Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Felty syndrome, a rare extra-articular manifestation of rheumatoid arthritis (RA), usually affects patients with long-standing disease. Patients with this syndrome typically present with neutropenia, splenomegaly, in addition to erosive RA. The development of unexplained neutropenia in healthy patients should prompt the work up for Felty syndrome, especially in patients with suggestive demographics, signs, and symptoms. Differentiation between large granular lymphocyte (LGL) leukemia and Felty syndrome is necessary as both can present with neutropenia, and are associated with RA. Immunosuppressive therapy has improved the prognosis of patients with Felty syndrome given the decreasing rates of splenectomies done in those patients over the last decades.
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PMID:Severe Neutropenia Complicated with Necrotizing Fasciitis Unveils a Diagnosis of Rheumatoid Arthritis: A Case Report. 3101 57

Large granular lymphocyte leukemia (LGL) is a clonal, lymphoproliferative disorder with an indolent disease course. T-cell LGL (T-LGL) is the most common type of LGL driven from T-cell lineage (85%). The coexistence of T-LGL with several types of autoimmune disorders, mostly rheumatoid arthritis (RA), has been reported. Felty's syndrome (FS) is defined by splenomegaly, low neutrophil count, and destructive arthritis and is usually seen in <1% of patients with RA. About 30% to 40% of patients with FS have been reported to have an expansion of large granulated lymphocytes in the circulation. FS and T-LGL are similar in terms of clinical manifestations, response to immunosuppressive therapy, their smoldering course, and immunogenetic findings, proposing FS and T-LGL with RA might be different aspects of a single disease spectrum. In this article, we present a case with long-standing RA who had never been on DMARD (Disease Modifying Anti-Rheumatic Drugs) treatment found to have constitutional symptoms, neutropenia, and splenomegaly, and the patient was diagnosed with T-LGL.
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PMID:T-Cell Large Granular Lymphocyte Leukemia in a Patient With Rheumatoid Arthritis. 3264 39


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