Gigantic splenomegaly in a 27-year-old male of South-East Asian descent with concurrent diagnosis of myeloproliferative neoplasm and hemoglobin H disease

2013 ◽  
Vol 91 (3) ◽  
pp. 284-285
Author(s):  
Yang Shi ◽  
Evan Kulbacki ◽  
David H. K. Chui ◽  
Endi Wang
2019 ◽  
Vol 12 ◽  
pp. 117954411989085 ◽  
Author(s):  
Ena Sharma ◽  
Brian Pedersen ◽  
Robert Terkeltaub

Objective: The interleukin-1 (IL-1) receptor antagonist anakinra is an effective, off-label option in acute gout flares, when conventional therapy options are narrowed. We performed a retrospective, randomized, case-controlled study to gain clinical insight on baseline factors for gout patients most likely to receive anakinra, and ultimate mortality of those who received anakinra. Methods: Of 1451 gout patients seen between January 2003 and January 2015 in a Veterans Affairs (VA) rheumatology group practice, under stringent managed care principles, 13 (100% male), who received anakinra at least once for flares, were compared with 1:4 age- and sex-matched gout controls. Each patient’s first rheumatology encounter was studied by factor analysis for variables associated with later anakinra. Results: At baseline, patients that received anakinra had higher urate burden (palpable tophi [10/13] vs controls [16/52], P = .003), serum urate ([10.6 mg/dL] vs controls [7.6 mg/dL], P < .0001), and East Asian descent ([7/13] vs [16/52], P = .041). The anakinra group had higher ultimate all-cause mortality ([6/13] vs controls [7/52], relative risk [RR] = 3.43, 95% confidence interval [CI] = 1.39-8.48, P = .0076). Factor analysis showed baseline visit palpable tophus and statin use to be most strongly associated with later anakinra use. Increased mortality of anakinra users, as per a factorial analysis, was linked more strongly to comorbidities than to anakinra. Conclusions: At baseline rheumatology gout encounter, higher urate, palpable tophi, statin prescription, and East Asian descent were associated with later anakinra use for flares. Mortality was more closely associated to the presence of comorbidities at baseline rheumatology visit than to anakinra prescription.


2016 ◽  
Vol 23 (1) ◽  
Author(s):  
Stacy L. McAllister ◽  
Katherine Sun ◽  
Eric R. Gross

Tumor Biology ◽  
2015 ◽  
Vol 37 (5) ◽  
pp. 6379-6387 ◽  
Author(s):  
Joy Shi ◽  
Anne Grundy ◽  
Harriet Richardson ◽  
Igor Burstyn ◽  
Johanna M. Schuetz ◽  
...  

Author(s):  
Lihong Chen ◽  
Zhuo Zhao ◽  
Zhen Wang ◽  
Ying Zhou ◽  
Xin Zhou ◽  
...  

AbstractResident physician training is associated with a substantial increase in depression in the United States, with rates increasing from about 4% before internship to 35% at least once during the first year of residency1.Here, we sought to assess whether the rate of depression among residents in China are similar to their US counterparts and identify the common and differential predictors of depression in the two training systems. We assessed 1006 residents across three cohorts (2016-2019) at 16 affiliated hospitals of Shanghai Jiao Tong University and Peking Union Medical College. In parallel, we assessed three cohorts of 7028 residents at 100+ US institutions.At the Chinese institutions, similarly, the proportion of participants who met depression criteria increased from 9% prior to residency to 35% at least once during the first year of residency (P<0.0001), an increase similar in magnitude to residents during internship in US institutions. Among factors assessed before residency, prior history of depression and depressive symptom score at baseline were common factors associated with depression during residency in both China and the US. In contrast, neuroticism and early family environment were strongly associated with depression risk in the US but not in China. Young age was a predictor of depression in China but not in the US sample. Among residency training factors, long duty hours and reduced sleep duration emerged as predictors of depression in both China and the US.To gain insight into whether differences in personal predictors between the residents in China compared to the US residents were driven more by differences between cohorts, or by training system differences, we compared US residents of East Asian descent to other US and Chinese residents. We found that for most predictors (age, Neuroticism, early family environment), US residents of East Asian descent were more similar to other US residents than to the residents training in China.Overall, the magnitude of depression increase and work-related drivers of depression were similar between China and the US, suggesting a need for system reforms, and that the types of effective reforms may be similar across the two systems.


2019 ◽  
Vol 23 (6) ◽  
pp. 595-601 ◽  
Author(s):  
Marisa Grace Ponzo ◽  
Monica Miliszewski ◽  
Mark G. Kirchhof ◽  
Paul A. Keown ◽  
Jan P. Dutz

Background and objective East Asians exposed to the urate-lowering drug allopurinol have a predilection for severe cutaneous drug reactions such as drug-induced hypersensitivity syndrome or drug reaction with eosinophilia and systemic symptoms (DRESS) and Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN). Screening is recommended in patients of East Asian descent for the presence of HLA-B*58:01 prior to allopurinol initiation to avoid these complications. Utilization rates of the HLA-B*58:01 predictive screening test within the Greater Vancouver area, which has a population composed of 40.1% people of East Asian descent, are unknown. Measures We identified cases of DRESS or SJS/TEN due to allopurinol using the Vancouver General Hospital dermatology consult service database. We next compared the frequency in which the HLA-B*58:01 screening test was ordered since 2012 to the estimated frequency of new prescriptions for allopurinol prescribed for the management of gout among the East Asians. Results We report 5 cases of East Asian patients exposed to allopurinol for management of gout between 2012 and 2016, who developed DRESS (4 patients) or SJS/TEN (1 patient). All were of HLA-B*58:01 genotype, representing preventable cases. The HLA-B*58:01 test was ordered 6 times in 2012, whereas the estimated number of new cases of allopurinol-prescribed gout among patients of East Asian descent during that time period was 13. For 2012, testing was ordered for only 46% of at-risk patients. Conclusion We continue to observe cases of severe cutaneous drug reactions among high-risk individuals due to allopurinol exposure. The HLA-B*58:01 screening test for allopurinol hypersensitivity is underutilized in our geographic area.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 326-326 ◽  
Author(s):  
Russell K. Brynes ◽  
Attilio Orazi ◽  
Kalpana Bakshi ◽  
Christine K Bailey ◽  
Geoffrey W. Chan

Abstract Introduction Eltrombopag, a thrombopoietin receptor agonist (TPO-RA), increases platelet counts in patients with cITP. TPO-RAs have been associated with varying degrees of bone marrow (BM) reticulin increases. Patients in the EXTEND study, an ongoing, open-label extension study of eltrombopag in patients with heavily pretreated cITP who received eltrombopag or placebo in a previous eltrombopag study, underwent annual BM biopsies while on study. Methods Central morphology review was done by 2 hematopathologists blinded to clinical information who assessed specimens for cellularity, megakaryocyte, erythroid, and myeloid quantity and appearance; trabecular bone changes; and reticulin and collagen deposition (European Consensus scale). Results In EXTEND, 302 patients with cITP were treated with eltrombopag for ≤6.5 years as of February 2013. Of these, 232 BM biopsy specimens collected from 117 patients from pretreatment and up to 5.5 y on treatment were available at the time of this analysis, and were centrally reviewed (Table). Of these, 5 patients provided a biopsy sample within <10 mo on treatment, 48 patients at 1 y, 68 at 2 y, 51 at 3 y, 30 at 4 y, and 11 at 5 y on treatment. Median age (range) of the 117 patients was 50 (20-78) y; 69 (59%) patients were female; 85% were Caucasian/European, 9% were East Asian, 2% were South East Asian, and 4% were Arabic/North African. Median time since ITP diagnosis was 58.6 (8.5-421) mo. Two hundred and nine on-treatment biopsies, collected from 115 patients, were graded for reticulin using the European Consensus scale. Most (63-80%) of the on-treatment biopsies, collected between 1 y and 5 y, were MF-0 or MF-1 (17-33%) (Figure). Reticulin fibers were increased (MF ≥2) in 5 specimens from 2/115 (1.7%) patients. All 5 specimens were positive for collagen, and 3 of the 5 specimens in the 2 patients were judged as hypercellular. Of the 2 patients, 1 patient (East Asian) had reticulin documented after 2 y (970 days) of treatment, and was preemptively withdrawn from the study. Per protocol, a post-treatment biopsy was performed; a BM specimen taken 7 mo following withdrawal was graded as MF-0 for reticulin and negative for collagen. The second patient (Caucasian) had MF-2 at 1 y, which increased to MF-3 at 2 y and contained megakaryocytes with myeloproliferative neoplasm-like features, and then returned to MF-2 at the 3 y and 4 y assessments; however, these biopsies at 3 y and 4 y showed no signs of myeloproliferative neoplasm-like features and had reverted to normal. The patient remains on treatment at the time of this analysis. Neither patient experienced any adverse event or abnormality in hematologic parameters potentially related to impaired BM function. Cellularity was normal in the majority of patients at each assessment timepoint. Most (82-99%) patients demonstrated megakaryocytic hyperplasia. Megakaryocyte morphology was normal in all patients in all years except for 1 patient in year 1. Seven patients (6%) had megakaryocytes reminiscent of those seen with polycythemia vera or essential thrombocythemia. Erythroid hyperplasia was observed in 13-27% and myeloid hyperplasia in 6-13% of patients. Trabecular bone thinning was found in 39-70% of patients over the 5-year observation period. No pathologic pattern of changes was identified among the on-treatment specimens. Discussion Reticulin fibrosis was considered a potential risk for cITP patients receiving TPO-RAs. In the ongoing EXTEND study, at the time of this analysis, reticulin was either absent or mildly increased in 113/115 (98%) patients treated with eltrombopag up to 5.5 years at the time of their biopsy. No patient with reticulin or collagen increases showed clinical signs or symptoms indicative of BM dysfunction, and the findings were reversible upon discontinuation of treatment. No pattern of increasing reticulin deposition with longer treatment periods was observed based on BM biopsy data available to date. These data suggest that for most cITP patients, treatment with eltrombopag is not associated with the development of BM reticulin or collagen fibrosis; however, the potential association of TPO-RAs and increased BM fibrosis in cITP still needs to be better understood. Disclosures: Brynes: GlaxoSmithKline: Research Funding. Bakshi:GlaxoSmithKline: Employment, Equity Ownership. Bailey:GlaxoSmithKline: Employment, Equity Ownership. Chan:GlaxoSmithKline: Employment.


2018 ◽  
Vol 42 (3) ◽  
pp. 613-623 ◽  
Author(s):  
Lara A. Ray ◽  
ReJoyce Green ◽  
Daniel J.O. Roche ◽  
Spencer Bujarski ◽  
Emily E. Hartwell ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document