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\begin{document}

\title{Sweet syndrome with differentiation syndrome related to Enasidenib}



\author[1]{Amr Mohamed}%
\affil[1]{Rochester Regional Health}%


\vspace{-1em}



  
  \date{May 25, 2024}


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\selectlanguage{english}
\begin{abstract}
Rash is frequently seen in patients with leukemia either as a sequence
of thrombocytopenia, or as result of infection or allergic drug rash,
however sometimes rash is pointing towards fatal complications of
underlying drug therapy or underlying disease , not being aware of these
alarming signs can have fatal consequences.%
\end{abstract}%



\sloppy


\textbf{Title} : Sweet syndrome with differentiation syndrome related to
Enasidenib

\textbf{Author} : Mohamed Amr, MD

\textbf{Affiliation} : Department of Internal Medicine, Rochester
General Hospital, Rochester, NY.

\textbf{Corresponding Author} : Mohamed Amr, MD

\textbf{Contact number} : 718-764-7202

\textbf{Affiliation address} : 1425 Portland Avenue, Rochester, NY,
14621.

\textbf{Email address} : amrelwagdycardiol@gmail.com

\textbf{Funding source} : none

\textbf{Conflict of interest} : none

\textbf{Article type} : Clinical image

\textbf{Key words} : Differentiation syndrome, Enasidenib, Sweet
syndrome.

\textbf{Key clinical message:} Multiple conditions as Drug rash,
thromboembolism, sepsis and heart failure can mimic differentiation
syndrome so keeping high index of suspicion is important as the
prognosis if treated is usually good and fatal if missed.

\textbf{Word count} : 255 words

\textbf{Figures:} 1

65-year-old female who was admitted to hospital for treatment of acute
myeloid leukemia, she was on Enasidenib for IDH2+ mutation in addition
to standard leukemia therapy, after one week of therapy she started
having fever up to 102F with generalized skin rash , her physical exam
was normal except for progressively worsening generalized skin rash as
shown in figure 1A.

The initial dilemma in her diagnosis was whether this is infectious rash
as she was neutropenic with absolute neutrophilic count (ANC) of 0, she
was started on cefepime followed by vancomycin, the next day her rash
continued to worsen with new shortness of breath, her chest x-ray showed
diffuse pulmonary infiltrates as shown in figure 1B and ANC did rapidly
rise to 5 and now the clinical picture is more suggestive of
differentiation syndrome with sweet syndrome.

She was a started on dexamethasone with resolution of the shortness of
breath, fever, lung infiltrates and skin rash , skin biopsy was
performed and it had shown prominent edema in the superficial dermis
with dense infiltrate of neutrophils in the upper and mid-dermis with
endothelial swelling and no evidence of vasculitis all consistent with
sweet syndrome.

The main message is that differentiation syndrome happen in 20\% of
patients receiving isocitrate dehydrogenase inhibitor{[}1{]}, it is
sometimes associated with sweet syndrome, missing the diagnosis lead to
fatal complications and the presentation can mimic drug rash ,
thromboembolism , infection or heart failure so keeping high index of
suspicion is important as the prognosis if treated is usually good
{[}1,2{]}.

\textbf{References}

1 - Fathi AT, DiNardo CD, Kline I, et al. Differentiation Syndrome
Associated With Enasidenib, a Selective Inhibitor of Mutant Isocitrate
Dehydrogenase 2: Analysis of a Phase 1/2 Study. JAMA Oncol. 2018 Aug 1;
4(8):1106-1110. doi: 10.1001/jamaoncol.2017.4695. PMID: 29346478; PMCID:
PMC5885269.

2 - DiNardo CD, Stein EM, de Botton S, et al. Durable Remissions with
Ivosidenib in IDH1-Mutated Relapsed or Refractory AML. N Engl J Med.
2018 Jun 21; 378(25):2386-2398. doi: 10.1056/NEJMoa1716984. Epub 2018
Jun 2. PMID: 29860938.

\textbf{Figure legends:}

Figure 1A: showing generalized papulovesicular skin rash on the anterior
abdominal wall.

Figure 1B: showing chest x-ray with evidence of bilateral diffuse
interstitial lung infiltrates, no cardiomegaly.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/sweet-syndrome/sweet-syndrome}

\end{center}
\end{figure}

\selectlanguage{english}
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\end{document}

