Difference between revisions of "Retinoic acid syndrome"

Line 12: Line 12:
 
**Requires immediate treatment with steroids
 
**Requires immediate treatment with steroids
 
**Steroids highly recommended even if other ddx possible
 
**Steroids highly recommended even if other ddx possible
 
==Differential==
 
*[[Sepsis]]
 
*Fungal infection
 
*Pulmonary hemorrhage
 
*[[Pneumonia]]
 
*Renal failure
 
*[[CHF]]
 
*[[Pulmonary embolism]]
 
*[[DIC]]
 
  
 
==Clinical Features==
 
==Clinical Features==
Line 35: Line 25:
 
*Never observed beyond induction therapy, when pt has achieved complete response
 
*Never observed beyond induction therapy, when pt has achieved complete response
  
==Diagnostics==
+
==Differential Diagnosis==
*Troponins for pericarditis
+
*[[Sepsis]]
 +
*Fungal infection
 +
*Pulmonary hemorrhage
 +
*[[Pneumonia]]
 +
*Renal failure
 +
*[[CHF]]
 +
*[[Pulmonary embolism]]
 +
*[[DIC]]
 +
 
 +
==Diagnosis==
 +
*[[Troponins]] for [[pericarditis]]
 
*Severe leukocytosis on CBC
 
*Severe leukocytosis on CBC
 
*[[Sepsis]] workup
 
*[[Sepsis]] workup
Line 49: Line 49:
  
 
==Management<ref>Sanz MA and Montesinos P. How we prevent and treat differentiation syndrome in patients with acute promyelocytic leukemia. May 1, 2014; Blood: 123 (18).</ref>==
 
==Management<ref>Sanz MA and Montesinos P. How we prevent and treat differentiation syndrome in patients with acute promyelocytic leukemia. May 1, 2014; Blood: 123 (18).</ref>==
*Dexamethasone 10 mg IV q12hrs
+
*[[Dexamethasone]] 10 mg IV q12hrs
 
**Most important therapy, with possible dramatic and rapid resolution of symptoms
 
**Most important therapy, with possible dramatic and rapid resolution of symptoms
 
**Continue treatment until complete disappearance of signs and symptoms
 
**Continue treatment until complete disappearance of signs and symptoms
Line 56: Line 56:
 
**Mechanical ventilation for respiratory failure
 
**Mechanical ventilation for respiratory failure
 
**Careful IVF admin, prioritizing blood products for coagulopathies (DIC)
 
**Careful IVF admin, prioritizing blood products for coagulopathies (DIC)
**Furosemide for fluid overload
+
**[[Furosemide]] for fluid overload
 
**CRRT/HD for refractory renal failure
 
**CRRT/HD for refractory renal failure
**Vasopressors
+
**[[Vasopressors]]
**Empiric antibiotics
+
**Empiric [[antibiotics]]
 
**Avoid invasive pleural/pericardial effusion diagnostics
 
**Avoid invasive pleural/pericardial effusion diagnostics
  
==Sources==
+
==Disposition==
*Weerakkody Y et al. All trans retinoic acid syndrome. Radiopaedia. http://radiopaedia.org/articles/all-trans-retinoic-acid-syndrome.
+
 
 +
==See Also==
 +
 
 +
==External Links==
  
 +
==References==
 
<references/>
 
<references/>
  
 
[[Category:Heme/Onc]]
 
[[Category:Heme/Onc]]

Revision as of 00:10, 3 May 2016

Background

  • Differentiation syndrome (DS) is most current term
  • APML pts 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 pts undergoing ATRA
    • Up to 31% of pts 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).