Hematology

Anatomy & Physiology of the Hematological System

  • Components:
      - White blood cells
      - Red blood cells
      - Platelets
      - Spongy bone
      - Compact bone
      - Blood vessels within bone marrow
      - Yellow marrow

Hematologic Diseases Overview

  • Alteration in Hemoglobin & Nutritional Anemia:
      - Iron Deficiency Anemia
      - Sickle Cell Anemia
      - Beta-Thalassemia (Cooley Anemia)

Iron Deficiency Anemia: Pathophysiology

  • Key Components:
      - Red blood cells (RBCs)
      - Hemoglobin molecule needs iron to carry oxygen molecule effectively.

Iron Deficiency Anemia

  • Etiology:
      - Affects infants aged 9 to 24 months.
      - Older children can also be affected.

  • Clinical Presentation:
      - Common Manifestations:
        - Pallor
        - Fussiness
        - Fatigue
        - Tachycardia
        - Sore or swollen tongue
        - Splenomegaly
        - Pica (craving non-nutritive substances)
      - Additional Manifestations:
        - Restless leg syndrome
        - Stomatitis (inflammation of the mouth)
        - Glossitis (inflammation of the tongue)
        - Increased susceptibility to infections
        - Decline in school performance

  • Lab Testing and Diagnostic Testing:
      - Complete Blood Count (CBC)
      - Hemoglobin (HgB) levels > 11 g/dL

  • Healthy RBCs vs. Iron Deficiency Anemia comparison to illustrate differences in morphology and functionality.

Treatment and Therapies for Iron Deficiency Anemia

  • Iron supplementation:
      - Recommended doses range from 1 to 6 mg/kg/day.

  • Iron-rich foods include:
      - Red meats (easiest for the body to absorb)
      - Tuna and salmon
      - Eggs
      - Tofu
      - Enriched grains
      - Dried beans, peas, and fruits
      - Leafy green vegetables
      - Iron-fortified breakfast cereals

Sickle Cell Anemia

Pathophysiology and Morphology

  • Morphological change leads to sickle-shaped hemoglobin, causing molecules to stick together and clump.

Clinical Presentation

  • Symptoms include:
      - Anemia
      - Low hemoglobin
      - Pallor
      - Fatigue
      - Acute and chronic pain
      - Hyposplenism (reduced spleen function)
      - Decreased activity levels
      - Extremity swelling

  • Vaso-occlusive crisis:
      - Characterized by pain, tissue infarction, and increased infection risk.

  • Triggers:
      - Some situations can precipitate crises.

  • Acute chest syndrome:
      - Signs include chest pain, cough, respiratory distress, fever, and hypoxemia.

Complications of Sickle Cell Anemia

  • Potential adverse outcomes include:
      - Infarction and pneumonia
      - Pulmonary hypertension
      - Atelectasis (lung collapse)
      - Congestive heart failure
      - Hemolysis
      - Anemia
      - Cerebral vascular accident (stroke)
      - Paralysis
      - Death
      - Retinopathy (vision problems)
      - Blindness
      - Hemorrhage
      - Avascular necrosis in joints
      - Hepatomegaly (enlarged liver)
      - Gallstones
      - Splenomegaly (enlarged spleen)
      - Splenic sequestration
      - Autosplenectomy (loss of spleen function)
      - Hematuria (blood in urine)
      - Hyposthenuria (dilute urine)
      - Abdominal pain
      - Dactylitis (swelling of hands and feet)
      - Priapism (painful prolonged erection)
      - Osteomyelitis (bone infection)
      - Chronic ulcers (rare in children)

Lab Testing & Diagnostic Testing for Sickle Cell Anemia

  • Platforms include Serial Complete Blood Counts (CBCs) to monitor hemoglobin levels and blood composition.

Treatment & Therapies

  • Focus on:
      - Pain management
      - Rehydration
      - Medications include Hydroxyurea, Crizanlizumab, Voxelotor, L-glutamine, and prophylactic antibiotics.
      - Blood transfusions
      - Oxygen supplementation
      - Bone Marrow Transplantation (BMT) as a potential cure.

Effect on Overall Health

  • Psychosocial impacts

  • Developmental considerations specific to the pediatric population

  • Health promotion and disease prevention strategies

  • Education on health maintenance and disease awareness for family and patients.

Other Hematologic Diseases

Reduced RBC/Blood Component Production

  • Conditions:
      - Aplastic Anemia
      - Hemophilia
      - Von Willebrand Disease

Aplastic Anemia

  • Pathophysiology & Screening:
      - Characterized by failure of bone marrow to produce sufficient blood cells due to loss of hematopoietic stem cells.
      - Can be inherited (genetic) or acquired (autoimmune, secondary to cytotoxic drugs, viral infections, or genetic disorders).

  • Clinical Presentation:
      - Low white blood cell (WBC) count
      - Low platelet count
      - Low red blood cell (RBC) count.

  • Lab Testing and Diagnostic Studies:
      - CBC
      - Bone marrow biopsy for definitive diagnosis.

  • Treatment & Therapies:
      - Blood transfusions to ameliorate symptoms.
      - Medications, including antibiotics and antivirals, depending on concurrent infections.
      - Immunosuppression regime.
      - Bone Marrow Transplant for those with severe cases.

Hemophilia

Pathophysiology & Etiology

  • Pathophysiology:
      - Insufficient or absent clotting factors VIII and IX result in bleeding and disruption of the clotting cascade.
      - Often inherited as an X-linked recessive disorder but can occur spontaneously as well.

Clinical Presentation

  • Classification by Severity:
      - Mild (most common)
      - Moderate
      - Severe

  • Manifestations vary by age groups:
      - Infants may show significant bleeding tendencies.
      - Young and older children can display symptoms depending on activity levels.
      - Hemarthrosis (bleeding into joints) is a common concern.

Lab Testing and Diagnostic Studies

  • Tests performed:
      - CBC, aPTT (activated Partial Thromboplastin Time), PT (Prothrombin Time), fibrinogen, and specific clotting factor assays.

  • Treatment & Therapies:
      - Intravenous replacement of the deficient clotting factor.
      - Infusion therapies to inhibit or stop bleeding, such as DDAVP (Desmopressin) and aminocaproic acid.
      - Specific care approaches for hemarthrosis are also crucial.

Excessive Loss of RBCs/Blood Components

  • Disorders:
      - Immune Thrombocytopenia
      - Disseminated Intravascular Coagulation (DIC)

Immune Thrombocytopenia
Pathophysiology & Screening

  • Autoantibodies lead to early destruction of platelets, resulting in thrombocytopenia.

  • Etiology varies but includes immune-mediated conditions.

Clinical Presentation

  • Symptoms include:
      - Petechial rash
      - Purpura (purple patches on skin)
      - Unusual bleeding or bruising
      - Additional symptoms like hematoma, blood in urine or stool, heavy menstrual bleeding, fatigue.

  • Lab Testing & Diagnostic Testing:
      - CBC showing platelets < 100,000/mcL.

  • Treatment & Therapies:
      - Close observation for potential resolution.
      - Platelet transfusions if necessary, glucocorticoids, intravenous immunoglobulin (IVIG), and potential splenectomy as a last resort.

Other Hematologic Disorders

  • Examples:
      - Neutropenia
      - Leukemia
      - Lymphoma

Neutropenia

  • Definition:
      - Neutrophils are a type of white blood cell produced in bone marrow responsible for protecting the body from infection.

  • Etiology may include:
      - Congenital
      - Chronic benign
      - Cyclic neutropenia
      - Medication-induced and other causes.

  • Clinical Presentation includes:
      - Fever
      - Fatigue
      - Swollen lymph nodes
      - Pain or rash.

  • Lab Testing & Diagnostic Testing:
      - Absolute neutrophil count (ANC) < 1500/mcL indicates neutropenia.

  • Management involves:
      - Treating the underlying cause
      - Use of antibiotics
      - Infection control measures.

Discussion Question: Neutropenia

  • What environmental protections or precautions should be in place when a client has neutropenia?

  • What should the nurse consider when placing a child or adolescent in isolation?