Hematology

Autoimmune Idiopathic Thrombocytopenic Purpura (ITP)

• Pathophysiology:
    - Characterized by platelet destruction by the immune system leading to increased risk for bleeding., affecting kids 2-6

• Etiology:
    - Acute viral illness: rubella or chicken pox, HIV and Hep B can be triggers
    - Drug reactions
    - Immune system dysfunction

• Signs and Symptoms of ITP
    - Bleeding tendencies
    - Petechiae (small red or purple spots on the skin)
    - Ecchymosis (bruising)

• Diagnostic Tests for ITP
    - Platelet count: less than 20,000/mm³ indicates severe thrombocytopenia
    - Bleeding time assessment
    - Bone marrow aspiration to evaluate the production of platelets

• Therapeutic Interventions for ITP
    - May resolve without treatment
    - Corticosteroids to reduce immune response
    - Chemotherapy if chronic and severe
    - Transfusions in cases of persistent bleeding
    - Intravenous Immunoglobulin (IVIG) therapy
    - Vitamin K supplements
    - Splenectomy (surgical removal of spleen may be considered)

• Nursing Care for ITP
  Implement bleeding precautions for patient safety, Recognize and report signs and symptoms of bleeding, Educate the patient and family about the condition and precautions needed.

• Bleeding Precautions for ITP Patients
    - Use an electric razor instead of a blade
    - Use a soft toothbrush for oral hygiene
    - Avoid invasive procedures or injections unless absolutely necessary
    - Maintain pressure on the site if blood draws are essential
    - Exercise caution when measuring blood pressure
    - Wear shoes or slippers for foot protection
    - Avoid situations that may cause bumps and bruises
    - Avoid aspirin and NSAIDs (non-steroidal anti-inflammatory drugs)
    - Administer stool softeners to prevent straining
    - Handle the patient gently during care
    - Blow nose gently to avoid bleeding.

Hemophilia

• Pathophysiology:
    - A condition characterized by missing clotting factors:

  • Hemophilia A: Factor VIII deficiency

  • Hemophilia B: Factor IX deficiency

• Etiology:
    - Primarily hereditary.

• Signs and Symptoms of Hemophilia
    - Episodes of bleeding in joints, muscles, subcutaneous tissue, and even intracranially (brain).

• Diagnostic Tests for Hemophilia
    - Partial Thromboplastin Time (PTT)
    - Measurement of specific factor levels (Factor VIII(8) or Factor IX(9))

• Therapeutic Interventions for Hemophilia
    - Desmopressin (available as injection or nasal spray to stimulate release of Factor VIII)
    - Replacement of clotting factors (Factor VIII or Factor IX)
    - Blood transfusions as needed during bleeding episodes

• Nursing Diagnoses for Hemophilia
    - Acute Pain
    - Risk for Bleeding

Polycythemia

• Definition: A condition characterized by an overabundance of RBCs in the bloodstream

• Laboratory Values:
    - Hemoglobin > 18 milligrams per deciliter
    - Hematocrit > 55%

• Impact: Thickening of the blood, leading to increased risk of clotting.( hyperviscosity)

• Signs and Symptoms of Polycythemia
    - Hypertension
    - Visual changes
    - Headache
    - Vertigo
    - Dizziness
    - Tinnitus
    - Bleeding
    - Chest pain
    - Dyspnea (shortness of breath)
    - Dark, flushed skin
    - Itching

• Therapeutic Interventions for Polycythemia
    - Phlebotomy (removal of excess blood, that blood can not be used for transfusion it is just discarded)
    - Low-dose aspirin to reduce clotting risk
    - Radiation therapy & Chemotherapy in severe cases: to prevent production of the cells
    -   - Medication for itching (antihistamines or corticosteroids)

• Patient Education for Polycythemia
    - Drink 3 liters of water daily
    - Avoid restrictive clothing
    - Elevate feet when possible
    - Report signs of iron deficiency
    - Report any signs of bleeding

Sickle Cell Anemia

• Definition: An inherited autosomal recessive disorder ( both parents need to pass it on, if only one it will give to the child and make them a carrier) characterized by the presence of sickle-shaped RBCs., hemoglobin is sensitive to o2 changes, when there is a decrease in oxygen the cell turns from biconclave to sickle shaped, RBC last only 10-20 days

• Etiology: african american,

• Signs and Symptoms of Sickle Cell Anemia

• Brain:
    - Thrombosis
    - Hemorrhage
    - Stroke (brain attack)

• Eye:
    - Retinal or conjunctival hemorrhage
    - Blindness

• Heart:
    - Heart failure

• Abdominal Organs:
    - Hepatomegaly
    - Gallstones
    - Splenic enlargement
    - Splenic infarction

• Bones and Joints:
    - Hand and foot syndrome

• Reproductive System (Penis):
    - Priapism (painful erection)

• Lungs:
    - Atelectasis (collapsed lung)
    - Infarction
    - Pneumonia

• Kidneys:
    - Dilute urine
    - Increased urination (diuresis)
    - Hematuria (blood in urine)

• Skin:
    - Stasis ulcersDiagnostic Tests and Treatments for Sickle Cell Anemia

• Diagnostic Tests:
    - Sickledex test: takes sample of blood, looking for specific abnormalities and will look at a period of time when the person is having low o2 stats, such as in a crisis.
    - Hemoglobin electrophoresis: take the blood and look for very specific part of the hemoglobin that is consisted to sickle cell anemia
    - CBC: decreased WBC, hemoglobin, and hematocrit
    - Erythrocyte sedimentation rate (ESR): decreased 

• Treatment Options:
    - Bone marrow transplant (definitive treatment)

• Crisis Treatment:
    - Antibiotics
    - Hydroxyurea (reduces sickle cell crisis frequency/pain)
    - Pain management
    - Blood transfusions
    - Fluid administration: dilutes
    - Oxygen supplementation
    - Preventive Measures:
      - Low-dose penicillin: for prophylaxis
      - Frequent transfusions
      - Treatment with Hydroxyurea

Patient Education for Sickle Cell Anemia

• Recommendations:
    - Avoid tight clothing: this can decrease amount of O2
    - Avoid strenuous exercise
    - Avoid alcohol
    - Avoid cold temperatures
    - Avoid smoking
    - Avoid unpressurized aircraft
    - Minimize exposure to infections

Capillary fragility test: blood pressure cuff, keep on 5 min, release, see distaole if they have hemacological conditions, you will see a lot of petechia

• Types of Blood Products: 

• Packed RBCs (PRBCs): one unit of blood is 350ml, normally stored in frozen capacity, dethaw blood for an hour, 

• Frozen RBCs

• Platelets: 

• Albumin: albumin to keep it in our intravascular space

• Fresh Frozen Plasma

• Cryoprecipitates: specific clotting factors that are man made