Chronic Myelomonocytic Leukemia Associated With TAFRO Syndrome-Like Symptoms

Thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome is a recent disease concept whose physiopathology remains largely unknown. Association of TAFRO syndrome features with immune-mediated or hematologic disorders have been reported but have not...

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Main Authors: Issam Drici-Tani, Lucile Grange, Martin Killian, Jean-Baptiste Gaultier, Michel Péoc'h, Manon Sapet, Ludovic Fouillet, Emmanuelle Tavernier-Tardy, Émilie Chalayer
Format: Article
Language:English
Published: American College of Physicians 2024-05-01
Series:Annals of Internal Medicine: Clinical Cases
Online Access:https://www.acpjournals.org/doi/10.7326/aimcc.2023.1018
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Summary:Thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome is a recent disease concept whose physiopathology remains largely unknown. Association of TAFRO syndrome features with immune-mediated or hematologic disorders have been reported but have not been reported with chronic myelomonocytic leukemia. Chronic myelomonocytic leukemia (CMML) is a myeloproliferative/myelodysplastic syndrome that is frequently associated with autoimmune or autoinflammatory features. Herein, we report 2 cases of CMML-associated TAFRO syndrome-like symptoms. The first one was fatal, and the second one was successfully treated with siltuximab. These reports question the Masaki criteria for TAFRO syndrome diagnosis, which require excluding an autoimmune, neoplastic, or infectious condition beforehand.
ISSN:2767-7664