Differentiation Syndrome

linda.hinkley@nuvancehealth.org

Differentiation Syndrome is a life-threatening complication of induction chemotherapy for patients with acute promyelocytic leukemia (APL).  Manifistations of this syndrome include fever, hypoxemia, edema, and, in the past, has been referred to as "cytokine storm."  How can the diagnosis of Differentiation Syndrome be captured?  Does ICD-10 provide for this condition?

Linda Hinkley, RN, MSN

hooter1964

Wow great question! I think we are stuck with an adverse effect of all-trans retinoic acid (ATRA; also known as tretinoin) 

ATRA therapy has also been associated in 10% to 25% of patients with the “retinoic acid syndrome” (nowadays referred to as differentiation syndrome because it is also seen with other differentiating

agents). It occurs within a few days of start of therapy and presents with signs and symptoms resembling capillary leak and respiratory distress syndrome: fever, dyspnea, hypoxemia,

pulmonary infiltrates, weight gain, peripheral edema, renal and hepatic dysfunction, and ascites. Differentiation syndrome carries a high mortality unless recognized and managed promptly.
Page 928 , 2013  HEMATOLOGY BASIC PRINCIPLES AND PRACTICE

I know of no code so I would watch for MCC's of acute noncardiogenic pulmonary edema and acute respiratory failure requiring intubation and mechanical ventilation. 
Its etiology is poorly understood. It is thought to be caused by a capillary leak syndrome induced by the rapid differentiation of leukemic promyelocytes.

mini.joseph

I have a similar case,but  documented  as below  

"differentiation syndrome for a 75 yo with IDH2 mutated AML in relapse on enasidenib"
 Is it adverse effect of chemo?

afrady@blr.com

mini.joseph said:

I have a similar case,but  documented  as below  

"differentiation syndrome for a 75 yo with IDH2 mutated AML in relapse on enasidenib"
 Is it adverse effect of chemo?

It appears so.

mini.joseph

Thank you!