Lamotrigine-associated haemophagocytic lymphohistiocytosis (HLH) confounded with underlying rheumatoid arthritis.

Haemophagocytic lymphohistiocytosis (HLH) is one of the rare haematological syndromes more commonly reported in infants/children than adults. This disease is known for its aggressive dysregulated immune response affecting the host rapidly, causing multiorgan dysfunction and thus carries a high mortality. The disease still remains cryptic in this current decade despite all the developments in the ever-evolving field of haematology. Due to its rare occurrence and being more frequent in infants and the paediatric population, the literature lacks enough data to standardise therapies. Such events in adults and the elderly are invariably related to an underlying insult such as infections, other autoimmune or rheumatological diseases or drugs. We describe an interesting case of a middle-aged Caucasian woman who presented with fever, pancytopenia and hepatitis, who was eventually diagnosed with HLH just in time to receive the life-saving specific treatment as per available guidelines.

Impact of Acquired Thrombocytopenia on Cardiovascular Outcomes in Patients With Coronary Artery Disease Undergoing Percutaneous Coronary Intervention: A Systematic Review and Meta-Analysis.

BACKGROUND: Acquired thrombocytopenia (aTP) is associated with a high frequency of bleeding and ischemic complications in patients undergoing percutaneous coronary intervention (PCI). Herein, we report a meta-analysis evaluating the adverse effects of aTP on cardiovascular outcomes and mortality post-PCI. METHODS: A literature search was performed using PubMed, Embase, Cochrane and, clinicaltrials.gov from the inception of these databases through October 2019. Patients were divided into two groups: 1) No Thrombocytopenia (nTP) and 2) Acquired Thrombocytopenia (aTP) after PCI. Primary endpoints were in-hospital, 30-day and all-cause mortality rates at the longest follow-up. The main summary estimate was random effects Risk ratio (RR) with 95% confidence intervals (CIs). RESULTS: Seven studies involving 57,247 participants were included. There was significantly increased in-hospital all-cause mortality (HR 10.73 [6.82-16.88]), MACE (HR 2.96 [2.24-3.94]), major bleeding (HR 4.78 [3.54-6.47]), and target vessel revascularization (TVR) (HR 7.53 [2.8-20.2]), in the aTP group compared to the nTP group. Similarly, aTP group had a statistically significant increased incidence of 30-day all-cause mortality (HR 6.08), MACE (HR 2.77), post-PCI MI (HR 1.98), TVR (HR 5.2), and major bleeding (HR 12.73). Outcomes at longest follow-up showed increased incidence of all-cause mortality (HR 3.98 [1.53-10.33]) and MACE (HR 1.24 [0.99-1.54]) in aTP group, while there was no significant difference for post-PCI MI (HR 0.94 [0.37-2.39]) and TVR (HR 0.96 [0.69-1.32]) between both groups. CONCLUSIONS: Acquired Thrombocytopenia after PCI is associated with increased morbidity, mortality, adverse bleeding events and the need for in-hospital and 30-day TVR.

Obstructive sleep apnea and peripheral vascular disease: a systematic review based on current literature.

INTRODUCTION: Obstructive sleep apnea (OSA) is an established risk factor for poor cardiovascular outcomes and coronary artery disease, but its influence on the development of peripheral artery disease (PAD) is not well established. The aim of our study was to understand the mutual prevalence of OSA and PAD and any reported statistical association between the two conditions. METHODS: PubMed, Ovid Embase, Web of Science, Cochrane library and clinicaltrials.gov databases were systematically searched up to 29 November 2018. A total of 844 articles were identified and 744 articles were screened for relevance. RESULTS AND CONCLUSION: Eleven prospective cohorts qualified for inclusion with N = 63,642 (M = 28,062, F = 35,494). All studies evaluated OSA severity primarily with apnea-hypopnea index (AHI) values. The overall prevalence of PAD was 20.5% (N = 13,068). Except for two studies, all studies reported an increased prevalence of OSA in patients with PAD. OSA severity was not found to have an association with poor ankle brachial index values or increasing daytime sleepiness as measured by Epworth sleepiness scale. Further prospective clinical trials are required to further delineate this finding.

Zinner Syndrome Unmasked by Workup for Renal Colic and Uncontrolled Hypertension.

Zinner syndrome is a rare hereditary disorder of the mesonephric duct. The triad of the absence of one kidney, ipsilateral cystic dilatation of the seminal vesicle, and ejaculatory duct obstruction makes the diagnosis. Mostly, it is asymptomatic. However, genitourinary manifestations and workup for the incidental absence of one kidney often uncover the disease. Ultrasound and CT scan can identify the absence of a kidney and seminal vesicle cyst, while MRI is the gold standard for diagnostic elaboration of the pelvic anatomy. In this article, we have presented a 51-year-old male patient who presented with renal colic and hypertension. Radiological investigations for the renal colic uncovered the diagnosis of Zinner syndrome incidentally. This case highlights the incidental nature, variability in the clinical presentation, and the diagnostic challenges of this rare disorder. It also emphasizes on the radiologist for a careful evaluation of the pelvic images in patients with unilateral absence of a kidney.