Disseminated intravascular coagulation in Stevens-Johnson syndrome and toxic epidermal necrolysis.

BACKGROUND: Patients with Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) have high mortality rates. Disseminated intravascular coagulation has been reported in SJS/TEN patients. The influence of this lethal complication in patients with SJS/TEN is not well known. OBJECTIVE: This study aimed to investigate the risk and outcomes of disseminated intravascular coagulation in patients with SJS/TEN. METHODS: We analyzed the disseminated intravascular coagulation profiles of patients receiving a diagnosis of SJS/TEN between 2010 and 2019. RESULTS: We analyzed 150 patients with SJS/TEN (75 with SJS, 22 with overlapping SJS/TEN, and 53 with TEN) and their complete disseminated intravascular coagulation profiles. Disseminated intravascular coagulation was diagnosed in 32 patients (21.3%), primarily those with TEN. It was significantly associated with systemic complications, including gastrointestinal bleeding, respiratory failure, renal failure, liver failure, infection, and bacteremia. Additionally, SJS/TEN patients with disseminated intravascular coagulation had elevated procalcitonin levels. Among patients with SJS/TEN, disseminated intravascular coagulation was associated with a greater than 10-fold increase in mortality (78.1% vs 7%). LIMITATIONS: The study limitations include small sample size and a single hospital system. CONCLUSION: Disseminated intravascular coagulation is a potential complication of SJS/TEN and associated with higher mortality. Early recognition and appropriate management of this critical complication are important for patients with SJS/TEN.

Detecting Lesional Granulysin Levels for Rapid Diagnosis of Cytotoxic T lymphocyte-Mediated Bullous Skin Disorders.

BACKGROUND: Bullous skin disorders are induced by different pathomechanisms and several are emergent, including Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN). Rapid diagnostic methods for SJS/TEN or cytotoxic T-lymphocyte (CTL)-mediated bullous disorders are crucial for early treatment. Granulysin, primarily expressed by CTLs, is a specific cytotoxic protein responsible for SJS/TEN and similar skin reactions. OBJECTIVE: To assess granulysin levels in blister fluids to differentiate SJS/TEN and similar CTL-mediated bullous reactions from other autoimmune bullous disorders. METHODS: Using ELISA, we measured granulysin in blister fluids from patients with bullous skin disorders, including SJS/TEN, erythema multiforme major, bullous fixed-drug eruption, bullous lupus erythematosus, paraneoplastic pemphigus, pemphigus vulgaris, bullous pemphigoid, purpura fulminans-related bullae, and hand-foot syndrome/hand-foot-skin reactions. We compared serum and blister granulysin levels in patients with SJS/TEN presenting varying severity, monitoring serial granulysin levels from acute to late stages. RESULTS: Overall, 144 patients presenting with bullous skin disorders were enrolled. Blister granulysin levels (mean ± SD) in CTL-mediated disorders, including TEN (n = 28; 3938.7 ± 3475.7), SJS-TEN overlapping (n = 22; 1440.4 ± 1179.6), SJS (n = 14; 542.0 ± 503.2), erythema multiforme major (n = 7; 766.3 ± 1073.7), generalized bullous fixed-drug eruption (n = 10; 720.4 ± 858.3), and localized bullous fixed-drug eruption (n = 16; 69.0 ± 56.4), were significantly higher than in non-CTL-mediated bullous disorders (P < .0001), including bullous lupus erythematosus (n = 3; 22.7 ± 20.1), paraneoplastic pemphigus (n = 3; 20.3 ± 8.6), pemphigus vulgaris (n = 3; 4.4 ± 2.8), bullous pemphigoid (n = 18; 4.0 ± 2.7), purpura fulminans (n = 4; 5.9 ± 5.5), and hand-foot syndrome/hand-foot-skin reactions (n = 6; 4.6 ± 3.5). Blister granulysin levels correlated with clinical severity of SJS/TEN (P < .0001). CONCLUSIONS: Determination of blister granulysin levels is a noninvasive and useful tool for rapid differential diagnosis of SJS/TEN and other similar CTL-mediated bullous skin disorders for treatment selection.

Recent advances in therapy of follicular lymphoma / 中国实验血液学杂志

Follicular lymphoma is a common pathological subtypes of lymphoma, it ranked only second to diffuse large B-cell lymphoma, accounts for 22% of non Hodgkin's lymphoma. Follicular lymphoma is a displaying biologically and clinically diverse disease. Patients may present indolent, asymptomatic disease or more aggressive, symptomatic disease with high tumor burden. Decision-making to treat in the frontline is based on histology manifestation, tumor burden and patient symptoms. Treatment for follicular lymphoma includes radiotherapy, chemotherapy, immunological therapy and autologous stem cell transplantation or allogeneic stem cell transplantation.Radiation therapy is the first treatment of early stage follicular lymphoma. For advanced follicular lymphoma chemotherapy, chemotherapy combined immunotherapy or immune radiotherapy, stem cell transplantation may be chosen. In recent years, drugs for lymphoma including bortezomib, lenalidomide, Ofatumumab, Epratuzumab and so on, and the therapeutic scheme present more and more update. In this article the advances of treatment for follicular lymphoma are summarzied.

Regulation of G protein-coupled receptor kinase 5 mRNA and protein level in rat brain by addictive drugs / 生理学报

G protein-coupled receptor kinase 5 (GRK5) plays an important role in the regulation of GPCR-transduced signals. Our previous study showed that acute administration of morphine could significantly increase GRK5 mRNA level in the cerebral cortex and hippocampus of the rat brain. The current study investigated the potential effects of acute administration of addictive drugs including morphine, heroine and cocaine on GRK5 mRNA level in the rat brain using in situ hybridization and analyzed the effects of acute and chronic morphine treatments on GRK5 protein level in the rat brain using Western blotting assay. Our results showed that 2 h after the initial morphine (10 mg/kg), cocaine (15 mg/kg) and heroine (1 mg/kg) treatment, the mRNA level of GRK5 in the parietal cortex increased about 110% (P<0.01), 70% (P<0.05) and 100% (P<0.01), respectively. In the temporal cortex, GRK5 mRNA level increased about 90% (P<0.01), 40% (P<0.05) and 80.0% (P<0.01), respectively . In the hippocampus, the mRNA level of GRK5 increased about 60% (P<0.01), 30% (P<0.05) and 80% (P<0.01). However, the mRNA level of GRK5 remained unchanged after acute morphine, cocaine or heroine treatment. In the cerebral cortex of the rat brain, the acute administration of morphine (NS-Mor) increased GRK5 protein level by about 60% while the chronic morphine treatment (Mor-Mor) increased GRK5 protein level even higher [about 130% compared with the control group (chronic saline treatment, NS-NS) group, P<0.01]. In the hippocampus, GRK5 protein level remained unchanged after acute administration of morphine (P>0.1),while the level of GRK5 protein tended to decrease after chronic morphine treatment (P=0.098). In the thalamus, acute morphine treatment caused no change in GRK5 protein level (P>0.1) while after chronic morphine treatment, GRK5 protein level decreased significantly (more than 90%, P<0.01), Taken together, our results indicate that addictive drugs can regulate GRK5 in the rat brain on protein level as well as on mRNA level and suggest that GRK5 may play a role in addiction of psychoactive substances.