Retinoic acid syndrome: Difference between revisions
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**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 | ||
==Clinical Features== | ==Clinical Features== | ||
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*Never observed beyond induction therapy, when pt has achieved complete response | *Never observed beyond induction therapy, when pt has achieved complete response | ||
== | ==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 | ||
== | ==Disposition== | ||
==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
- Sepsis
- Fungal infection
- Pulmonary hemorrhage
- Pneumonia
- Renal failure
- CHF
- Pulmonary embolism
- DIC
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
- ↑ 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).