@entity15835 complicating XXXX : A single center case series and comparison with literature.

@entity1 @entity3329 @entity11533 @entity1910 @entity409 @entity15835 @entity177 @entity5642 @entity205 @entity11534 @entity4841 @entity221 @entity491
OBJECTIVES: @entity15835 ( @entity15835 ) is a life-threatening condition that can complicate @entity11533 ( @entity11533 ). Due to its rarity, there is no clear consensus concerning treatment recommendations and outcomes. We studied the clinical manifestations and outcomes of a relatively large cohort of @entity1 with @entity15835 and @entity11533 , and compared the data with the literature reports. METHODS: We performed a retrospective review of 7 adult @entity1 with @entity15835 complicating @entity11533 at the Cleveland Clinic (CCF) over 7 years. All @entity1 underwent bone marrow biopsies. Through MEDLINE and PubMed literature searches, we identified 48 cases of @entity15835 / @entity11533 . We compared the data of the CCF and literature cohorts. RESULTS: We identified 7 @entity1 with @entity15835 complicating @entity11533 (6 females and 1 males) for our CCF cohort, with 4 cases simultaneously presenting with @entity15835 and @entity11533 . The mean age at diagnosis of @entity15835 was 41.9    20.2 years and mean follow-up time was 18.6    16.0 months. All @entity1 had @entity221 , @entity4841 , and typical @entity1910 ; 6 had @entity491 , 4 had sore throat, and 3 had @entity3329 . These @entity1 with @entity11533 also had @entity15835 , with @entity177 and disseminated @entity5642 in 4, lung involvement in 3, and @entity11534 and shock in 2. There was significant @entity409 in all @entity1 and 6 had bi-cytopenias. At onset of @entity15835 , all 7 @entity1 had active @entity11533 . In addition to systemic glucocorticoids, 5 @entity1 received anakinra, with 3 @entity1 receiving combination therapy with @entity205 . We also identified 48 cases (35 females and 13 males) for the literature cohort with the mean age at diagnosis of @entity15835 of 40.2    16.0 years and mean follow-up time of 17.5    32.3 months. While the 2 cohorts were similar clinically, in the CCF cohort, more @entity1 had @entity177 (p < 0.001), higher soluble IL-2 receptor level (p = 0.01), and lower ESR (p = 0.02) as compared with the literature cohort. All of our @entity1 survived with a better outcome than the literature cohort. CONCLUSION: @entity15835 can be a serious complication of active @entity11533 . Our study of a relatively large cohort in conjunction with literature suggests that prompt recognition and treatment with early addition of anakinra, systemic glucocorticoids, and @entity205 as a triple regimen may improve clinical outcomes.
