Abstract
Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder caused by mutations (mainly p.M694V in exon 10) in the MEFV gene. It is the most common hereditary fever syndrome. Daily and lifelong colchicine administration can prevent both fever attacks and occurrence of inflammatory amyloidosis. Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell disorder classified as a myelodysplastic/myeloproliferative neoplasm. The median age of CMML diagnosis is 70 years and current treatment includes hydroxyurea and/or 5-azacitidine.
Highlights
Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder caused by mutations in the MEFV gene
ResultsThe patient, who was homozygous for the p.M694V mutation, was explored at the age of 83 for a profound doi:10.1186/1546-0096-13-S1-P177 Cite this article as: Awad et al.: Chronic myelomonocytic leukemia as a cause of fatal uncontrolled inflammation in familial Mediterranean fever
Summary
Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder caused by mutations (mainly p.M694V in exon 10) in the MEFV gene. It is the most common hereditary fever syndrome. And lifelong colchicine administration can prevent both fever attacks and occurrence of inflammatory amyloidosis. Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell disorder classified as a myelodysplastic/myeloproliferative neoplasm. The median age of CMML diagnosis is 70 years and current treatment includes hydroxyurea and/or 5-azacitidine
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