Paraneoplastic Autoimmune Multiorgan Syndrome: Diagnostic Lessons for Pediatricians.
Bilade Cherqaoui, Léa Jaume, Gudrun Schleiermacher et al.
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Case in brief
An 11-year-old boy developed subacute erythroblastopenia, followed by polymorphic mucocutaneous findings, severe myositis with a dermatomyositis-like phenotype, and mild hepatitis. Progressive lymphadenopathy eventually led to diagnosis of T-lymphoblastic lymphoma associated with paraneoplastic autoimmune multiorgan syndrome. The syndrome reached remission with a Janus kinase inhibitor in addition to antitumor therapy.
What made this case unusual
The lymphoma diagnosis was delayed as the presentation evolved from erythroblastopenia to a multisystem immune-mediated syndrome resembling juvenile dermatomyositis, with mucosal and hematologic abnormalities.
Diagnostic / clinical pearl
A dermatomyositis-like presentation with atypical hematologic or mucosal findings should prompt consideration of a paraneoplastic process and early collaboration with oncology.
Why it matters
In this case, remission of the paraneoplastic syndrome required immunomodulatory treatment in addition to antitumor therapy.