Hemoglobin

Hand Foot Syndrome in Sickle Cell Anemia

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Hand Foot Syndrome in Sickle Cell Anemia, Dactylitis in Sickle Cell Anemia

  • See Also
  1. Sickle Cell Anemia
  2. Transient Red Cell Aplasia
  3. Acute Chest Syndrome
  4. Acute Vaso-Occlusive Episode in Sickle Cell Anemia
  5. Aplastic Crisis in Sickle Cell Anemia
  6. Cerebrovascular Accident in Sickle Cell Anemia
  7. Hematuria in Sickle Cell Anemia
  8. Osteomyelitis in Sickle Cell Anemia
  9. Priapism in Sickle Cell Anemia
  10. Pulmonary Hypertension in Sickle Cell Anemia
  11. Septic Arthritis in Sickle Cell Anemia
  12. Sickle Cell Anemia Related Pulmonary Hypertension
  13. Sickle Cell Anemia with Splenic Sequestration
  • Epidemiology
  1. Often the first vaso-Occlusion crisis finding in young children (as early as 6 months of age)
  2. Occurs in up to 50% of children with Sickle Cell Anemia by the age of 2 years
  3. Rare over age 4 years old
  • Pathophysiology
  1. Not to be confused with Hand Foot Syndrome that occurs with certain Chemotherapy agents in non-sickle disease
  2. Occurs in Sickle Cell Anemia with vaso-Occlusion
  3. Hematopoiesis occurs in the peripheral extremities in younger children
    1. As children age, hematopoiesis shifts to the more proximal extremities
  4. Caused by ischemia or infarction of Bone Marrow
    1. Affects Metacarpal or Metatarsal Bones and phalanges
  • Symptoms
  1. Painful swelling and tenderness of either the dorsal hand or foot
  2. Non-pitting soft tissue swelling over the dorsal, proximal hand or foot (Metacarpals/Metatarsals, proximal phalanges)
  3. Digits may appear mildly erythematous
  • Signs
  1. Low grade fever (variably present)
  • Imaging
  1. No bony abnormalities (contrast with other disorders on the differential diagnosis)
  • Differential Diagnosis
  1. Osteomyelitis (Salmonella)
  • Course
  1. Dactylitis episodes resolve spontaneously within 1 week
  2. Dactylitis before age 12 months predicts severe lifetime Sickle Cell Disease (RR 2.6)
  • References
  1. Lopez (2025) Crit Dec Emerg Med 30(3): 4-12
  2. Welsh and Welsh (2016) Crit Dec Emerg Med 30(11): 15-23