Many of the PedEM Morsels accentuate the need to be vigilant, although reasonable, and guard against the significant pathology that may present in a benign manner. Often this merely requires maintaining an “active” mind when evaluating children.  The “active” mind searches for clues for possible Kawasaki Disease when evaluating the child with prolonged fever.  Vigilance allows us to actively ponder HUS when evaluating the patient with diarrhea.  Vigilance, however, requires knowledge about the subtle clues that we need to actively search for.  Let us, then, review some of the subtle clues that may raise your suspicion for one of the more alarming diseases: Leukemia.

Leukemia: Basics

• Cancer is the #1 cause of death due to disease in developed countries.
  • Traumatic injuries are still the overall leading cause of death.
  • In the US, ~ 10,380 new cases are expected to occur among children in 2016 [American Cancer Society]

• Few known risk factors for childhood cancer  [American Cancer Society]
  • Children are more sensitive to ionization radiation, but the actual number of resultant cases is unknown.
  • Small percentage attributed to inherited genetic mutations.
  • Certain syndromes are associated with increased risk for cancer. (ex, Down Syndrome)

• Leukemia is the most common childhood malignancy
  • Accounts for ~30% of all childhood cancers
  • Acute lymphocytic leukemia (ALL) does occur in adults, but 75% of cases are in patients 0-19 years of age.
  • Leukemia, can also occur in neonates. (See Neonatal Leukemia)

Leukemia: Presentation

• Recent meta-analysis looked at clinical presentation of childhood leukemia. [Clarke, 2016]
  • Worth a gander.
  • Advocates for reasonable vigilance using focused History and Physical Exam (novel concept).

• The early presentation is often with non-specific symptoms.
  • Similar to the multitude of other self-limiting illnesses – makes it easy to miss.
  • Pallor, Fever, and Fatigue are commonly encountered
    • Also common in many other conditions
    • These should cause us to actively look for other findings / symptoms

• Look for clues, even if non-specific:
  • At diagnosis, 5 features were present in >50% of cases: [Clarke, 2016]
    • Hepatomegaly (64%)
    • Splenomegaly (61%)
    • Pallor (54%)
    • Fever (53%)
    • Bruising (52%)
  • In 33% – 50% of cases, 8 additional features were prominent: [Clarke, 2016]
    • Recurrent Infections (49%)
    • Fatigue (46%)
    • Limb pain (43%)
    • Hepatosplenomegaly (42%)
    • Bruising/petechiae (42%)
    • Lymphadenopathy (41%)
    • Bleeding tendency (38%)
    • Rash (35%)
  • Abdominal symptoms (ex, anorexia, weight loss, pain, and distention) are also common.
• One clue may not require investigation, but one clue leading to the discovery of another… and another… well, that’s how you build a case.

Leukemia: Remain Vigilant

• Fortunately, leukemia is relatively rare, and hopefully your patient will not have it, but…
• Our vigilance today makes a difference tomorrow.
  • Fever and Abdominal Pain should make us think of more than Appendicitis or AGE.
  • Limb pain requires more thought than just Septic Arthritis vs Growing Pain.
  • Not all lymph nodes are “shotty.”
  • Prolonged fever may be due to a virus, and contemplation of Incomplete Kawasaki Disease or Occult Pneumonia scores you bonus points, but let’s not overlook other ominous causes.
• Vigilance does not cost a lot of money.
  • Perform a thoughtful history – “Did I ask about unexplained bleeding from the gums?”
  • Be actively looking for clues during your exam – and specifically document the presence of lack of hepatomegaly, splenomegaly, lymphadenopathy, and/or pallor.

References