Hematology

Sickle Cell Disease

Pathophysiology

  • Sickled red blood cells can still transport oxygen but have a tendency to clump together, increasing blood viscosity and impairing circulation.
  • Effects: Reduced oxygen delivery → pain, swelling (edema), and tissue ischemia

Treatment

  • Supportive Care: Oxygen (if hypoxic), IV fluids, and pain management
  • Hydroxyurea: Increases RBC size and flexibility, reducing the likelihood of sickling

Common Triggers

  • Exposure to cold temperatures
  • High altitudes
  • Infection (listed twice, emphasizing its significance)
  • Acidosis

Potential Complications

  • Acute Chest Syndrome (Sickle Cell Crisis)
    • Symptoms: Chest pain, shortness of breath, cough, wheezing, low oxygen levels, diminished breath sounds, lung infiltrates on X-ray
  • Gallstones (Cholelithiasis)
  • Infections: Pneumonia, meningitis, osteomyelitis
  • Organ Damage: Kidney failure, bone disease, heart failure

Hemophilia & Bleeding Disorders

Types of Hemophilia

  • Type A: Deficiency in Factor VIII
  • Type B: Deficiency in Factor IX
  • Von Willebrand Disease: Lacking a plasma protein essential for proper platelet function

Management & Treatment

  • First Aid Measures:
    • Immobilize the affected area
    • Elevate the limb
    • Apply ice and light pressure to reduce swelling and bleeding
  • Medical Interventions:
    • Clotting factor replacement therapy
    • Fresh Frozen Plasma (FFP) for severe cases
    • Joint aspiration to alleviate pain caused by internal bleeding

Causes of Secondary Erythrocytosis

Excess Erythropoietin (EPO) Production

  • Tumors that secrete EPO:
    • Renal cell carcinoma
    • Hepatocellular carcinoma
    • Pheochromocytoma

Genetic & Acquired Mutations

  • Conditions linked to mutations:
    • Polycythemia vera
    • EPO receptor gene mutations
    • Congenital methemoglobinemia
    • High-affinity oxygen hemoglobins

Chronic Hypoxia-Related Causes

  • Lung & Heart Conditions:
    • COPD
    • Right-to-left cardiac shunts
    • Sleep apnea
    • Obesity-related hypoventilation
  • Environmental & Hematologic Factors:
    • Living at high altitudes
    • Red blood cell abnormalities
    • Long-term carbon monoxide exposure

Other Contributing Factors

  • Substances & Medical Interventions:
    • Use of anabolic steroids or diuretics
    • Blood doping
    • Self-administered EPO injections
    • POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, Skin changes)

Infectious Diseases

Measles (Rubeola)

  • Incubation Period: 10–14 days
  • Highly Contagious: Spreads from 5 days before rash onset to 5 days after
  • Rash Characteristics:
    • Morbilliform rash (resembles measles)
    • Macular lesions (2–10 mm), may merge together
  • Symptoms:
    • Cough, photophobia, eyelid swelling, fever, joint pain
    • Koplik’s spots (small white spots inside the mouth)
    • The Three C’s: Cough, Coryza (runny nose), Conjunctivitis
  • Treatment: Supportive care

Mumps

  • Transmission: Respiratory droplets, saliva
  • Most Contagious: 1–2 days before parotid gland swelling and up to 5 days after
  • Symptoms:
    • Swollen salivary glands, fever, fatigue, difficulty opening the mouth (trismus)
  • Complications:
    • Orchitis (testicular inflammation)
    • Pancreatitis
    • Oophoritis (ovarian inflammation)

Pertussis (Whooping Cough)

  • Cause: Bacterial infection that impairs secretion clearance
  • Transmission: Droplet precautions; may lead to secondary pneumonia
  • Incubation: 4–42 days
  • Stages:
    1. Catarrhal Stage (≈1 week): Congestion, mild fever
    2. Paroxysmal Stage (1–6 weeks): Intense coughing fits ending in a “whoop,” worse at night
    3. Convalescent Stage: Gradual recovery
  • Treatment:
    • Oxygen via nasal cannula, suctioning
    • Antibiotics
    • Hydration

Chickenpox (Varicella-Zoster Virus)

  • Precautions: Airborne transmission
  • Spread: Infectious 48 hours before rash appears until all lesions crust over
  • Treatment:
    • Antivirals if indicated
    • Supportive care
    • Varicella-zoster immune globulin for pregnant patients

Meningitis

Key Symptoms of Bacterial Meningitis

  • Most Common: Severe headache, high fever
  • Neck Stiffness (Nuchal Rigidity):
    • Found in only 1/3 of patients under 6 months old
    • Present in 95% of patients older than 19 months

Classic Signs of Meningeal Irritation (Meningismus)

  1. Triad of Symptoms:
    • Neck stiffness
    • Headache
    • Sensitivity to light (photophobia)
  2. Kernig’s Sign: Hamstring stiffness preventing full leg extension
  3. Brudzinski’s Sign: Involuntary bending of knees and hips when the neck is flexed

Not typically seen in neonates due to an immature immune response

Signs of Meningitis in Newborns

  • General Symptoms:
    • Fever or hypothermia
    • Breathing difficulties, jaundice
    • Extreme fatigue, poor feeding, failure to thrive
    • High-pitched cry, vomiting, irritability
  • Specific Signs:
    • Seizures
    • Bulging soft spot on the head (fontanelle)

Hepatitis

  • No vaccine available for Hepatitis C
  • Hepatitis C risks:
    • Can lead to liver cancer or cirrhosis if untreated

Sexually Transmitted Infections (STIs)

Chlamydia

  • Most prevalent STI
  • Can lead to pelvic inflammatory disease (PID) or tubo-ovarian abscess if untreated

Symptoms

  • Pain or burning sensation during urination
  • Abnormal discharge:
    • Women: Milky or yellow with a noticeable odor
    • Men: White, cloudy, or watery

Gonorrhea

Symptoms:

  • Burning sensation during urination
  • Unusual discharge:
    • Women: Increased vaginal discharge, which may be creamy, yellow, or slightly green
    • Men: Discharge that appears white, yellow, or green

Trichomoniasis

  • Up to 70% of cases are asymptomatic
  • Possible symptoms:
    • Itching and irritation
    • Strong, fishy odor

Discharge:

Men: Thin, white discharge

Women: Increased vaginal discharge, which may be thin, white, yellow, or green

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