Retinoic acid syndrome: Difference between revisions
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==Background== | ==Background== | ||
*Differentiation syndrome (DS) is most current term | |||
*APML pts undergoing ATRA treatment (Tretinoin, Vesanoid) | |||
**And/or with arsenic trioxide therapy (Trisenox, ATO) | |||
**ATRA differentiates APML blasts into mature granulocytes | |||
**Differentiation of blasts may cause "cytokine storm" and lead 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> | |||
**Up to 25% of pts undergoing ATRA Tx | |||
**Up to 31% of pts undergoing ATO Tx | |||
*Up to 40% mortality in patients requiring mechanical ventilation | |||
*Diagnosis made clinically with symptomology and includes immediate treatment with steroids | |||
**Steroid treatment highly recommended even if other ddx possible | |||
==Differential== | |||
*[[Sepsis]] | |||
*Fungal infection | |||
*Pulmonary hemorrhage | |||
*[[Pneumonia]] | |||
*Renal failure | |||
*[[CHF]] | |||
*[[Pulmonary embolism]] | |||
*[[DIC]] | |||
==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 | |||
==Diagnostics== | |||
*Troponins for pericarditis | |||
*Severe leukocytosis on CBC | |||
*[[Sepsis]] workup | |||
*[[CHF]] workup, echo | |||
*[[Pulmonary embolism]] workup | |||
*[[DIC]] workup | |||
*Diagnostic bronchoscopy, BAL, bx usually not required | |||
*Imaging | |||
**CXR - pleural effusions, ground-glass opacities, increased cardiac silhouette | |||
**CT chest - pleural effusions, peripheral nodules | |||
==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 | |||
**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) | |||
*Furosemide for fluid overload | |||
*CRRT for refractory renal failure | |||
*Supportive | |||
**Mechanical ventilation for respiratory failure | |||
**Careful IVF admin, prioritizing blood products for coagulopathies (DIC) | |||
**Vasopressors | |||
**Empiric antibiotics | |||
**Avoid invasive pleural/pericardial effusion diagnostics | |||
==Sources== | ==Sources== | ||
*Weerakkody Y et al. All trans retinoic acid syndrome. Radiopaedia. http://radiopaedia.org/articles/all-trans-retinoic-acid-syndrome. | |||
<references/> | <references/> | ||
[[Category:Heme/Onc]] | [[Category:Heme/Onc]] | ||
Revision as of 22:58, 2 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)
- ATRA differentiates APML blasts into mature granulocytes
- Differentiation of blasts may cause "cytokine storm" and lead to tissue damage
- Incidence of DS[1]
- Up to 25% of pts undergoing ATRA Tx
- Up to 31% of pts undergoing ATO Tx
- Up to 40% mortality in patients requiring mechanical ventilation
- Diagnosis made clinically with symptomology and includes immediate treatment with steroids
- Steroid treatment highly recommended even if other ddx possible
Differential
- Sepsis
- Fungal infection
- Pulmonary hemorrhage
- Pneumonia
- Renal failure
- CHF
- Pulmonary embolism
- DIC
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
Diagnostics
- Troponins for pericarditis
- Severe leukocytosis on CBC
- Sepsis workup
- CHF workup, echo
- Pulmonary embolism workup
- DIC workup
- 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
- 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)
- Furosemide for fluid overload
- CRRT for refractory renal failure
- Supportive
- Mechanical ventilation for respiratory failure
- Careful IVF admin, prioritizing blood products for coagulopathies (DIC)
- Vasopressors
- Empiric antibiotics
- Avoid invasive pleural/pericardial effusion diagnostics
Sources
- Weerakkody Y et al. All trans retinoic acid syndrome. Radiopaedia. http://radiopaedia.org/articles/all-trans-retinoic-acid-syndrome.
- ↑ 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.
- ↑ Sanz MA and Montesinos P. How we prevent and treat differentiation syndrome in patients with acute promyelocytic leukemia. May 1, 2014; Blood: 123 (18).