Mesothelioma in Familial Mediterranean Fever With Colchicine Intolerance: A Case Report and Literature Review
A 65-year-old Italian physician affected by Familial Mediterranean fever (FMF) was hospitalized due to progressive abdominal enlargement, which had begun 6 months before admission. Physical examination revealed ascites and bilateral leg edema. Abdominal CT scan showed ascitic fluid and extensive mul...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2020-05-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/article/10.3389/fimmu.2020.00889/full |
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| author | Rosa Talerico Carmine Cardillo Francesco De Vito Francesca Schinzari Manuel Soldato Maria Cristina Giustiniani Elena Verrecchia Raffaele Manna |
| author_facet | Rosa Talerico Carmine Cardillo Francesco De Vito Francesca Schinzari Manuel Soldato Maria Cristina Giustiniani Elena Verrecchia Raffaele Manna |
| author_sort | Rosa Talerico |
| collection | DOAJ |
| description | A 65-year-old Italian physician affected by Familial Mediterranean fever (FMF) was hospitalized due to progressive abdominal enlargement, which had begun 6 months before admission. Physical examination revealed ascites and bilateral leg edema. Abdominal CT scan showed ascitic fluid and extensive multiple peritoneal implants; peritoneal CT-guided biopsy revealed an epithelial-type malignant mesothelioma. The patient's past medical history revealed recurrent episodes of abdominal pain and fever from the age of 2. Clinical diagnosis of FMF was suspected at the age of 25, while genetic analysis, performed at the age of 50, confirmed homozygosity for the M694I mutation in the MEFV gene. Treatment with the first line FMF drug colchicine was started and stopped several times because of worsened leukopenia. The patient in fact had a history of asymptomatic leukopenia/lymphopenia from an early age; the intake of colchicine aggravated his pre-existing problem until the definitive suspension of the drug. As for second-line drugs, canakinumab was first prescribed, but due to prescription issues, it was not possible to be administered. When he was given anakinra, there was a worsening of leukopenia leading to septic fever. Systematic literature review indicates that, in most cases, recurrent peritoneal inflammation results in benign peritoneal fibrosis or less commonly in encapsulating peritonitis. There are only a few reported cases of recurrent peritoneal inflammation progressing from FMF to peritoneal mesothelioma (MST). In such cases, intolerance to colchicine or its erratic intake may lead to long-term recurrent inflammation, which usually precedes the development of the tumor, while pre-existing leukopenia, as in our patient, could also be a factor promoting or accelerating the tumor progression. In conclusion, we suggest that in the presence of intolerance or resistance to colchicine, interleukin (IL)-1 inhibition could suppress peritoneal inflammation and prevent MSTs. |
| first_indexed | 2024-12-19T13:29:10Z |
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| issn | 1664-3224 |
| language | English |
| last_indexed | 2024-12-19T13:29:10Z |
| publishDate | 2020-05-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj.art-161762937db442219dac36b41fdfc81a2022-12-21T20:19:27ZengFrontiers Media S.A.Frontiers in Immunology1664-32242020-05-011110.3389/fimmu.2020.00889523935Mesothelioma in Familial Mediterranean Fever With Colchicine Intolerance: A Case Report and Literature ReviewRosa Talerico0Carmine Cardillo1Francesco De Vito2Francesca Schinzari3Manuel Soldato4Maria Cristina Giustiniani5Elena Verrecchia6Raffaele Manna7Department of Internal Medicine and Gastroenterology, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, ItalyDepartment of Internal Medicine and Gastroenterology, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, ItalyDepartment of Internal Medicine and Gastroenterology, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, ItalyDepartment of Internal Medicine and Gastroenterology, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, ItalyDepartment of Internal Medicine and Gastroenterology, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, ItalyDepartment of Pathology, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, ItalyDepartment of Internal Medicine, Rare Diseases and Periodic Fevers Research Centre, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, ItalyDepartment of Internal Medicine, Rare Diseases and Periodic Fevers Research Centre, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, ItalyA 65-year-old Italian physician affected by Familial Mediterranean fever (FMF) was hospitalized due to progressive abdominal enlargement, which had begun 6 months before admission. Physical examination revealed ascites and bilateral leg edema. Abdominal CT scan showed ascitic fluid and extensive multiple peritoneal implants; peritoneal CT-guided biopsy revealed an epithelial-type malignant mesothelioma. The patient's past medical history revealed recurrent episodes of abdominal pain and fever from the age of 2. Clinical diagnosis of FMF was suspected at the age of 25, while genetic analysis, performed at the age of 50, confirmed homozygosity for the M694I mutation in the MEFV gene. Treatment with the first line FMF drug colchicine was started and stopped several times because of worsened leukopenia. The patient in fact had a history of asymptomatic leukopenia/lymphopenia from an early age; the intake of colchicine aggravated his pre-existing problem until the definitive suspension of the drug. As for second-line drugs, canakinumab was first prescribed, but due to prescription issues, it was not possible to be administered. When he was given anakinra, there was a worsening of leukopenia leading to septic fever. Systematic literature review indicates that, in most cases, recurrent peritoneal inflammation results in benign peritoneal fibrosis or less commonly in encapsulating peritonitis. There are only a few reported cases of recurrent peritoneal inflammation progressing from FMF to peritoneal mesothelioma (MST). In such cases, intolerance to colchicine or its erratic intake may lead to long-term recurrent inflammation, which usually precedes the development of the tumor, while pre-existing leukopenia, as in our patient, could also be a factor promoting or accelerating the tumor progression. In conclusion, we suggest that in the presence of intolerance or resistance to colchicine, interleukin (IL)-1 inhibition could suppress peritoneal inflammation and prevent MSTs.https://www.frontiersin.org/article/10.3389/fimmu.2020.00889/fullFamilial Mediterranean fever (FMF)malignant mesothelioma (MST)colchicineperitoneal recurrent inflammationcanakinumabanakinra |
| spellingShingle | Rosa Talerico Carmine Cardillo Francesco De Vito Francesca Schinzari Manuel Soldato Maria Cristina Giustiniani Elena Verrecchia Raffaele Manna Mesothelioma in Familial Mediterranean Fever With Colchicine Intolerance: A Case Report and Literature Review Frontiers in Immunology Familial Mediterranean fever (FMF) malignant mesothelioma (MST) colchicine peritoneal recurrent inflammation canakinumab anakinra |
| title | Mesothelioma in Familial Mediterranean Fever With Colchicine Intolerance: A Case Report and Literature Review |
| title_full | Mesothelioma in Familial Mediterranean Fever With Colchicine Intolerance: A Case Report and Literature Review |
| title_fullStr | Mesothelioma in Familial Mediterranean Fever With Colchicine Intolerance: A Case Report and Literature Review |
| title_full_unstemmed | Mesothelioma in Familial Mediterranean Fever With Colchicine Intolerance: A Case Report and Literature Review |
| title_short | Mesothelioma in Familial Mediterranean Fever With Colchicine Intolerance: A Case Report and Literature Review |
| title_sort | mesothelioma in familial mediterranean fever with colchicine intolerance a case report and literature review |
| topic | Familial Mediterranean fever (FMF) malignant mesothelioma (MST) colchicine peritoneal recurrent inflammation canakinumab anakinra |
| url | https://www.frontiersin.org/article/10.3389/fimmu.2020.00889/full |
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