Difference between revisions of "Retinoic acid syndrome"

(Text replacement - "*CXR" to "*CXR")
 
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==Background==
 
==Background==
 
*Differentiation syndrome (DS) is most current term
 
*Differentiation syndrome (DS) is most current term
*APML patients undergoing ATRA treatment (Tretinoin, Vesanoid)
+
*Occurs in [[AML|Acute promyelocytic leukemia]] patients undergoing ATRA treatment (Tretinoin, Vesanoid)
 
**And/or with arsenic trioxide therapy (Trisenox, ATO)
 
**And/or with arsenic trioxide therapy (Trisenox, ATO)
 
**Chemo treatment differentiates APML blasts into mature granulocytes
 
**Chemo treatment differentiates APML blasts into mature granulocytes
**Differentiation of blasts may cause "cytokine storm" leading to tissue damage
+
**Differentiation of blasts may cause [[sepsis]]-like "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 patients undergoing ATRA
 
**Up to 25% of patients undergoing ATRA
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*Diagnosis made clinically with symptomology
 
*Diagnosis made clinically with symptomology
 
**Requires immediate treatment with steroids
 
**Requires immediate treatment with steroids
**Steroids highly recommended even if other ddx possible
+
**Steroids highly recommended even if other diagnoses possible
  
 
==Clinical Features==
 
==Clinical Features==
*Unexplained fever
+
*Unexplained [[fever]]
*Unexplained hypotension
+
*Unexplained [[hypotension]]
 
*Weight gain > 5 kg
 
*Weight gain > 5 kg
*Dyspnea with pulmonary infiltrates
+
*[[Dyspnea]] with pulmonary infiltrates
 
*[[Pericardial effusion]]
 
*[[Pericardial effusion]]
*Pleural effusion
+
*[[Pleural effusion]]
*Renal failure
+
*[[Renal failure]]
 
*Vascular capillary leak syndrome
 
*Vascular capillary leak syndrome
 
*[[DIC]]
 
*[[DIC]]
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==Differential Diagnosis==
 
==Differential Diagnosis==
 
*[[Sepsis]]
 
*[[Sepsis]]
*Fungal infection
+
*[[Fungal infection]]
*Pulmonary hemorrhage
+
*[[diffuse alveolar hemorrhage|Pulmonary hemorrhage]]
 
*[[Pneumonia]]
 
*[[Pneumonia]]
*Renal failure
+
*[[Renal failure]]
 
*[[CHF]]
 
*[[CHF]]
 
*[[Pulmonary embolism]]
 
*[[Pulmonary embolism]]
 
*[[DIC]]
 
*[[DIC]]
 +
 +
{{Template:Oncologic emergencies DDX}}
  
 
==Evaluation==
 
==Evaluation==
 +
*Ultimately, a clinical diagnosis
 
*[[Troponins]] for [[pericarditis]]
 
*[[Troponins]] for [[pericarditis]]
*Severe leukocytosis on CBC
+
*Severe [[leukocytosis]] on CBC
 
*[[Sepsis]] workup
 
*[[Sepsis]] workup
 
*[[CHF]] workup, echo
 
*[[CHF]] workup, echo
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*[[DIC]] workup
 
*[[DIC]] workup
 
*Coagulation factor levels
 
*Coagulation factor levels
*Diagnostic bronchoscopy, BAL, bx usually not required
+
*Diagnostic bronchoscopy, BAL, biopsy usually not required
 
*Imaging
 
*Imaging
 
**[[CXR]] - pleural effusions, ground-glass opacities, increased cardiac silhouette
 
**[[CXR]] - pleural effusions, ground-glass opacities, increased cardiac silhouette
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*Temporary discharge ATRA or ATO only in severe cases of DS (renal failure or ICU admit)
 
*Temporary discharge ATRA or ATO only in severe cases of DS (renal failure or ICU admit)
 
*Supportive
 
*Supportive
**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/[[hemodialysis|HD]] for refractory renal failure
 
**[[Vasopressors]]
 
**[[Vasopressors]]
 
**Empiric [[antibiotics]]
 
**Empiric [[antibiotics]]
**Avoid invasive pleural/pericardial effusion diagnostics
+
**''Avoid'' invasive pleural/pericardial effusion diagnostics
  
 
==Disposition==
 
==Disposition==
 +
*Admit
  
 
==See Also==
 
==See Also==

Latest revision as of 17:56, 1 October 2019

Background

  • Differentiation syndrome (DS) is most current term
  • Occurs in Acute promyelocytic leukemia patients undergoing ATRA treatment (Tretinoin, Vesanoid)
    • And/or with arsenic trioxide therapy (Trisenox, ATO)
    • Chemo treatment differentiates APML blasts into mature granulocytes
    • Differentiation of blasts may cause sepsis-like "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 diagnoses possible

Clinical Features

Differential Diagnosis

Oncologic Emergencies

Related to Local Tumor Effects

Related to Biochemical Derangement

Related to Hematologic Derangement

Related to Therapy

Evaluation

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

Management[2]

  • Dexamethasone 10mg IV q12hrs
    • Most important therapy, with possible dramatic and rapid resolution of symptoms
    • Continue treatment until complete disappearance of signs and symptoms
  • Temporary discharge ATRA or ATO only in severe cases of DS (renal failure or ICU admit)
  • Supportive

Disposition

  • Admit

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).