自身免疫性胰腺炎13例临床诊治分析

目的：分析并总结我院收治的自身免疫性胰腺炎（ AIP）临床特征及诊治经验。方法回顾性分析2009年至2013年我院收治的13例AIP的临床资料。结果13例AIP中,中位年龄48岁,临床表现主要为上腹疼痛不适,梗阻性黄疸及体重下降。胰腺弥漫性增大及局限性增大各6例,胰体尾假性囊肿及胰腺结石各1例,胰管扩张4例。胰腺外器官受累9例（胆管病变8例、胆囊病变7例、腹膜后纤维化1例、腹腔淋巴结肿大6例、脾静脉变细4例、干燥综合征及溃疡性结肠炎各1例）。7例行血免疫标志物检查,IgG升高5例,类风湿因子（ RF）及抗核抗体（ ANA）升高各3例。8例被误诊而行手术治疗。经激素治疗后,患者预后良好。结论 AIP是一种少见的自身免疫性疾病,无特异临床表现,易与胰腺癌及胆管癌混淆,误诊率高。必须充分认识和重视AlP的诊治,避免因误诊而行不必要的手术治疗。     

Plexiform hypoglossal schwannoma of the tongue and the submandibular region

ABSTRACT: Hypoglossal schwannomas usually develop in the intracranial portion of the brain. Incidence of hypoglossal schwannomas of the submandibular region is extremely rare. A 27-year-old patient presented to us with hypoglossal schwannoma of the tongue and the submandibular region. The tumor was excised intraorally combined with submandibular approach. Histopathologic examination revealed a plexiform schwannoma (a rare variant).     

Thrombotic Thrombocytopenic Purpura and Relapes: Why do case series differ?

We examined the retrospective case series of the Itallan Cooperative Group to determine the incidence of relapes in TTP patients. Of 60 patients who have crossed the 10-year threshold from the first episode, Only 9 (15%) relapsed during that period, a figure far lower than that reported recently. Such difference is hardly explainable on the basis of our current knowledge of the biological behaviour of TTP. Furthermore, we unsuccessfully analyzed the treatment performed in each of our relapsed patients, in search of some element that could retrospectively predict the subsequent relapse     

SIE,SIES, GITMO revised guidelines for the management of follicular lymphoma

By using the GRADE system, we updated the guidelines for management of follicular cell lymphoma issued in 2006 from SIE, SIES, and GITMO group. We confirmed our recommendation to frontline chemoimmunotherapy in patients with Stage III–IV disease and/or high tumor burden. Maintenance rituximab was also recommended in responding patients. In patients relapsing after an interval longer than 12 months from frontline therapy, we recommended chemoimmunotherapy with non cross‐resistant regimens followed by rituximab maintenance. High dose chemotherapy followed by hematopoietic stem cell transplant was recommended for young fit patients who achieve a response after salvage chemoimmunotherapy. Am. J. Hematol. 88:185–192, 2013. © 2012 Wiley Periodicals, Inc.     

Treatment of Kasabach-Merritt syndrome by embolisation of a giant liver hemangioma.

We report the case of a 14-month-old child with Kasabach-Merritt Syndrome, due to a giant liver hemangioma. The therapeutic approach consisted of peripheral transcatheter embolisation of the right hepatic artery with Ivalon microspheres without the addition of thrombogenic material. This procedure brought to a sensible permanent reduction of the size of the liver hemangioma with normalisation of the previous altered coagulation parameters after 6 years of follow-up.     

Upfront high-dose sequential therapy (HDS) versus VACOP-B with or without HDS in aggressive non-Hodgkin's lymphoma: long-term results by the NHLCSG.

BACKGROUND: There is not univocal concordance for using high-dose sequential therapy (HDS) as first-line treatment for aggressive non-Hodgkin's lymphoma (NHL). We designed this study to evaluate the usefulness of HDS followed by high-dose therapy (HDT) with autologous stem cell transplantation as front-line treatment in different subsets of aggressive NHL. PATIENTS AND METHODS: Among 223 patients aged 15-60 years with aggressive, advanced stage NHL, 106 patients were randomized to VACOP-B (etoposide, doxorubicin, cyclophosphamide, vincristine, prednisone, bleomycin) for 12 weeks (plus HDS/HDT in case of persistent disease) (arm A), and 117 patients to VACOP-B for 8 weeks plus upfront HDS/HDT (arm B). RESULTS: According to the intention-to-treat analysis, the complete response rate was 75% for arm A and 72.6% for arm B. With a median follow-up of 62 months there was no difference in 7-year probability of survival (60% and 57.8%; P = 0.5), disease-free survival (DFS) (62% and 71%; P = 0.2) and progression-free survival (PFS) (44.9% and 40.9%; P = 0.7) between the two arms. Subgroup analyses confirmed that the best results in terms of survival, DFS and PFS were achieved by patients with large B-cell NHL without bone marrow (BM) involvement, independently of the treatment arm. Results were poorer in other categories of patients and poorest in patients with BM involvement. CONCLUSIONS: Aggressive NHL patients do not benefit from upfront HDS/HDT.     

SARS-CoV-2 infection in patients with chronic lymphocytic leukemia: The Italian Hematology Alliance on COVID-19 cohort

COVID-19, the disease caused by SARS-CoV-2, is still afflicting thousands of people across the globe. Few studies on COVID-19 in chronic lymphocytic leukemia (CLL) are available. Here, we analyzed data from the CLL cohort of the Italian Hematology Alliance on COVID-19 (NCT04352556), which included 256 CLL patients enrolled between 25 February 2020 and 1 February 2021. Median age was 70 years (range 38–94) with male preponderance (60.1%). Approximately half of patients (n = 127) had received at least one line of therapy for CLL, including 108 (83.7%) who were on active treatment at the time of COVID-19 or received their last therapy within 12 months. Most patients (230/256, 89.9%) were symptomatic at COVID-19 diagnosis and the majority required hospitalization (n = 176). Overall, after a median follow-up of 42 days (IQR 24–96), case fatality rate was 30.1%, and it was 37.5% and 24.4% in the first (25 February 2020–22 June 2020) and second wave (23 June 2020–1 February 2021), respectively (p = 0.03). At multivariate analysis, male sex (HR 1.82, 95% CI 1.03–3.24, p = 0.04), age over than 70 years (HR 2.23, 95% CI 1.23–4.05, p = 0.01), any treatment for CLL given in the last 12 months (HR 1.72, 95% CI 1.04–2.84, p = 0.04) and COVID-19 severity (severe: HR 5.66, 95% CI 2.62–12.33, p < 0.0001; critical: HR 15.99, 95% CI 6.93–36.90, p < 0.0001) were independently associated with poor survival. In summary, we report a dismal COVID-related outcome in a significant fraction of CLL patients, that can be nicely predicted by clinical parameters.