Chimeric antigen receptor (CAR) T cell–related HLH/MAS is an unusual manifestation of severe cytokine release syndrome (CRS) with poor prognosis and a challenging diagnosis. The establishment of specific diagnosis criteria is essential, and the combination of several techniques for CAR T‐cell follow‐up, allows a more precise management of this complication.
31 August 2019 in Intractable & Rare Diseases Research
Lemièrre's syndrome (LS) is an uncommon disease characterized by septic thrombophlebitis of the jugular vein in the context of otorhinolaryngologic infections. These patients are often young and the pharyngotonsillar infection is the most frequent primary focus, but other foci like acute otitis media or otomastoiditis have been described. Although the internal jugular vein is the most commonly affected site, a few case reports have been published with thrombosis of other veins, such as the facial vein or transverse sinus. We report the case of a 93-year-old woman with an atypical presentation of LS presenting with thrombophlebitis of the internal jugular vein, transverse sinuses and Herophili torcula after an acute otitis media complicated with acute otomastoiditis. Infectious cerebral venous thrombosis (CVT) is rare and accounts for 6-12% of the total in large adult series and is usually associated to otorhinolaryngologic infections. CVT is an atypical presentation of LS that can be potentially lethal, especially during the acute phase. For this reason, clinical suspicion and early treatment are vital to improve the prognosis of these patients. Although surgical treatment is recommended in cases of LS complicated with CVT, conservative management with antibiotics and anticoagulation lead to ad integrum restitutio without neurological sequelae in our case, suggesting that surgical treatment may not be necessary in all cases of LS complicated with CVT.
The optimal strategy for early surveillance after first complete response is unclear in Hodgkin lymphoma. Thus, we compared the various follow-up strategies in a multicenter study. All the included patients had a negative positron emission tomography/computed tomography at the end of induction therapy. From January 2007 to January 2018, we recruited 640 patients from 15 centers in Spain. Comparing the groups in which serial imaging were performed, the clinical/analytical follow-up group was exposed to significantly fewer imaging tests and less radiation. With a median follow-up of 127 months, progression-free survival at 60 months of the entire series was 88% and the overall survival was 97%. No significant differences in survival or progression-free survival were found among the various surveillance strategies. This study suggests that follow-up approaches with imaging in Hodgkin lymphoma provide no benefits for patient survival, and we believe that clinical/analytical surveillance for this group of patients could be the best course of action.