Hematologic Disorders and Anemia Overview

Hematologic Disorders

• Red blood cells (RBCs) or erythrocytes are responsible for transporting nutrients and oxygen to body tissues and removing waste products.
• Platelets or thrombocytes are responsible for blood clotting.
• White blood cells (WBCs) or leukocytes, including granulocytes (neutrophils, eosinophils, and basophils) and agranulocytes, fight infection.

Anemia

• Anemia is a decrease in RBCs or hemoglobin, resulting in reduced oxygen delivery to tissues.
• Three categories of anemia:
  • Loss of RBCs (e.g., bleeding)
  • Hypoproliferative anemia (impaired RBC or hemoglobin production)
  • Hemolytic anemia (excessive RBC destruction)
• Factors determining anemia severity:
  • Speed of development (rapid onset = more severe)
  • Duration (acute vs. chronic)
  • Client metabolic needs (more active = increased needs)
  • Concurrent disorders/disabilities

Symptoms of Anemia

• General symptoms: Weakness, fatigue, and malaise
• Eyes: Yellowing
• Respiratory system: Shortness of breath
• Cardiovascular system: Chest pain, angina, heart attack
• Muscular system: Weakness
• Gastrointestinal system: Changes in stool color
• Neurological system: Peripheral numbness, ataxia (loss of coordination), confusion
• Skin: Pallor, jaundice, easy bruising
• Other: Bleeding gums, angular cheilosis, tachycardia, palpations, dyspnea, dizziness, brittle/ridged/concave nails, nausea, vomiting, melena stools, anorexia, presence of coffee ground emesis, menstrual flow changes

Types of Anemia

• Lead poisoning: A nonessential metal; causes RBC hypo-proliferation and possibly hemolysis when present in the body.
• Vitamin B12 deficiency: Deficiency in a critical enzyme cofactor for DNA synthesis; can cause anemia and occurs with:
  • Inadequate intake
  • Atrophic gastritis (thinning of stomach lining)
• Folic acid deficiency: Deficiency in folic acid; can be caused by alcoholism, malabsorption, pregnancy, or lactation.
• G6PD deficiency: An inherited enzyme deficiency that causes RBC destruction faster than production. Fava beans are a common trigger.
• Other hemolytic anemias: Anemia caused by autoimmune disease, specific infections (e.g., malaria), or adverse medication reactions.

Iron Deficiency Anemia

• Most common type of anemia, especially in children. Iron is crucial for hemoglobin production.
• Risk factors vary by age group:
  • Infants (exclusive breastfeeding beyond 6 months): Breast milk may not be a sufficient iron source.
  • Toddlers: Picky eating and excessive cow's milk consumption.
  • Adolescents: Increased dietary needs and potential poor dietary choices, menstruation.

Expected Lab Findings

• Complete blood count (CBC) showing low RBC, hemoglobin (Hgb), and hematocrit (Hct) values
• RBC indices reflecting red blood cell size (MCV) and hemoglobin content per RBC (MCH)
• Low ferritin (iron stores)
• Low TIBC (transferrin saturation)

Anemia Management

• Aim to address the underlying cause of the anemia.
• Administer iron supplements (oral or IV)
• Manage symptoms and complications of anemia.
• Consider blood transfusions in severe cases to boost oxygen-carrying capacity.

Sickle Cell Anemia

• Genetic disorder causing abnormal hemoglobin (sickle hemoglobin).
• RBCs become rigid and sticky, leading to vessel blockage and causing pain and damage to organs.
• Chronic condition involving various crises
• Common amongst African Americans (1 in 365 have disease, 1 in 13 have trait)

Sickle Cell Crises

• Sequestration crisis: Blood pooling in the liver and spleen, causing decreased blood volume and shock.
• Aplastic crisis: Decreased RBC production, leading to profound anemia. Triggered by viral infection or folic acid depletion.
• Hyperhemolytic crisis: Accelerated RBC destruction characterized by anemia, jaundice, and reticulocytosis.
• Vaso-occlusive crisis: Sickled cells block blood vessels, causing severe pain (especially in bones, joints, chest, or abdomen), inflammation, and tissue damage (necrosis).

Clinical Manifestations of Sickle Cell Anemia

• Central nervous system: Stroke (CVA) is a risk due to blocked blood vessels; also paralysis, retinopathy (blindness), and hemorrhage.
• Hematologic: Hemolysis (breakdown of RBC), splenic sequestration (pooling of blood in the spleen).
• Hepatomegaly / Splenomegaly: Enlarged liver or spleen
• Musculoskeletal: Avascular necrosis (bone damage from reduced blood flow), dactylitis (painful swelling of hands and feet), priapism (prolonged erection), osteomyelitis.
• Renal: Hematuria
• Other: Jaundice, abdominal pain, fever, infection (pneumonia), fatigue and weakness, visual disturbances, shortness of breath.

Managing Sickle Cell Anemia

• Pain management (including opioids, on schedule), hydration, oxygen therapy
• Education to minimize triggers (infection, dehydration, stress)
• Treat acute crises (e.g., blood transfusions, antibiotics, hydration)
• Prophylactic management (e.g., hydroxyurea, penicillin, vaccines)
• Screen for complications (e.g., stroke, acute chest syndrome).

Immune Thrombocytopenic Purpura (ITP)

• Immune system mistakenly attacks and destroys platelets.
• Usually develops 1-3 weeks after infection.
• Risk factors include autoimmune disorders, certain medications, and pregnancy.
• Signs and symptoms: Easy bruising, petechiae or purpura, prolonged or spontaneous bleeding, bleeding gums or nosebleeds, menorrhagia, internal bleeding

Hemophilia

• Genetic bleeding disorder: Deficiency of clotting factors (factors VIII or IX) causes excessive bleeding.

• Types: Hemophilia A (factor VIII deficiency), Hemophilia B (factor IX deficiency), von Willebrand disease (related clotting factor).

• Clinical Presentation: Spontaneous or prolonged bleeding, easy bruising, menorrhagia, hematochezia, hematuria, hemarthrosis (bleeding into joints), intracranial bleeding.

• Complications: Severe or recurrent hemarthrosis, permanent joint deformity, disability.

• Lab findings: Prolonged aPTT, factor VIII/IX levels low in Hemophilia A/B, normal PT.