Week 13 - Hematology Part 2

Thalassemia

Thalassemia

a hereditary anemia

Mediterranean, African, and Southeast Asian ancestry

Thalassemia is most often associated with what ancestry?

defective synthesis of hemoglobin, αlpha-globulin or beta-globulin genes

The cause of thalassemia is

Hypochromia
Extreme microcytosis
Hemolysis

Thalassemia is characterized by

Hypochromia

an abnormal decrease in the hemoglobin content of erythrocytes

Extreme microcytosis

smaller-than-normal erythrocytes

Hemolysis

Destruction of red blood cells

1. Onset is usually insidious
2. Signs are primarily related to progressive anemia, expansion of marrow cavities of the bone and development of hemosiderosis
3. Early signs often include progressive pallor, poor feeding and lethargy
4. Further signs: hemorrhage, bone pain, exercise intolerance, jaundice, & protuberant abdomen
5. Hemosiderosis of the eye and lungs

Clinical manifestations of thalassemia

Onset
Signs
Early signs
Further signs
Effects to eyes and lungs

1. Family history
2. Certain ancestry

Risk factors of Thalassemia

Thalassemia is passed from parents to children through mutated hemoglobin genes.

Explain family history as risk factor in Thalassemia

Thalassemia occurs most often in African Americans and in people of Mediterranean and Southeast Asian descent.

Explain certain ancestry as risk factor in Thalassemia

1. Decrease hemoglobin
2. RBC = increase in number
3. Hgb electrophoresis
4. Chorionic villus sampling and Amniocentesis- prenatal testing

Diagnostic evaluation of thalassemia

Hgb electrophoresis

measures hemoglobin levels and looks for abnormal types of hemoglobin.

➢ Iron overload
➢ Infection
➢ Bone deformities
➢ Enlarged spleen
➢ Slowed growth rates
➢ Heart problem

Complications of thalassemia

1. Frequent and regular transfusion of packed RBC's to maintain Hgb levels above 10 g/dL
2. Iron chelation therapy with deferoxamine (Desferal)
3. Splenectomy
4. Supportive management of symptoms
5. Bone marrow transplant

Management of thalassemia

Iron chelation therapy with deferoxamine (Desferal)

reduces toxic effects of excess iron & increases iron excretion thru urine & feces

Poor because no known cure

Prognosis and survival rate of thalassemia

late adolescence or early adulthood

Thalassemia often fatal in

Polycythemia vera

a type of blood cancer that causes bone marrow to make too many red blood cell

thicken your blood, slowing its flow, which may cause serious problems, such as blood clots.

The excess cells in polycythemia vera does what

1. Reddish purple hue of skin & mucosa, pruritus
2. Splenomegaly, hepatomegaly
3. Epigastric discomfort, abdominal discomfort
4. Painful fingers & toes from paresthesias
5. Altered mentation
6. Weakness, fatigue, night sweats, bleeding tendency
7. Hyperuricemia

Assessment of polycthemia vera

Hyperuricemia

is when you have too much uric acid in your blood.

CBC
Bone marrow aspiration and biopsy

Diagnostic tests for PV

➢ Blood clots
➢ Enlarged spleen
➢ Problems due to high levels of red blood cells
➢ Other blood disorders

Complications of PV

Hyperviscosity
Hyperplasia
Hyperuricemia
Pruritus

Management of PV could depend on four things

phlebotomy at intervals determined by CBC results to decrease RBC mass

Management of PV (Hyperviscosity)

250 - 500 mL

When treating hyperviscosity, generally [___] removal at a time

1. myelosuppressive therapy, generally using hydroxyurea or IV
2. radioactive phosphorus (32P)

Management of PV (Hyperplasia)

hydroxyurea or IV

myelosuppressive therapy uses

Allupurinol M (Zyloprim)

Management of PV (Hyperuricemia)

Antihistamines (Cimitidine)

Management of PV (Pruritus)

1. Encourage/assist ambulation
2. Assess for early signs of thromboembolic complications
3. Monitor CBC & assist with phlebotomy as ordered

Nursing intervention for PV

swelling of limbs,
increased warmth,
pain

early signs of thromboembolic complications include

●Educate about risk of thrombosis;
● encourage patient to maintain normal activity pattern & avoid long periods of rest
● Avoid hot showers
● Report at regular intervals for follow up blood works

Patient education for PV

Hemophilia

a rare disorder in which the blood doesn't clot in the typical way

Blood doesn't have enough blood clotting proteins (clotting factors)

Why does hemophilia happen?

Hemophilia A (Classic Hemophilia)
Hemophilia B (Christmas Disease)

Types of hemophilia

Factor VIII deficiency (Antihemophilic Factor/AHF)

What is lacking in Hemophilia A?

Factor IX deficiency (Christmas Factor)

What is lacking in Hemophilia B?

1. Intrinsic Pathway - Activated by damage to the blood vessel.

2. Extrinsic Pathway - Activated by external trauma that causes blood to escape from the vessel.

3. Common Pathway - Both pathways converge here

Explain the coagulation process

Factors XII, XI, IX, VIII

Intrinsic pathway involves

Factors III and VII

Extrinsic pathway involves

Factors X, V, II (prothrombin), and I (fibrinogen)

Common pathway involves

● Abnormal bleeding in response to trauma or surgery. (muscles/joints)
● Joint bleeding causing pain, tenderness, swelling, and limited range of motion.
● Tendency to bruise easily.
● Epistaxis
● Hemarthrosis

Assessment of hemophilia

1.Administer factor VIII/ IX concentrate.
2. Monitor for bleeding and maintain bleeding precautions.
3. Monitor for joint pain; IMMOBILIZE the affected extremity if joint pains occur.
4. Monitor urine for hematuria.
5. Instruct re: activities, NO CONTACT SPORTS
6. Instruct on how to control bleeding
7. DDVAP (Desamino D-Arginine Vasopressin/Desmopressin)
8. Soft toothbrush + regular dental check ups
9. Refer to national hemophilia association
10. Avoid Aspirin
11. Provide diet information

Implementation for hemophilia

Immobilize the affected extremity

What to do if there is joint pain in hemophilia?

DDVAP - Desamino-D-Arginine Vasopressin (Desmopressin)

promotes the release of Factor VIII in hemophilia A

R - Rest
I - Ice
C - Compression
E - Elevation

What does RICE mean in treating hemophilia?

R - No weight on injury for first 24 to 48 hours

I - ice for 15 to 20 minutes every 3 to 4 hours

C - compress injury to reduce swelling

E - elevate above the level of the heart

Explain RICE and how they work

➢ Exercise regularly.
➢ Avoid certain pain medications.
➢ Avoid blood-thinning medications. ➢ Practice good dental hygiene..
➢ Get vaccinations.
➢ Protect your child from injuries that could cause bleeding.

Health teaching for hemophilia

Disseminated Intravascular Coagulation

coagulation disorder that prompts overstimulation of the normal clotting cascade

simultaneous thrombosis and hemorrhage

DIC results in

● Purpura on lower extremities & abdomen
● Hemorrhagic bullae, acral cyanosis, focal gangrene in skin

Assessment of DIC

1. Marked decrease of blood platelets
2. Low levels of fibrinogen and other clotting factors
3. Prolonged prothrombin and partial thromboplastin times and abnormal erythrocyte morphologic characteristics

Diagnostic test of DIC

1. Monitor for signs of bleeding of all hema tests : blood, urine, stool, git
2. Administer IV fluid replacement as ordered
3. Administer oxygen inhalation as ordered
4. Administer medications as ordered (Vitamin K, Heparin, Pitressin (Vasopressin) via heplock)
5. Provide Heplock, avoid IM, SQ and any venipunctures
6. Institute NGT decompression
7. Prevent complications

Nursing management of DIC

Idiopathic Thrombocytopenia Purpura

illness that can lead to bruising and bleeding

Low levels of the cells that help blood clot, also known as platelets, most often cause the bleeding.

What causes Idiopathic Thrombocytopenia Purpura?

150,00 to 450,00/ml of blood

Normal platelet count

increased bleeding vulnerability

Low platelet count could lead to

stroke

high platelet count could lead to

●Petechiae
● Ecchymosis
● Blood in any body secretions, bleeding form mucous membranes, nosebleeds.
● Decreased platelet count
● Anemia
● Easy bruising
● Blood in stool or urine
● CBC reveals platelet count below 20,000/mm3
● Bone marrow aspiration done to rule out leukemia

Assessment of ITP

Ecchymosis

bruises under the skin that are formed due to a break in the blood vessel

Drug therapy
1. Prednisone
2. IVIG (intravenous immune globulin)
3. Anti-D Antibody

Medical management of ITP

Drug therapy
1. Prednisone
2. IVIG (intravenous immune globulin)
3. Anti-D Antibody

- decreases anti-platelet antibodies

IVIG

helps to effectively increase platelet count

Anti-D Antibody

a one dose treatment for ITP

●Given to pt's 1 year but less than 19 years old
● Normal WBC and hemoglobin
● no active bleeding present
● no concurrent infection
● Diphenhydramine and hydrocortisine are made ready for possible allergic reaction

Considerations of Anti-D Antibody

●Prevent, control and minimize bleeding.
● Prevent bruising
● Provide support to client and be sensitive to change in body image.
● Protect from infection.
●Administer analgesics (acetaminophen) as ordered; avoid aspirin.
● Administer meds orally, rectally, or I.V. rather than I.M.

Nursing management of ITP