New paraneoplastic syndrome in chronic basophilic leukemia

Chronic basophilic leukemia (CBL) is an extremely rare disorder. A female patient presented with recurrent attacks of chills, fever and abdominal pain was found to have simultaneous cyclic oscillation in leukocyte counts and C-reactive protein values. She was initially diagnosed with familial Medite...

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Bibliographic Details
Published in:International journal of hematology 2013-04, Vol.97 (4), p.498-504
Main Authors: Cehreli, Cavit, Ates, Halil, Cehreli, Ruksan, Sercan, Zeynep, Demirkan, Fatih
Format: Article
Language:English
Subjects:
Aged
Basophils - metabolism
Basophils - pathology
Biological and medical sciences
Blood Chemical Analysis
Bone Marrow - pathology
Case Report
Chromosome Aberrations
Female
Gene Expression
Hematologic and hematopoietic diseases
Hematology
Humans
Immunophenotyping
Interleukin-6 - blood
Interleukin-6 - genetics
Janus Kinase 2 - genetics
Leukemia, Myelogenous, Chronic, BCR-ABL Positive - complications
Leukemia, Myelogenous, Chronic, BCR-ABL Positive - diagnosis
Leukemia, Myelogenous, Chronic, BCR-ABL Positive - genetics
Leukemia, Myelogenous, Chronic, BCR-ABL Positive - pathology
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
Medical sciences
Medicine
Medicine & Public Health
Mutation
Oncology
Paraneoplastic Syndromes - diagnosis
Paraneoplastic Syndromes - etiology
Paraneoplastic Syndromes - genetics
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Summary:Chronic basophilic leukemia (CBL) is an extremely rare disorder. A female patient presented with recurrent attacks of chills, fever and abdominal pain was found to have simultaneous cyclic oscillation in leukocyte counts and C-reactive protein values. She was initially diagnosed with familial Mediterranean fever and treated with colchicine. Diagnosis of CBL was established by morphologic studies of peripheral blood and bone marrow. Her febrile attacks recurred with marked elevation in serum interleukin-6 (IL-6) level when basophil counts climbed to peak levels during cyclic oscillation. Molecular studies by real-time PCR showed IL-6 gene expression in neoplastic basophils separated by magnetic-activated cell sorting infiltrating the bone marrow, suggesting that IL-6 is released by neoplastic basophils of an underlying CBL, resulting in a new paraneoplastic syndrome that mimics autoinflammatory disorders.
ISSN:0925-5710
1865-3774
DOI:10.1007/s12185-013-1281-4