neutropenia + thrombocytopenia are also seen

Causes:
1. Idiopathic 2. Exposure to myelotoxic agents, autoimmune disorder

- benzene, gold compounds, chloramphenicol, arsenic, sulfonamides

Diagnostic Finding
Bone Marrow Aspiration
- Most definitive - Demonstrates conversion of red bone marrow to fatty red bone marrow

Assessment
1. Pancytopenia 2. Petechiae, purpura, bleeding, pallor, weakness, tachycardia, fatigue 3. Infections are common

Medical Management
1. Bone Marrow Transplantation
- Treatment of choice if a suitable donor exists

2. Immunosuppressive Therapy
- Presumed that pts lymphocytes destroy the stem cell - Antithymocyte & cyclosporine

3. Supportive Therapy
- Transfusion of RBC and platelets
- Discontinue any offending agents

Nursing Interventions
1. Administer immunosuppressive medications

2. Administer blood transfusion as prescribed

3. Advise pt to obtain Medic-Alert bracelet 4. Assess carefully for signs of infection and bleeding

Hemolytic Anemia
- RBCs have a shortened lifespan

- RBC = decreased available O2 hypoxia

A. Sickle Cell Anemia

- Hemoglobin (hemoglobin A) is partly or completely replaced by an abnormal sickle hemoglobin (hemoglobin S) - Happens when there is decreased O2 tension

- Insufficient O2 causes the cell to assume a sickle shape causes clumping together and later obstruction e.g. fever, emotional and physical stress, cold environment - Genetic factor - Sickling response is reversed when provided with adequate O2 and hydration - After repeated sickling, the cell become permanently sickled.

Manifestations
Spleen
Lungs CNS Kidneys Bone Skin Eye

Primary site of sickling infarction and phagocytosis by macrophage

Infarction pulmonary HPN Infarction Sickling damage to renal medulla Increased erythroid production (compensation) Infarction Infarction Pulmonary infiltrate CVA Hematuria, renal failure

infection
Chest pain, dyspnea, Weakness Dehydration

Widening of medullary Bone pain spaces Skin ulcer Scarring and hemorrhage Pain , decrease healing Decreased vision; blindness

Sickle Cell Crisis

1. Vasoocclusive Crisis
- Most common
- Stasis of blood with clumping of the cells in the microcirculation, ischemia and infarction necrosis - s/sx: fever, pain and tissue engorgement

2. Splenic Sequestration
- Life threatening caused by pooling of blood in the spleen (autosplenectomy) - Liver and Lungs are involved in adults - s/sx: profound anemia, hypovolemia and shock

3. Aplastic Crisis
- Results from infection with the human parvovirus
- Causes production of RBC to shut down for about 10 days and level falls rapidly & marrow cannot compensate

- s/sx: anemia and pallor

Medical Management
1. Hydroxyurea
- Chemotherapeutic agent used to decrease permanent formation of sickle cells
- S/E: a. Chronic suppression of WBC

b. Potential development of malignancy

2. Arginine
- Enhances nitric oxide potent vasodilator to decrease pulmonary pressure

3. Transfusion Therapy
- Long term RBC transfusion - Risks include: a. Iron Overload b. Infection and thrombosis from the site c. Hepatitis and HIV d. Significant financial cost

3. Supportive Therapy
PAIN significant issue

a. Lasting hours to days b. Adequate hydration is important c. O2 may also be required d. Pain medication may be given (aspirin, nsaids, morphine) e. Physiotherapy can also be used (heat, massage) f. Cognitive and behavioral intervention (distraction, relaxation)

Nursing Interventions
1. Managing pain - Make use of pain scale - Relaxation and breathing exercises 2. Preventing and Managing Infection - Monitor s/sx of infection 3. Minimizing deficient knowledge - inform: Keep warm Maintain hydration Avoid stressful events

B. G-6-PD
- Glucose-6-Phospate Dehydrogenase Deficiency - G-6-PD produces an enzyme w/in the erythrocyte for membrane stability - Hemolysis only results when erythrocytes are stressed - Commonly affected: African-American, Greek, Italian, Asian and Jewish - X-linked defects

- Medications causing hemolysis:

1. Anti-malarial 2. Bactrim/Septra 3. Aspirin 4. Vit. K

5. HydroDIURIL 6. OHA 7. Chloramphenicol

Assessment
1. Asymptomatic most of the time 2. Pallor, jaundice and hemoglobinuria

3. Hemolysis is often mild and self limiting

Diagnostic Test
1. Screening Test (NB Screening) 2. Quantitative Assay of G-6-PD

Medical Management
1. Stop the offending medications 2. Transfusion if severe hemolysis occurs (Mediterranean Type)

Nursing Management
1. Pt is educated and given a list of medications to avoid 2. Medic-Alert

Polycythemia Vera
- Uncontrolled mechanisms of the myeloid stem cells resulting to hypercellular bone marrow activity. - erythrocyte, leukocyte and platelet counts are elevated
Manifestations:
a. Increase blood volume - headache, dizziness, tinnitus, fatigue, paresthesia and blurred vision.
b. Increase blood viscosity - angina, claudication, dyspnea, thrombophlebitis

c. Increase BP d. Generalized pruritus e. Splenomegaly

Diagnostic Test
1. Elevated erythrocyte count

2. Increased leukocyte and platelet

3. Elevated hematocrit (60%)

Complications
1. Thromboses - CVA - MI 2. Bleeding - Very large platelets but dysfunctional

Medical Management
1. Phlebotomy - to diminish blood viscosity

2. Anagrelide (Agrylin) inhibits platelet aggregation

3. Interferon Alpha-2b (Intron-A) for pruritus - anti-histamines are not effective 4. Chemotherapeutic Agents (Hydroxyurea) - Suppress bone marrow but increases risk for leukemia

- Lowers incidence of thrombolytic complications than phlebotomy

Nursing Management
Nurses role is primarily an EDUCATOR
1. Avoid smoking, obesity, poorly controlled hypertension
2. Avoid tight or restrictive clothing (stockings) 3. Avoid crossing legs 4. Avoid aspirin

5. Minimize alcohol intake (GI bleeding)

6. Avoid Iron Preparations 7. Bathe in tepid or cool water or sodium bicarbonate

Hemophilia
Hemophilia A - Deficient or defective factor VIII Hemophilia B - Deficient or defective factor IX - Both hemophilias are inherited as X-linked traits - Females are carriers and are asymptomatic

Manifestations
1. Joint bleeding (hemarthrosis) 75% of all bleeding - knees, elbows, ankles, hips, wrist and shoulders - Recurrent joint hemorrhages can result to ankylosis 2. Hematoma can occur without known trauma in severe factor deficiency 3. Damage sensation of the peripherals, weakness and atrophy 4. Spontaneous hematuria and GI bleeding 5. Most dangerous site of bleeding is the head

Medical Management
1. Infusion of Factor VIII and IX - Given during active bleeding or as preventive measure before procedures

- Patient and family are instructed how to administer by IV at home - Patient sometimes develop antibodies to the concentrate - If so, we give Recombinant Factor VIIa
2. Aminocaproic Acid (Amicar) - Used to treat mucosal bleeding

3. Demopressin (DDAVP) - Used for pt with mild forms of Hemophilia A

Nursing Management
A. Extensive teaching about activity restrictions and self care measures
1. Avoid aspirin, NSAIDs, herbs and alcohol 2. OTC cold remedies are avoided 3. Dental hygiene to avoid dental extractions 4. Nasal packing is avoided 5. Splint joints for signs of bleeding 6. Avoid all injections if possible 7. Invasive procedures should be minimized or performed after administration of factor replacements 8. Frequently assess surgical sites post surgery 9. Wear Medic-Alert bracelet 10. Frequent v/s monitoring

B. Analgesics for pain associated with hematoma and hemorrhage to joints C. Warm bath promotes relaxation

Procuring blood and blood products

Donors should be in good health and without any of the ff:
1. History of viral hepatitis 2. History of blood transfusions 3. History of untreated syphilis or malaria 4. History of drug abuse of IV drug use 5. Skin infections 6. Recent history of asthma, urticaria or allergy to medications 7. Pregnant 8. History of untreated exposure to infectious diseases 9. Recent tattoo 10. Cancer 11. Aspirin usage within 48-72

All donors are expected to meet the ff minimal requirements:

1. Body weight should exceed 50 kg (110lbs) for 450 mL donation

2. 17 y.o are disqualified

3. Oral temp should not exceed 37.5C 4. PR should be regular between 50-100 bpm 5. BP of 90/60 180/100 6. Hgb should be at least: women - 12.5g/dL men - 13.5g/dL

Complications of Blood Transfusion

A. Febrile Non-Hemolytic Reaction
- Reaction of pts antibodies to donor WBC - s/sx: chills and fever - Antipyretics for fever

B. Acute Hemolytic Reaction

- Most dangerous and life threatening

- Pt antibodies combine with donor RBC and start hemolysis - Incompatibility of blood (hemolysis) - Errors in blood component labeling and patient identification - s/sx: fever, chills, low-back pain - n/v, chest tightness, dyspnea - hypotension, bronchospasm

Management:
- Discontinue immediately - Meticulous attention to labels - Accurate identification of recipient - Maintain blood volume

C. Allergic Reaction
- Due to hypersensitivity to allergens found in the transfused blood - s/sx: urticaria, itching, flushing Management: - anti-histamines for mild reactions

- Epinephrine and corticosteroid

D. Circulatory overload
- Too much blood infused - s/sx: dyspnea, orthopnea, tachycardia, sudden anxiety - Jugular vein distention, increased BP - Crackles at the base of the lungs - if noted, transfusion is discontinued - Notify physician - KVO with NSS

E. Bacterial Contamination
- Incidence is very low - Usually due to organisms on the donors skin - Most bacteria cannot survive in cold temperature used to store RBC platelets are at greater risk s/sx: - Fever, chills & hypotension - Transfusion is discontinued - IV with NSS - Notify physician and blood bank is informed - Blood container is returned to blood bank for C & S test