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\begin{document}

\title{DADA2 presenting as nonimmune hemolytic anemia with recurrent macrophage
activation syndrome}



\author[1]{Vaishnavi Iyengar}%
\author[1]{Akshaya Chougule}%
\author[1]{Vijaya  Gowri}%
\author[1]{Prasad Taur}%
\author[1]{Minnie Bodhanwala}%
\author[1]{Shakuntala Prabhu}%
\author[1]{Mukesh Desai}%
\affil[1]{Bai Jerbai Wadia Hospital for Children}%


\vspace{-1em}



  
  \date{May 20, 2024}


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\begin{abstract}
Hemolytic anemia is a common clinical problem with diverse etiology.
10-year-old boy presented with severe pallor. He had hemolytic facies,
hepatosplenomegaly \& deformity of small joints. Investigations revealed
DAT-ve anemia, reticulocytosis and low haptoglobin. BM showed erythroid
hyperplasia. Past history revealed recurrent fever, rash,
hepatosplenomegaly, lymphadenopathy and poly arthritis diagnosed as
systemic-JIA and MAS necessitating admission twice. Hence, a diagnosis
of hemolytic anemia with auto inflammatory disease was considered. Whole
Exome revealed compound heterozygous mutation in CECR1 gene. DADA2 is a
syndrome with varied manifestations including vasculitis, hematological
and immunological abnormalities. Clue in our patient was presence of
periodic fever.%
\end{abstract}%



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