Retinoic acid syndrome: Difference between revisions
Ostermayer (talk | contribs) (Text replacement - " pts" to " patients") |
ClaireLewis (talk | contribs) No edit summary |
||
(7 intermediate revisions by 4 users not shown) | |||
Line 1: | Line 1: | ||
==Background== | ==Background== | ||
*Differentiation syndrome (DS) is most current term | *Differentiation syndrome (DS) is most current term | ||
* | *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 | **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 | ||
Line 11: | Line 11: | ||
*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 | **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]] | ||
*Never observed beyond induction therapy, when | *Never observed beyond induction therapy, when patient has achieved complete response | ||
==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== | |||
*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 | ||
Line 43: | Line 46: | ||
*[[DIC]] workup | *[[DIC]] workup | ||
*Coagulation factor levels | *Coagulation factor levels | ||
*Diagnostic bronchoscopy, BAL, | *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 | ||
**CT chest - pleural effusions, peripheral nodules | **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>== | ==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]] | *[[Dexamethasone]] 10mg 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 | ||
*Temporary | *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
- 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 patient has achieved complete response
Differential Diagnosis
Oncologic Emergencies
Related to Local Tumor Effects
- Malignant airway obstruction
- Bone metastases and pathologic fractures
- Malignant spinal cord compression
- Malignant Pericardial Effusion and Tamponade
- Superior vena cava syndrome
Related to Biochemical Derangement
- Hypercalcemia of malignancy
- Hyponatremia due to SIADH
- Adrenal insufficiency
- Tumor lysis syndrome
- Carcinoid syndrome
Related to Hematologic Derangement
Related to Therapy
- Chemotherapy-induced nausea and vomiting
- Cytokine release syndrome
- Chemotherapeutic drug extravasation
- Differentiation syndrome (retinoic acid syndrome) in APML
- Stem cell transplant complications
- Catheter-related complications
- Tunnel infection
- Exit site infection
- CVC obstruction (intraluminal or catheter tip thrombosis)
- Catheter-related venous thrombosis
- Fracture of catheter lumen
- Oncologic therapy related adverse events
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
- 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
- Admit
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).