The clinicopathological data of 13 patients with Langerhans cell histiocytosis (LCH) from January 2004 to January 2014 were retrospectively analyzed.There were 8 males and 5 females with a mean age of 35 (6-63)years.For all 13 cases,there were 15 lesions.Histologically,there was a diffuse distribution of Langerhans cells accompanied by a variable number of eosinophils and some other inflammatory cells.Immunohistochemical study showed tumor cells were positive for S-100 protein,CD1a and Langerin.During a follow-up period of 6-101 months,all survived.The diagnosis of LCH was assisted by immunophenotypic analyses for S-100,CD1a and Langerin.Combination therapy was effective.Most patients had an excellent prognosis.

Objective To examine the clinical features of sporadic patients with H7N9 avian influenzain Taizhou city of Zhejiang province and to characterize its viral genes.Methods Fifteen patients with H7N9 influenza infection confirmed by Zhejiang Provincial Centre for Disease Control and Prevention during January 201 4 and January 201 5 were included in the study.The basic diseases,poultry exposure history,clinical manifestation,laboratory examination,imaging features,treatment and outcome and viral gene sequencing were analyzed retrospectively.Results The first clinical symptoms were fever and cough in all patients,acuterespiratory distress syndrome(ARDS)occurred in 1 3 patients,the average time from onset to antiviral therapy was (7 ±2)d.Among 1 5 patients,9 survived and 6 died,including 2 died of multiple organ failure (MOF).The phylogenetic tree showed that there was highly homologous in hemagglutinin (HA)and neuraminidase(NA)genes between human H7N9 virus strains and poultry reference strains.The result of genetic sequencing indicated that human H7N9 virus strains had mutations at 226 (Q226L)sites in HA protein.Conclusions ARDS is likely to occur in patients with H7N9 viral infection,and early antiviral treatment usually leads to a good prognosis.With the occurrence of adaptive mutation in avian influenza virus H7N9,spread from poultry to the human beings may take place.

Objective: To study the clinicopathologic characteristics, immunophenotype and ALK gene alterations of gastrointestinal inflammatory myofibroblastic tumor. Methods: Clinical data, histological features and immunohistochemical results were analyzed in 7 cases of gastrointestinal inflammatory myofibroblastic tumor at Zhejiang Province Taizhou Hospital from January 2005 to December 2016. ALK gene status was investigated by ALK fluorescence in situ hybridization. Results: There were 4 female and 3 male patients. The age of patients ranged from 1 to 72 years (median age=53 years and mean age=40 years). The tumor was located in stomach (n=4), left hemicolon (n=1), right hemicolon (n=1) and rectum (n=1). Histologically, the tumors consisted of spindle fibroblast and myofibroblast cells growing in bundles with inflammatory infiltration primarily composed of plasma cells and lymphocytes. Immunohistochemical study showed spindle tumor cells were positive for vimentin (7/7), SMA (7/7), but were negative for CD34, CKpan, CD117, DOG1, S-100 and desmin. Two cases expressed ALK protein and fluorescence in-situ hybridization revealed the presence of ALK gene rearrangement in the both cases. Conclusions Gastrointestinal inflammatory myofibroblastic tumor is a rare neoplasm that is easily misdiagnosed. Its surgical removal is a reliable treatment. ALK may be a potential novel therapeutic target for inflammatory myofibroblastic tumor.

In recent years， new advances have been achieved for the research on the pathogenesis， diagnosis， and treatment of primary biliary cholangitis （PBC）. Accurate diagnosis， prognosis assessment， and risk stratification based on the clinicopathological features of patients are important for the treatment of PBC. This article summarizes the advances in the clinicopathological features and treatment of PBC.

Nine cases of mesenteric desmoid-type fibromatosis were diagnosed and treated in Taizhou Hospital, Wenzhou Medical University between January 2010 and May 2022, including 2 females and 7 males, aged 16 to 59 years. The lesions were in the mesentery of small intestine with 7 cases, ileocecal junction with 1 cases and transverse colon with 1 case. The tumors had an unclear boundary and no envelope, the section was solid, gray and tough. The mean maximum diameter was (10.7±8.5) cm (range 3.5-33.0 cm). Microscopically, fusiform fibroblasts and myofibroblasts were parallel, bunched or staggered, buried in a large amount of extracellular collagen. The cell morphology was relatively consistent, without obvious atypia, and mitosis was rare. Immunohistochemistry showed that the tumor cells were positive for vimentin (9/9), β-catenin (9/9), while smooth muscle actin (5/9) stains were focally positive. Ki-67 proliferation index was 1%-10%. Cytokeratin Pan, S-100, STAT6, CD117, DOG1, CD34, desmin and anaplastic lymphoma kinase stains were negative. Genetic analysis showed that there were 7 cases of c.121G>A(p.Thr41Ala) mutation of CTNNB1 gene, 1 case of c.121G>A(p.Thr41Ala) and 1 case of c.134C>T(p.Ser45Phe) double mutation, and 1 case of wild type. Tumors were surgically resected in all 9 cases. Eight cases had no recurrence or metastasis, 1 case had recurrence 6 months later, and no recurrence or metastasis after additional surgical resection.
Male ; Female ; Humans ; Fibromatosis, Aggressive/diagnosis* ; Immunohistochemistry ; Fibroblasts/metabolism* ; Mesentery/pathology* ; beta Catenin/analysis*

OBJECTIVETo study the prevalence of IgG4-positive plasma cells in Rosai-Dorfman disease and to assess the association between Rosai-Dorfman disease and IgG4-related sclerosing disease (IgG4-SD).

METHODSThe clinicopathologic features of 12 tissue samples of Rosai-Dorfman disease (11 extranodal and one nodal) from nine patients were reviewed. The degree of fibrosis and occlusive phlebitis was studied by HE staining. The expression of IgG4 and IgG in plasma cells were studied by immunohistochemistry (EnVision) and quantitatively analyzed by medical image analysis system.

RESULTSNine tissue samples showed different degree of fibrosis (four tissue samples were mild, one tissue sample was moderate and four tissue samples were severe) and two tissue samples showed occlusive phlebitis in the lesional tissue. Immunohistochemical study showed marked infiltration by IgG4-positive plasma cells (> 50 per high-power field) in four tissue samples, moderate infiltration (30 to 50 per high-power field) in two tissue samples, mild (10 to 29 per high-power field) in three cases and negative infiltration (< 10 per high-power field) in three tissue samples (P < 0.01). Three tissue samples fulfilled the diagnostic criteria of IgG4-SD (> 50 IgG4-positive plasma cells per high-power field and IgG4-to-IgG ratio > 40%), including one tissue sample each of Rosai-Dorfman disease in the left facial skin, above the left eye socket, and in the right parotid.

CONCLUSIONSSome cases of Rosai-Dorfman disease fulfill the diagnostic criteria and show the histologic features of IgG4-SD. They may represent members of the IgG4-SD spectrum. The detection of IgG4-positive plasma cells in the lesional tissues of Rosai-Dorfman disease may have clinical pathological significance.

Fibrosis ; Histiocytosis, Sinus ; diagnosis ; immunology ; Humans ; Immunoglobulin G ; chemistry ; Immunohistochemistry ; Phlebitis ; pathology ; Plasma Cells ; chemistry