Hematological System NCLEX Review

Hematological Disorders

Blood and blood products:

Given via 18- or 20- gauge needle for IV access, use filter tubing

(NOTE: Always assess the integrity of IV site before administration of blood products)

Table one: Blood Components

Product	Adverse Reactions	Nursing Considerations
Packed red blood cells	Less common/ Allergic reaction	Compatible solution- 0.9% NACL Monitor client during transfusion Administered over 2-4 hours Standard blood filter tubing
Platelets	Febrile reactions	Compatible solution- 0.9% NACL Nonwettable filter Infuse 4 Units/hr (cannot bolus)
Plasma	Volume overload risk	Administer with straight line set Can bolus infusion in patients in which coagulation factors become unstable
Albumin	Volume overload risk	25% albumin given at 1ml/min Can be used as a bolus in patients with patients in shock
Prothrombin	Risk for hepatitis, allergic reaction	Straight line infusion set
Factor VIII	Allergic and febrile reaction	Drip set or syringe

Table 2 Blood Group Compatibility

Blood Group	Donor	Recipient
A	A, AB	O, A
B	B, AB	O, B
AB	AB	O, A, B, AB
O	O, A, B, AB	O

Nursing Interventions for administering blood products:

1) Ask client about allergies or previous blood transfusion reaction

2) Ensure blood compatibility via type and screen prior to administration

3) Check the line for air bubbles

4) Check blood by two nurses:

Providers order
Client consent
Client ID and MRN number
Blood type

5) Obtain baseline vital signs

6) Prime tubing with 0.9% NACL

7) Start infusion at a slow rate and stay with client for the first 15 minutes

8) Recheck vital signs 15 minutes after transfusion has started

9) If no reaction, increase infusion rate (2 hours/unit) depending on patients cardiovascular status, however, infuse blood products at (4 hours/unit) for elderly patients

10) Vital signs every hour

11) Ask client to report any itching, shortness of breath or flank pain over the kidneys

12) Note: Change entire IV tubing for each unit of blood

If transfusion reaction is suspected:

Immediately stop blood transfusion
Restart 0.9% NACL
Notify the health care provider
Return blood products and blood tubing to blood bank
Obtain CBC, culture and retyping
Collect Urine sample and monitor for hematuria

Table 3 Blood transfusion reactions

Reaction Type

Cause

Symptoms

Nursing Interventions

Allergic reaction/ Hypersensitivity

Hypersensitivity to antibodies present in donors blood

Mild- itching, flushing, urticaria

Anaphylaxis- dyspnea, hypotension, flushing

Prevention with premedication of antihistamines

Stop transfusion and infuse 0.9% NACL

Notify MD

Supportive care: Antihistamines, oxygen, corticosteroids

Acute Intravascular hemolytic reaction

Blood incompatibility

Nausea, vomiting, hypotension, tachycardia, fever, lower back pain, hematuria

Stop transfusion

Notify MD

Supportive care: Manage airway, oxygen, antihistamines

Febrile nonhemolytic reaction

Most common reaction

Antibodies to donor platelets or leukocytes

Nausea, chills, fever, flushing, tachycardia

Stop transfusion

Notify MD

Antipyretics

Sometimes seen with clients after administration of multiple transfusions

Sepsis

Contaminated blood products

Hypotension, tachycardia, high fever, shock

Stop transfusion

Notify MD

Obtain blood cultures

Antibiotic therapy, IV fluids, vasopressors, corticosteroids

Circulatory overload

Large volume in a short amount of time

Crackles, dyspnea

Decrease Infusion rate

Administer Diuretics if appropriate

Monitor high risk clients (elderly, heart disease, children)

Autologous transfusion- Individuals donate their own blood to be given, usually 4-6 weeks before a surgical procedure.

Prevents viral infection from donated blood
Useful in clients with a history of transfusion reactions
Rare blood type

Contraindicated in clients with:

Acute infection
Chronic comorbidities
Vascular disease
Hemaglobin less than 11 g/L or hematocrit less than 33%

General view for Hematological Disorders

Sickle Cell Anemia constitutes a group of diseases termed hemoglobinopathies, in which hemoglobin A is partly or completely replaced by abnormal sickle hemoglobin S.

Sickledex is frequently used because it can be performed on blood from a fingerstick and yields accurate results in 3 minutes.

Sickling response is reversible under conditions of adequate oxygenation and hydration; after repeated sickling, the cell becomes permanently sickled.

Precipitating Factors:

" Fe-E-D-O2 "

Fever
Emotional or physical stress
Dehydration
Oxygen deprivation

Sickle Cell Crisis:

Vaso-Occlusive Crisis caused by stasis of blood with clumping of cells in the microcirculation, ischemia, and infarction

Manifestations: Fever; painful swelling of hands, feet, and joints; and abdominal pain

Splenic Sequestration caused by pooling and clumping of blood in the spleen (hypersplenism)

Manifestations: Profound anemia, hypovolemia, and shock

Hyperhemolytic Crisis an accelerated rate of red blood cell destruction

Manifestations: Anemia, jaundice, and reticulocytosis

Aplastic Crisis caused by diminished production and increased destruction of red blood cells, triggered by viral infection or depletion of folic acid

Manifestations: Profound anemia and pallor

Nursing Interventions:

Maintain adequate hydration
Administer oxygen and blood transfusions as prescribed
Administer analgesics as prescribed (around the clock)
Provide comfort and rest
Diet: high-calorie, high protein diet, with folic acid supplementation
Administer antibiotics as prescribed
Ensure that the child receives pneumococcal and meningococcal vaccines and an annual influenza vaccine
Monitor for signs of complications: increasing anemia, decreased perfusion, and shock (mental status changes, pallor, vital sign changes).

Avoid meperidine for pain is avoided because of the risk of normeperidine-induced seizures.

Iron Deficiency Anemia red blood cells are "microcytic and hypochromic"; depleted iron production, resulting in a decreased supply of iron for the manufacture of hemoglobin in red blood cells.

Signs and Symptoms:

Pallor
Weakness and fatigue
Low hemoglobin and hematocrit levels

Nursing Interventions:

Increase oral intake of iron; iron-fortified formula or supplements
Diet: Iron rich foods
Iron supplements administration:

Give between meals
Give with multivitamin or fruit juice to increase absorption
Do NOt give with milk or antacids
Can cause (black stools, constipation, and foul aftertaste)
Use straw when administering liquid iron supplements
Instruct good oral hygiene

Aplastic Anemia red blood cells are "normocytic and normochromic" but would be reduced in number (rbc,wbc,pc).

Signs and Symptoms:

" 4PS "

Pancytopenia (deficiency of erythrocytes, leukocytes, and thrombocytes)
Pallor
Purpura
Petechiae

Nursing Interventions:

Prepare the child for bone marrow transplantation
Administer immunosuppressive medications as prescribed (Corticosteroids and cyclosporine)
Administer blood transfusions if prescribed and monitor for transfusion reactions

Hemophilia a bleeding disorder resulting from a deficiency of specific coagulation proteins (clotting factor VIII hemophilia A or Classic hemophilia and IX hemophilia B or Christmas disease. X-linked recessive disorder; Male (affected) > Female (carrier).

Signs and Symptoms:

Abnormal bleeding in response to trauma or surgery (sometimes after circumcision)
Epistaxis (nosebleeds)
Joint bleeding causing pain, tenderness, swelling, and limited range of motion
Ecchymosis

Nursing Interventions:

Maintain bleeding precautions
Avoidance of contact sports
Prepare to administer factor VIII concentrates
DDAVP (1-deamino-8-D-arginine vasopressin), a synthetic form of vasopressin, increases plasma factor VIII to treat mild hemophilia.
Monitor for joint pain; immobilize the affected extremity if joint pain occurs.
Assess neurological status (child is at risk for intracranial hemorrhage)
Monitor urine for hematuria
Control joint bleeding; apply pressure (15 minutes) for superficial bleeding

von Willebrand’s Disease is a hereditary bleeding disorder that is characterized by a deficiency of or a defect in a protein termed von Willebrand factor.

Signs and Symptoms:

" EEEG "