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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author Issam Drici-Tani
Lucile Grange
Martin Killian
Jean-Baptiste Gaultier
Michel Péoc'h
Manon Sapet
Ludovic Fouillet
Emmanuelle Tavernier-Tardy
Émilie Chalayer
author_facet Issam Drici-Tani
Lucile Grange
Martin Killian
Jean-Baptiste Gaultier
Michel Péoc'h
Manon Sapet
Ludovic Fouillet
Emmanuelle Tavernier-Tardy
Émilie Chalayer
author_sort Issam Drici-Tani
collection DOAJ
description 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.
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issn 2767-7664
language English
publishDate 2024-05-01
publisher American College of Physicians
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series Annals of Internal Medicine: Clinical Cases
spelling doaj-art-019daf083033457a8502f57331da9d092025-08-20T03:18:46ZengAmerican College of PhysiciansAnnals of Internal Medicine: Clinical Cases2767-76642024-05-013510.7326/aimcc.2023.1018Chronic Myelomonocytic Leukemia Associated With TAFRO Syndrome-Like SymptomsIssam Drici-Tani0Lucile Grange1Martin Killian2Jean-Baptiste Gaultier3Michel Péoc'h4Manon Sapet5Ludovic Fouillet6Emmanuelle Tavernier-Tardy7Émilie Chalayer81Service de Médecine Interne, Centre Hospitalier Universitaire de Saint-Étienne, Saint-Étienne, France1Service de Médecine Interne, Centre Hospitalier Universitaire de Saint-Étienne, Saint-Étienne, France1Service de Médecine Interne, Centre Hospitalier Universitaire de Saint-Étienne, Saint-Étienne, France1Service de Médecine Interne, Centre Hospitalier Universitaire de Saint-Étienne, Saint-Étienne, France4Service d'Anatomie et Cytologie Pathologiques, Centre Hospitalier Universitaire de Saint-Étienne, Saint-Étienne, France5Département d'Hématologie et de Thérapie Cellulaire, Centre Hospitalier Universitaire de Saint-Étienne, Saint-Étienne, France5Département d'Hématologie et de Thérapie Cellulaire, Centre Hospitalier Universitaire de Saint-Étienne, Saint-Étienne, France5Département d'Hématologie et de Thérapie Cellulaire, Centre Hospitalier Universitaire de Saint-Étienne, Saint-Étienne, France3CIC EC 1408 INSERM, Centre Hospitalier Universitaire de Saint-Étienne, Saint-Étienne, FranceThrombocytopenia, 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.https://www.acpjournals.org/doi/10.7326/aimcc.2023.1018
spellingShingle Issam Drici-Tani
Lucile Grange
Martin Killian
Jean-Baptiste Gaultier
Michel Péoc'h
Manon Sapet
Ludovic Fouillet
Emmanuelle Tavernier-Tardy
Émilie Chalayer
Chronic Myelomonocytic Leukemia Associated With TAFRO Syndrome-Like Symptoms
Annals of Internal Medicine: Clinical Cases
title Chronic Myelomonocytic Leukemia Associated With TAFRO Syndrome-Like Symptoms
title_full Chronic Myelomonocytic Leukemia Associated With TAFRO Syndrome-Like Symptoms
title_fullStr Chronic Myelomonocytic Leukemia Associated With TAFRO Syndrome-Like Symptoms
title_full_unstemmed Chronic Myelomonocytic Leukemia Associated With TAFRO Syndrome-Like Symptoms
title_short Chronic Myelomonocytic Leukemia Associated With TAFRO Syndrome-Like Symptoms
title_sort chronic myelomonocytic leukemia associated with tafro syndrome like symptoms
url https://www.acpjournals.org/doi/10.7326/aimcc.2023.1018
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