消化内镜在胃肠间质瘤诊治中的应用

胃肠间质瘤是消化道最常见的非定向分化的间叶性肿瘤。自从1983年Mazur等提出这一概念,人们在病因、病理机制、临床表现、诊断及治疗方法等方面进行了大量研究,对其认识逐步加深。此文主要对其内镜下的诊治研究进行综述。     

Localized intra-abdominal fibromatosis of the small bowel mimicking a gastrointestinal stromal tumor： A case report

Intra-abdominal fibromatosis (IAF) is a benign mesenchymal lesion that can occur throughout the gastrointestinal tract. Although rare, it is the most common primary tumor of the mesentery and can develop at any age. We describe a rare case of primary IAF involving the mesentery and small bowel which clinically, macroscopically and histologically mimicked malignant gastrointestinal stromal tumor (GIST). This report highlights the fact that benign IAF can be misdiagnosed as a malignant GIST localized in the mesentery or arising from the intestinal wall. Their diagnostic discrimination is essential because of their very different biological behaviors and the fact that the introduction of effective therapies involving tyrosine kinase inhibitor STI571 (imatinib mesylate) has greatly changed the clinical approach to intra-abdominal stromal spindle cell tumors.     

经术前治疗转化低位直肠间质瘤一例

胃肠道间质瘤累及直肠少见,仅占直肠恶性肿瘤的0.1%,但危险等级高,总体预后不良。尤其低位直肠间质瘤,更为罕见,既往治疗以手术为主,总体保肛率不高,并容易损伤邻近周围组织器官。现报道1例女性低位直肠间质瘤患者,予以口服伊马替尼术前治疗转化后,行肛门肿物切除术,术后继续予以口服伊马替尼,随访7个月未见复发。对于部分初始不可切除或切除困难,以及手术可能对相邻脏器功能产生较严重影响的特殊部位间质瘤,经伊马替尼进行术前治疗后有望获得手术机会且减少对脏器功能的损伤。     

Intra-abdominal desmoid tumors mimicking gastrointestinal stromal tumors——8 cases: A case report

BACKGROUND Intra-abdominal desmoid tumors(DTs) can mimic recurrence or progression of gastrointestinal stromal tumors(GISTs). Differential diagnosis is important to avoid unnecessary or inappropriate treatment.CASE SUMMARY All 8 patients experienced surgical resection of GIST, and median time to diagnosis of DT was 1.8 years after surgical resection. All sites of DT were in the peritoneum around the surgical sites of GIST. The following clinical suspicion coupled with radiological findings contributed to the suspicion of intraabdominal DTs:(1) Occurrence of a new single lesion in the peritoneum around the surgical sites of GIST;(2) uncontrolled lesion with imatinib while other lesions being controlled with imatinib;(3) well-defined ovoid shaped lesion with delayed or mild enhancement and absence of necrosis, hemorrhage, and cystic change on computed tomography; and(4) a lesion showing mild or no hypermetabolic activity on 18 fluorodeoxyglucose-positron emission tomography,contrary to initially hyperactive lesion of GIST. All DTs were surgically removed except for one unresectable DT and only one DT recurred at another site of peritoneum, which was also surgically removed.CONCLUSION Intra-abdominal DT should be a differential diagnosis for a new single lesion in patients with GIST.     

Unrelated donor bone marrow transplantation for children with juvenile myelomonocytic leukaemia

Children with juvenile myelomonocytic leukaemia (JMML) have a poor outcome, with survival in a minority of patients. The major limitation on success of sibling donor bone marrow transplantation for JMML has been reported to be relapse. A total of 46 children with a diagnosis of JMML underwent unrelated donor marrow (URD) transplantation facilitated by the National Marrow Donor Program. Forty-three of 46 patients had neutrophil engraftment at a median of 20 d post transplant, with platelet recovery in 28 of 40 evaluable patients at a median of 34.5 d. Thirty-two of 44 evaluable patients developed acute graft-versus-host-disease (GVHD) (Grades 2-4) and chronic GVHD developed in 14 of 35 evaluable patients. At a median follow-up of 2.0 years, probabilities of survival and disease-free survival were 42% and 24% respectively. The probability of relapse was 58% at 2 years and represents the major cause of treatment failure. Multivariate analysis revealed that chronic GVHD was associated with reduced relapse [risk ratio 0.20 (95% CI 0.04-1.02, P=0.05)] improved survival [risk ratio 0.13 (95% CI 0.03-0.68, P=0.02)] and event-free survival [risk ratio 0.23 (95% CI 0.06-0.94, P=0.04)]. This study demonstrates that relapse is the major cause of treatment failure in patients with JMML undergoing URD transplantation. With lower relapse observed in patients with chronic GVHD, new treatment strategies that focus on enhancing the graft-versus-leukaemia effect may improve survival.     

Improvement of interleukin 2 production,clonogenic capability and restoration of stromal cell function in human immunodeficiency virus-type-1 patients after highly active antiretroviral therapy

Haematological abnormalities frequently occur in patients infected by human immunodeficiency virus-type 1 (HIV-1). Increasing evidence indicates that bone marrow suppression (BM) results from viral infection of accessory cells, with impaired stromal function and alteration of haematopoietic growth factor network. We have investigated the effects of antiretroviral therapy on cytokine and chemokine production by BM cells and stromal cells in a group of HIV-1-infected subjects before and during treatment. Compared with uninfected controls, an altered cytokine and chemokine production by BM cells was observed before treatment, characterized by decreased interleukin 2 (IL-2) and elevated tumour necrosis factor-alpha, macrophage inflammatory protein (MIP)-1alpha, MIP-1beta, and RANTES (regulated on activation, normal T cell-expressed and secreted) levels, along with a defective BM clonogenic activity. Antiretroviral therapy showed increased BM clonogenic capability, associated with normalization of IL-2 production and chemokine receptors expression on CD34+ cells. Pre-therapy, BM accessory cells were represented by macrophage-like cells, in some cases positive for HIV-1 DNA, suggesting that these cells are the main target of HIV-1 infection. During therapy, the stromal cells became predominantly fibroblastoid-like, as observed in normal controls, and were negative for HIV-1 DNA. Controlling HIV-1 replication may produce amelioration of stem cell activity, and restoration of stromal cell pattern and functions, with increased IL-2 production at BM level.     

哮喘患者诱导痰中TSLP、IL-25水平测定及意义

目的探讨胸腺基质淋巴细胞生成素（TSLP）、IL-25在支气管哮喘（下称哮喘）发病中的作用。方法选择急性发作期及慢性期哮喘患者各15例（哮喘组）及健康查体者15例（对照组）,采用超声雾化吸入4％高渗盐水法采集诱导痰标本,以ELISA法测定诱导痰中髑LP、IL-25水平,分析两者相关性。结果哮喘组诱导痰中TSLP及IL-25水平均显著高于对照组,且急性发作期显著高于慢性期（P均〈0．05）。结论TSLP、IL-25可能在哮喘发生、发展中具有重要作用,有望作为哮喘治疗的潜在靶点。