Difference between revisions of "Retinoic acid syndrome"

(Text replacement - " pts" to " patients")
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==Background==
 
==Background==
 
*Differentiation syndrome (DS) is most current term
 
*Differentiation syndrome (DS) is most current term
*APML pts undergoing ATRA treatment (Tretinoin, Vesanoid)
+
*APML patients undergoing ATRA treatment (Tretinoin, Vesanoid)
 
**And/or with arsenic trioxide therapy (Trisenox, ATO)
 
**And/or with arsenic trioxide therapy (Trisenox, ATO)
 
**Chemo Tx differentiates APML blasts into mature granulocytes
 
**Chemo Tx differentiates APML blasts into mature granulocytes
 
**Differentiation of blasts may cause "cytokine storm" leading to tissue damage
 
**Differentiation of blasts may cause "cytokine storm" leading to tissue damage
 
*Incidence of DS<ref>Montesinos P and Sanz MA. The Differentiation Syndrome in Patients with Acute Promyelocytic Leukemia: Experience of the Pethema Group and Review of the Literature. Mediterr J Hematol Infect Dis. 2011; 3(1): e2011059.</ref>
 
*Incidence of DS<ref>Montesinos P and Sanz MA. The Differentiation Syndrome in Patients with Acute Promyelocytic Leukemia: Experience of the Pethema Group and Review of the Literature. Mediterr J Hematol Infect Dis. 2011; 3(1): e2011059.</ref>
**Up to 25% of pts undergoing ATRA
+
**Up to 25% of patients undergoing ATRA
**Up to 31% of pts undergoing ATO
+
**Up to 31% of patients undergoing ATO
 
*Up to 40% mortality in patients requiring mechanical ventilation
 
*Up to 40% mortality in patients requiring mechanical ventilation
 
*Diagnosis made clinically with symptomology
 
*Diagnosis made clinically with symptomology

Revision as of 16:49, 21 June 2016

Background

  • Differentiation syndrome (DS) is most current term
  • APML patients undergoing ATRA treatment (Tretinoin, Vesanoid)
    • And/or with arsenic trioxide therapy (Trisenox, ATO)
    • Chemo Tx differentiates APML blasts into mature granulocytes
    • Differentiation of blasts may cause "cytokine storm" leading to tissue damage
  • Incidence of DS[1]
    • Up to 25% of patients undergoing ATRA
    • Up to 31% of patients undergoing ATO
  • Up to 40% mortality in patients requiring mechanical ventilation
  • Diagnosis made clinically with symptomology
    • Requires immediate treatment with steroids
    • Steroids highly recommended even if other ddx possible

Clinical Features

  • Unexplained fever
  • Unexplained hypotension
  • Weight gain > 5 kg
  • Dyspnea with pulmonary infiltrates
  • Pericardial effusion
  • Pleural effusion
  • Renal failure
  • Vascular capillary leak syndrome
  • DIC
  • Never observed beyond induction therapy, when pt has achieved complete response

Differential Diagnosis

Diagnosis

  • Troponins for pericarditis
  • Severe leukocytosis on CBC
  • Sepsis workup
  • CHF workup, echo
  • Pulmonary embolism workup
  • DIC workup
  • Coagulation factor levels
  • Diagnostic bronchoscopy, BAL, bx usually not required
  • Imaging
    • CXR - pleural effusions, ground-glass opacities, increased cardiac silhouette
    • CT chest - pleural effusions, peripheral nodules

Management[2]

  • Dexamethasone 10 mg IV q12hrs
    • Most important therapy, with possible dramatic and rapid resolution of symptoms
    • Continue treatment until complete disappearance of signs and symptoms
  • Temporary d/c ATRA or ATO only in severe cases of DS (renal failure or ICU admit)
  • Supportive
    • Mechanical ventilation for respiratory failure
    • Careful IVF admin, prioritizing blood products for coagulopathies (DIC)
    • Furosemide for fluid overload
    • CRRT/HD for refractory renal failure
    • Vasopressors
    • Empiric antibiotics
    • Avoid invasive pleural/pericardial effusion diagnostics

Disposition

See Also

External Links

References

  1. Montesinos P and Sanz MA. The Differentiation Syndrome in Patients with Acute Promyelocytic Leukemia: Experience of the Pethema Group and Review of the Literature. Mediterr J Hematol Infect Dis. 2011; 3(1): e2011059.
  2. Sanz MA and Montesinos P. How we prevent and treat differentiation syndrome in patients with acute promyelocytic leukemia. May 1, 2014; Blood: 123 (18).