2011年 · 第124卷第05期

For decades now, transplant physicians, during follow-up of successfully transplanted renal failure patients, have ordered frequent checks of the serum creatinine and urinalysis.1 These remain commonplace tools. Blood pressure measurement is preformed on every clinic visit along with a check of body weight and a check for edema。

Acute antibody mediated rejection (AMR) is receiving more and more attention, which is mediated by different mechanisms from T cell mediated rejection, thereby requiring other approaches to prevention and treatment. Preexisting alloantibodies and pre-transplant sensitization are important risk factors for development of acute AMR early after renal transplantation。

Abstract：Background The status of sensitization in kidney transplant recipients in the last 10 years and the trend of induction and maintenance therapy in patients of different panel-reactive antibody (PRA) levels have not been analyzed. The aim of this study was to investigate the current status of pre-transplant sensitization and its association with graft outcome.Methods A total of 155 570 kidney transplants reported to United Network for Organ Sharing (UNOS) during 2000-2009 were included in this study. We investigated the current status of pre-transplant sensitization and its association with graft outcome, and also compared the usage trend of 16 induction agents and 7 maintenance immunosuppressants in patients at different PRA levels. The difference of distributions of categorical variables between groups was investigated using the chi-square test. Unpaired t test or one-way analysis of variance (ANOVA) were used for numerical variables. The survival rates of transplant recipients were estimated using Kaplan-Meier methods and significance was determined by Log-rank test. Two-side P value ＜0.05 was considered statistically significant. All statistical analyses were performed using STATA 10 with all available updates as of March 2010 (StataCorp LP, College Station, Texas 77845, USA).Results Despite the fact of the decreased percentages of kidney transplant recipients with presensitization history, the mean PRA levels of all kidney recipients has been increasing in the last 7 years, which was possibly due to the introduction of more sensitive antibody testing techniques. The percentage of patients with treated rejection episodes within one year post-transplant were significantly higher in sensitized patients (PRA=50%-100%:14.3% and PRA=1%-49%:13.9%) than in non-sensitized patients (12.4%). Both 1- and 5-year graft survival rates improved in the last 10 years; this was more significant in high PRA patients. Thymoglobulin was the most commonly used induction agent in last 10 years. Its users increased from 10% to 46% in non-sensitized patients, from 12% to 57% in PRA 1%-49% patients,and from 19% to 63% in PRA 50%-100% patients. The users of Campath, intravenous immunoglobulin (IVIG), and Rituximab have been increasing and reached 16%, 20%, and 11% in highly sensitized patients. In the last 5 years,steroid-free patients were 33%-36%, 30%-37%, and 10%-25% for PRA 0, 1%-49%, and 50%-100% respectively.Almost 90% of patients were on Prograf at discharge. It seems that Myfortic users have been increasing since 2005 and it may soon replace mycophenolate mofetil (MMF) if long-term follow-up study conforms its safety and efficacy.Conclusions Application of sensitive antibody testing techniques increased the mean PRA levels of transplant recipients in spite of a decreased percentage of sensitized recipients. Induction and maintenance therapy differed in patients at different PRA levels。

Abstract：Background Transforming growth factor-β1 (TGF-β1) is known to be a key fibrogenic cytokine in a number of chronic fibrotic diseases, including chronic allograft nephropathy. We examined the effects of inhibition of TGF-β1 expression by RNA interference on renal allograft fibrosis, and explored the mechanisms responsible for these effects.Methods A Sprague-Dawley-to-Wistar rat model of accelerated kidney transplant fibrosis was used. Sixty recipient adult Wistar rats were randomly divided into four groups: group T (sham-operated group), group T (plasmid-transfected group), group H (control plasmid group), and group Y (transplant only group). Rats in group T were transfected with 200μg of TGF-β1 short hairpin RNA (shRNA). Reverse transcription-polymerase chain reaction and Western blotting were used to examine the expression of TGF-β1, Smad3/7, E-cadherin, and type I collagen. The distribution of type I collagen was measured by immunohistochemistry. The pathologic changes and extent of fibrosis were assessed by hematoxylin and eosin and Masson staining. E-cadherin and α-smooth muscle actin immunohistochemical staining were used to label tubular epithelial cells and fibroblasts, respectively.Results Plasmid transfection significantly inhibited the expression of TGF-β1, as well as that of its target gene, type I collagen (P ＜0.05 and P ＜0.01, respectively). In addition, the degree of fibrosis was mild, and its development was delayed in plasmid-transfected rats. In contrast, TGF-β1-shRNA transfection maintained the expression of E-cadherin in tubular epithelial cells while it inhibited the transformation from epithelial cells to fibroblasts. Blood urea nitrogen and serum creatinine were lower in the plasmid group than in the control groups (P ＜0.05 and P＜0.01, respectively).Conclusions This study suggests that transfection of a TGF-β1-shRNA plasmid could inhibit the fibrosis of renal allografts. The mechanism may be associated with the downregulation of Smad3 and upregulation of Smad7, resulting in suppressed epithelial-myofibroblast transdifferentiation and extracellular matrix synthesis。

Abstract：Background Immunosuppression for immunologically high-risk kidney transplant patients usually involves antithymocyte globulin induction with triple drug maintenance therapy. Alemtuzumab, a humanized anti-CD52 antibody,was expected to be a promising induction therapy agent for kidney transplantation. However, currently no consensus is available about its efficacy and safety. This study aimed to evaluate the efficacy and safety of alemtuzumab as immune induction therapy in highly sensitized kidney transplant recipients.Methods In this prospective, open-label, randomized, controlled trial, we enrolled 23 highly immunological risk patients (panel reactive antibody ＞20%). They were divided into two groups: alemtuzumab group (trial group) and anti-thymocyte globulin (ATG) group (control group). Patients in the alemtuzumab group received intravenous alemtuzumab (15 mg) as a single dose before reperfusion. At the 24th hour post-operation, another dosage of alemtuzumab (15 mg) was given.The control group received a bolus of rabbit ATG (9 mg/kg), which was given 2 hours before kidney transplantation and lasted until the removal of vascular clamps when the anastomoses were completed. Maintenance immunosuppression in both groups comprised standard triple therapy consisting of tacrolimus, prednisone, and mycophenolate mofetil (MMF).Acute rejection (AR) and infection episodes were recorded, and kidney function was monitored during a 2-year follow-up.χ2 test, t test and Kaplan-Meier analysis were performed with SPSS17.0 software.Results Median follow-up was 338 days. In both the alemtuzumab group and ATG group, creatinine and blood urea nitrogen values in surviving recipients were similar (P ＞0.05). White blood cell counts were significantly reduced in the alemtuzumab group for the most time points up to 6 months (P ＜0.05). One patient receiving alemtuzumab died for acute myocardial infarction at the 65th day post-operation. Two ATG patients died for severe pulmonary infection or cardiac and pulmonary failure. Cumulative 2-year graft survival rate was 90.9% in the alemtuzumab group and 81.8% in ATG group (P ＞0.05) respectively. There was one graft failure in the alemtuzumab group and two graft failures in ATG group, with all graft failures at tributed to rejection episodes. The alemtuzumab group had a 2-year cumulative freedom from rejection rate of 81.8%, compared with 72.7% for the ATG group (P ＞0.05).Conclusion Alemtuzumab induction therapy for highly sensitized kidney transplant recipients is an effective and safe protocol yielding an acceptable acute rejection rate。

Abstract：Background Sensitized recipients have a high risk of immunological graft loss due to hyperacute rejection and/or accelerated acute rejection. The presence of major histocompatibility complex class I-related chain A (MICA) antibodies has also been described associated with an increased rate of kidney-allograft rejection. The aim of this study was to describe the expression of MICA antibodies in sensitized recipients of renal transplantation and evaluate its influence on the kidney transplantation recipients.Methods A total of 29 sensitized recipients were included in this study. All patients received the MICA antibodies detection before and after protein A immunoadsorption. Panel reactive antibody (PRA), HLA-matches, acute rejection and postoperative one to four-week serum creatinine level were also collected and analyzed, respectively. No prisoners were used in this study.Results Eight patients (27.6%) in all 29 sensitized recipients expressed the MICA antibodies but did not show higher acute rejection rate than the non-expressed patients (3/8, 37.5% vs. 8/21, 38.1%; P=1.000). Recipients with PRA ＞40%showed higher expression levels of MICA antibodies than the recipients with PRA ＜40% (7/16, 43.8% vs. 1/13, 8.3%;P=0.044). HLA mismatch did not have any effect on the expression of MICA antibodies (P=1.000). MICA antibodies positive group had higher serum creatinine level than the control in postoperative one week ((135.4±21.4) μmol/L vs.(108.6±31.6) μmol/L, P=0.036), but no significant difference in postoperative four weeks ((89.0±17.1) μmol/L vs.(77.1±15.9) μmol/L, P=0.089). MICA antibodies decreased significantly after protein A immunoadsorption.Conclusions MICA antibodies increase in the sensitized recipients, which have significant effects on the function of allograft in early postoperative period. Protein A immunoadsorption can decrease MICA antibodies effectively in sensitized recipients。

Abstract：Background Invasive kidney biopsy is a priority diagnostic method for the acute rejection after renal transplantation for the past decades. However, no effective and noninvasive assay for predicting the severity of acute rejection is in wide use at present. This study was designed to investigate the predictive value of programmed death 1 (PD-1) mRNA for acute rejection after renal transplantation with real-time reverse transcriptase polymerase chain reaction (RT-PCR). A noninvasive diagnostic method has been expected to replace the tranditional kidney biopsy for the diagnosis of acute rejection and prediction of the outcome after kidney transplantation.Methods The whole blood samples from 19 subjects with acute rejection, 20 subjects with delayed graft function (DGF)and 21 subjects with stable recipients after kidney transplantation in a single kidney transplantation center between 2006 and 2009 were collected. The messenger RNA (mRNA) of PD-1 was analyzed with real-time RT-PCR. The associations of PD-1 mRNA levels with acute rejection and disease severity were investigated.Results The log-transformed ratio of PD-1 mRNA to GAPDH mRNA was higher in peripheral blood mononuclear cell (PBMC) from the group with acute rejection (4.52±1.1) than that from the group with DGF (1.12±0.6) or the group with normal biopsy results (0.7±0.4) (P ＜0.01, by the Kruskal-Wallis test). PD-1 mRNA levels were correlated with serum creatinine levels measured at the time of biopsy in the acute rejection group (Spearman's correlation coefficient, r=0.81,P=0.03), but not in the group with DGF or the group with normal biopsy results. PD-1 mRNA levels identified subjects at risk for graft failure within six months after the incident episode of acute rejection.Conclusions Our data suggest that PD-1 status may be a new predictor of acute rejection and the levels of PD-1mRNA in whole blood cells may positively correlate with the severity of acute rejection after renal transplantation.Meanwhile, the data provide the rational for interfering into the PD-1/PD-L1 as a novel therapy against the acute rejection after renal transplantation in clinical settings。

Abstract：Background Long-term use of steroid with large dosage might cause many adverse effects in kidney transplant patients; reducing steroid dosage to a low level for maintenance is helpful in avoiding the side-effects, but meanwhile,acute rejection may rise to be a main concern. The present research monitored the immune function changes and the incidence of acute rejection and infection after rapid steroid reduction to investigate the safety of this strategy.Methods A prospective trial was conducted, using tacrolimus and mycophenolate mofetil as the basic immunosuppressive regimen, in addition to antibody induction with basiliximab. Corticosteroid dosage was rapidly reduced to 10 mg/d seven days post-transplantation in the experimental group, and the standard corticosteroid therapy was employed in the control group. Patient immunity was monitored by the Immune Cell Function Assay pre- and two weeks post-transplantation. The incidence of acute rejection and infection were compared between the experimental and control group.Results Comparison of intracellular adenosine triphosphate (iATP) values detected two weeks post-transplantation for the control group ((324±45) ng/ml) and the experimental group ((345±91) ng/ml) did not reveal a significant difference (P＞0.05). The incidence of acute rejection was analogous between groups (P ＞0.05), while an increased incidence of infection was observed in the control group (53% (n=16)) versus the experimental group (22% (n=6), P ＜0.05). Overall,recipients in the control group had longer and more recurrent infections than those in the experimental group (P ＜0.05).Patients in the control group had a lower immune response ((235±35) ng/ml) than those in the experimental group ((286±16) ng/ml) when infection occurred (P ＜0.05).Conclusion Rapid reduction of steroid early after kidney transplantation does not lead to a significant rise in patient immunity. It is a safe and effective therapy for kidney transplant patients。

Abstract：Background Simultaneous pancreas-kidney transplantation (SPKT) is the best treatment option for diabetic patients with advanced chronic renal failure. The current study aimed to analyze the surgical indications, treatments and prognosis of SPKT.Methods We retrospectively analyzed 40 cases of SPKT performed between December 1999 and January 2010 in our center, including the survival rate, complications and the reasons of reoperation.Results Of all the 40 SPKT cases, the one-year survival rates of the recipients, kidney and pancreas transplant graft were 97.6%, 97.6% and 92.7%, while 97.6%, 91.1%, 92.7% at 3 years and 83.6%, 78.0%, 79.4% at 5 years, respectively.After SPKT, 10 patients need reoperation because of surgical complications (14 operations). The reoperation rate was 25%, including 2 patients (4 operations) with hematuria, 4 patients with abdominal hemorrhage, 2 patients (3 operations)with abdominal infection, 1 patient with pancreatic venous thrombosis, 1 patient with anastomotic leakage, and 1 patient with fistula.Conclusion Although SPKT provides a successful and effective treatment for diabetics with end-stage renal disease,how to reduce the complications of this treatment still need further effort。

Abstract：Background Staphylococcus aureus (S.aureus) remains as an important microbial pathogen resulting in community and nosocomial acquired infections with significant morbidity and mortality. Few reports for S. aureus in lower respiratory tract infections (LRTIs) have been documented. The aim of this study was to explore the molecular epidemiology of S.aureus in LRTIs in China.Methods A multicenter study of the molecular epidemiology of S. aureus in LRTIs was conducted in 21 hospitals in Beijing, Shanghai and twelve other provinces from November 2007 to February 2009. All the collected S. aureus strains were classified as minimum inhibitory concentration (MIC), mecA gene, virulence genes Panton-Valentine Leukocidin (PVL)and y-hemolysin (hlg), staphylococcal cassette chromosome mec (SCCmec) type, agr type, and Multilocus Sequence Typing (MLST).Results Totally, nine methicillin-sensitive S. aureus (MSSA) and 29 methicillin-resistant S. aureus (MRSA) strains were isolated after culture from a total of 2829 sputums or bronchoalveolar lavages. The majority of MRSA strains (22/29) had a MIC value of ≥512 μg/ml for cefoxitin. The mecA gene acting as the conservative gene was carried by all MRSA strains.PVL genes were detected in only one S. aureus strain (2.63%, 1/38). The hlg gene was detected in almost the all S.aureus (100% in MSSA and 96.56% in MRSA strains). About 75.86% of MRSA strains carried SCCmec Ⅲ. Agr type 1 was predominant (78.95%) among the identified three agr types (agr types 1,2, and 3). Totally, ten sequence type (ST) of S. aureus strains were detected. A new sequence type (ST1445) was found besides confirming ST239 as the major sequence type (60.53%). A dendrogram generated from our own MLST database showed all the bootstrap values≤50%.Conclusion Our preliminary epidemiology data show SCCmec Ⅲ, ST239 and agr type 1 of S. aureus as the predominant strains in LRTIs in Mainland of China。

Abstract：Background Factors of cell-mediated immunity and allergy together play their roles in the pathogenesis of pulmonary tuberculosis (PTB) and its prognosis. The purpose of this study was to investigate the computed tomographic demonstrations of HIV seropositive PTB and the relationship between its pathogenesis and CD4+ T-lymphocyte count.Methods The documented CT images of a total of 44 patients with HIV seropositive PTB, definitely diagnosed by etiological or pathological examinations, their clinical data and their CD4+ T-lymphocyte count were retrospectively reviewed.Results There were 15 cases of miliary tuberculosis, accounting for 34.1% of the total cases; 15 cases of nodular tuberculosis, 34.1%; 6 cases of ground-glass opacity, 13.6%; 5 cases of cord-liked fiber shadows, 11.4%; 16 cases of flaky and flocculating shadows, 36.4%; 5 cases of cavitation, 11.4%; 5 cases of tumor shadows, 11.4%; 2 cases of pleural thickening, 4.5% and 11 cases of pleural effusion, 25.0%; 1 case of calcification, 2.3%; 16 cases of lymphadenectasis,36.4%. The foci were located around the pulmonary hilum, anterior segment of superior lobe, basal segment of inferior lobe, medial lobe and lingual lobe. CD4+ T-lymphocyte count was closely related to the imaging demonstrations of HIV seropositive PTB.Conclusions CT scanning can demonstrate various signs of PTB. CD4+ T-lymphocyte level determines the variety of imaging demonstrations of HIV seropositive PTB and its prognosis。

Abstract：Background Bacterial infections remain a serious complication following coronary artery bypass grafting (CABG). The objective of the study was to determine the effectiveness of a guideline for the appropriate use of antibiotics in CABG during the perioperative period.Methods Six hundred and fourteen hospitalized patients who had undergone CABG from January to June 2006 were randomly allocated to an intervention group and a control group. The data on the hospital stay, days of antibiotic used,types of prophylactic antibiotics used, surgical wound infection and pulmonary infection and antibiotic costs for the patients were compared.Results The postoperative hospitalization days of the intervention group were significantly fewer than that for the control group (P＜0.05). The time of antibiotic use and post-infection treatment time were also significantly less in the intervention group than in the control group (P ＜0.05). The average hospital daily cost and total cost of antibiotics were less in the intervention group than in the control group (P＜0.05). Compared with the control group, prophylactic antibiotic use in the intervention group was more reasonable.Conclusions The guideline for the appropriate use of antibiotics in CABG during the perioperative period is effective strategies for reducing antibiotic costs, the time of antibiotic use and post-infection treatment time without compromising the patients' clinical outcome。

Abstract：Objective To review the role of polymorphism of major histocompatibility complex class I-related chain A (MICA) gene and antibodies against MICA antigens in transplant immunology.Data sources The data used in this review were mainly from our own results and from the relevant English language literatures published from 1999 to 2010. Some data presented in this review are in press.Study selection Articles regarding MICA gene discovery and pioneering finding of antibodies against MICA antigen and allograft rejection were selected. This review chronicles the development of our understanding of the role that MICA antigens and antibodies may play in organ transplantation.Results Polymorphic glycoprotein MICA antigens were detected on freshly isolated human umbilical cord endothelial cells, but not on peripheral lymphocytes. Antibodies were found and typing of recipients and donors by sequencing the MICA alleles has established that de novo antibodies produced in kidney transplant recipients are directed at mismatched MICA epitopes and are associated with acute rejection and chronic transplant failure. The specificity of antibodies against the epitopes of MICA antigens were well characterized by donor MICA typing, single antigen array testing with antibody absorption and elution. Acute graft-versus-host disease was observed in stem-cell recipients who were mismatched for MICA.Conclusions Immunization against mismatched MICA epitopes encountered in donor organs after transplantation may result in antibodies against MICA alleles. Testing for MICA donor-specific antibodies (DSA) which are associated with early failure of kidney transplants may be helpful for identifying some of the targets of antibodies against antigens other than the human leukocyte antigen (HLA) and for improving transplantation outcome。

Abstract：s from medical meetings related to transplant were also used.Study selection Articles were selected if they were trial results or case studies for the use of bortezomib in the sensitized patient population.Results The early data using bortezomib as a part of desensitization regimens has shown success. Although one cycle (4 doses) of bortezomib seems to have affect on many patients, it also seems likely that to provide complete desensitization multiple cycles will be required. Regarding safety, bortezomib has been shown to have minimal side effects. The most common side effects reported are those of thrombocytopenia and anemia. These side effects are dose related and self limiting upon discontinuation of the treatment.Conclusions Bortezomib with plasmapheresis is a promising new alternative to desensitization protocols that use either high dose intravenous immune globulin (IVIG) or Iow dose IVIG and plasmapheresis. The efficacy on antibody reduction looks to be batter that that of the IVIG based regimens without significant addition toxicity. The results of ongoing prospective trials are positive and their complete results are greatly anticipated。

Abstract：Background Women with a history of preeclampsia have twice the risk of cardiovascular diseases, and there is a graded relationship between the severity of preeclampsia and the risk of cardiac disease. Moreover, metabolic scores are associated with developing preeclampsia. However, since there are no diagnostic criteria for metabolic syndrome during pregnancy and pregnant women undergo metabolic changes, it is difficult to elucidate the relationship between preeclampsia and metabolic syndrome. We carried out a cross-sectional study to investigate the relationship between metabolic syndrome and preeclampsia among women with a history of severe preeclampsia shortly after an indexed pregnancy.Methods We recruited 62 women with a history of severe preeclampsia 1 to 3 years after an indexed pregnancy. Blood pressure and body compositional indices were recorded. Fasting blood samples were tested for glucose, total cholesterol,high density lipoprotein-cholesterol, low density lipoprotein-cholesterol, triglycerides, and insulin. A questionnaire was used to collect demographic data including pre-pregnancy weight and family history of diseases associated with cardiovascular diseases. Criteria for metabolic syndrome were defined by the National Cholesterol Education Program,Adult Treatment Panel Ⅲ 2001 (NCEP Ⅲ) and International Diabetes Federation 2005 (IDF). Data were analyzed by the α2 test and multivariate Logistic regression.Results According to NCEP Ⅲ and IDF standards, 17 (27%) and 24 (39%) women, respectively, were identified as having metabolic syndrome. Being overweight pre-pregnancy and currently overweight were risk factors, and currently overweight was an independent risk factor. A combination of blood pressure and waist circumference was predictive of metabolic syndrome with a sensitivity of 91.67% and specificity of 94.74%.Conclusions An unfavorable metabolic constitution in women may lead to metabolic syndrome, preeclampsia, and long-term cardiovascular disease. In women with severe preeclampsia, therapeutic interventions should include weight-control shortly after pregnancy, especially among women who were previously overweight。

Abstract：Background Sensitization in transplant candidates increases risk of irreversible immunologic injury of graft in the early period postoperatively. Elimination of anti-human leukocyte antigen (HLA) antibodies using protein A immunoadsorption (IA) might benefit these patients.Methods Protein A IA was used in 21 patients with high panel reactive antibody (PRA). The patients had IA 1-6 times (median 5 times) with the interval period was 2-5 days (median 2.5 days).Results Total 67 IA procedures were carried out smoothly in all patients, IA treatment reduced PRA I (pre (31.4+3.8)%vs. post (24.4±3.4)%, P ＜0.01) and II (pre (37.1±4.3)% vs. post (34.1+3.9)%, P ＜0.01). However, PRA did not change in some patients after the treatment. The serum immunoglobulin (IgG, IgM and IgA) and complement C3, C4 level were decreased significantly. Hemoglobin and albumin levels were slightly decreased associated with IA procedures. Flu-like symptoms were observed in a few of cases during the procedure but generally mild and transient.Conclusion Protein A IA is capable to efficiently remove serum immunoglobulin and complement, reduce HLA class I and class II PRA in high sensitized transplant candidates, which is likely to benefit the kidney transplantation in these patients。

Abstract：Congenital left ventricular diverticulum is a very rare cardiac abnormality and it is not completely understood about its etiology, clinical manifestation, diagnosis, treatment and prognosis. This article presents a case of large congenital diverticulum of the left ventricle. The clinical manifestation included paroxysmal supraventricular tachycardia. The diagnosis was made by chest fluoroscopy observation and confirmed by 64-slice CT-angiography. The arrhythmia alleviated instead of antiarrhythmic drugs but by radiofrequency catheter ablation. Due to the rapid growth of the diverticulum, the patient underwent surgical resection finally. Owing to the fatal risks, clinicians should improve the understanding of this disease by deeply studying more cases, in order to standardize the treatment。

Abstract：Transumbilical single-incision laparoscopic surgeries have attracted the attention of surgeon. Here we report a patient with multiple hepatic hemagiomas and symptomatic cholelithiasis who underwent laparoscopic left lateral hepatecomy and left hepatic hemangioma enucleation with single incision followed by cholecystectomy. The duration of the operation was 155 minutes and the blood loss was 100 ml. There were no complications during or after the treatment. This surgical treatment yields a good cosmetic effect and rapid recovery。

Abstract：Migration of metallic foreign body into the cerebral circulation is rarely seen. Most of the cases reported were due to gunshot wounds and shotgun wounds to the neck and face. When the foreign body is near the great vessel, it must be removed immediately or will cause complications. This study reported a case of delayed metallic foreign body embolus to the ophthalmic artery resulting from an injury to the right neck, which arose from the presence of metallic emboli to the cerebral circulation。

Abstract：A case of cutaneous Rosai-Dorfman disease (CRDD) presenting as a granulomatous rosacea-like rashs was reported. A 45-year-old Chinese woman presented with a 1-month history of a widespread nonpruiginous papulonodular eruption.The rash had begun on her face and rapidly progressed to involve the neck and extremities. She was otherwise healthy,with no history of fever, malaise, or weight loss. Physical examination revealed multiple symmetrically distributed discrete and coalescing red plaques, papules and nodules scattered over the face, neck and extremities. No appreciable lymphadenopathy or hepatosplenomegaly was noted. There was no mucosal involvement. The biopsy specimen obtained from the face demonstrated the epidermis was normal, while the superficial dermis contained sheets of histiocytes with abundant, focally foamy cytoplasm. The histiocytes were surrounded by a patchy lymphocytic and plasma cell infiltrate. There was no significant histiocytic atypia. Some of these histiocytes engulfed, without destroying,lymphocytes and neutrophils (emperipolesis). Immunohistochemical staining revealed that the histiocytes were strongly positive for S100 protein, weakly positive for CD68, and negative for CD1a. A diagnosis of CRDD was made. Oral prednisone therapy was initiated at a dosage of 30 mg/d for 3 weeks and then tapered over the ensuing 2 weeks. After 5weeks of treatment, the lesions had markedly improved。

Abstract：A case of hereditary benign telangiectasia without family history was reported. A 39-year-old woman presented with small and tiny telangiectases on the face, neck, upper trunk and forearms at birth. The numbers and sizes of the lesions increased gradually and she had no hemorrhagic diathesis and systemic diseases. No similar patients were found in her family. Upon physical examination, telangiectases were found on the face, neck, upper trunk and forearms; and a telangiectatic erythema was found on the right forearm 25 mm ×40 mm in size. Histopathology examination showed a normal epidermis and dilation of the capillaries at upper dermis. Hereditary benign telangiectasia without family history was diagnosed。

Abstract：Diffuse pulmonary lymphangiomatosis (DPL) is a rare disease that is characterized by diffuse proliferation of abnormal pulmonary lymphatic channels. DPL occurs mostly in children and young adults and often undergoes a progressive clinical course, eventually causing deterioration of the lung. Both the clinical diagnosis and treatment of DPL remain a challenge. Here, we report a case of DPL in a 53-year-old Chinese woman with comprehensive investigations including pulmonary function tests, computer tomography (CT), bronchoscopy and histological examination of the lung biopsy, and review the literature。