Objective:To investigate the application and feasibility of the night-vision puncture technique in performing peripherally inserted central catheter(PICC).Methods:Seventy patients were randomly divided into ultrasound guided puncture group or modified blind puncture group,35 cases in each group.The puncture success rate,the achievement ratio of catheterization,the puncture site,arm circumference,catheterization time and complications were recorded in two groups.Results:Two groups had no significant difference in the puncture success rate,the achievement ratio of catheterization,the puncture site,arm circumference,phlebitis incidence,subcutaneous congestion (bleeding) (P ＞ 0.05).The catheterization time in modified blind puncture group was shorter than the ultrasound guided puncture group (P ＜ 0.001).In modified blind puncture group,a negative correlation (correlation coefficient:-0.475,P =0.004)between arm circumference and the puncture success rate was found.Conclusion:The puncture success rate of the modified blind puncture technique in performing peripherally inserted central catheter (PICC) is high and close to the puncture success rate of the PICC under ultrasound.Additionally,the modified blind puncture technique does not increase the incidence of complications and delay the catheter time.

Objective: To evaluate the clinical characteristics, diagnosis criteria, treatment and prognosis in patients with Bing-Neel Syndrome (BNS) . Methods: The clinical characteristics, lab data, treatment and outcomes of 3 Bing-Neel syndrome patients diagnosed at Peking Union Medical College Hospital were collected. Results: The clinical presentation was heterogeneous without any specific common signs or symptoms. One patient was diagnosed with BNS 42 months after diagnosis of Waldenström macroglobulinemia (WM) by cerebrospinal fluid (CSF) cytology and flow cytometry, but dead of infection during the first course of chemotherapy. BNS was the first manifestation of WM in the other 2 cases. They were diagnosed by flow cytometry and cytology of CSF. The detection of MYD88^L265P mutation in CSF contributed to diagnosis and to sequential monitoring of minimal residual disease. They received systemic chemotherapy of FC (fludarabine + cyclophosphamide) ± rituximab and intrathecal therapy, followed by maintenance therapy of chlorambucil or R2 (rituximab + lenalidomide) . They were followed 17 and 20 months respectively without progression of disease. Conclusion The diagnosis approach of BNS should be based on a combination of CSF cytology, flow cytometry and detection of the MYD88^L265P mutation. The detection of MYD88^L265P mutation may be useful in the monitoring of minimal residual disease.

Objective: To evaluate the impact of KIT D816 mutation on the salvage therapy in relapsed acute myeloid leukemia (AML) with t(8;21) translocation. Method: The characteristics of the first relapsed AML with t(8;21) translocation from 10 hospitals were retrospectively collected, complete remission (CR₂) rate after one course salvage chemotherapy and the relationship between KIT mutation and CR₂ rate was analyzed. Results: 68 cases were enrolled in this study, and 30 cases (44.1%) achieved CR₂. All patients received KIT mutation detection, and KIT D816 mutation was identified in 26 cases. The KIT D816 positive group had significantly lower CR₂ compared with non-KIT D816 group (23.1% vs 57.1%, χ²=7.559, P=0.006), and patients with longer CR₁ duration achieved significantly higher CR₂ than those with CR₁ duration less than 12 months (74.1% vs 31.9%, χ²=9.192, P=0.002). KIT D816 mutation was tightly related to shorter CR₁ duration. No significant difference of 2 years post relapse survival was observed between KIT D816 mutation and non-KIT D816 mutation group. Conclusion KIT D816 mutation at diagnosis was an adverse factor on the salvage therapy in relapsed AML with t(8;21) translocation, significantly related to shorter CR1 duration, and can be used for prediction of salvage therapy response. KIT D816 mutation could guide the decision-making of salvage therapy in relapsed AML with t(8;21) translocation.

Objective: To evaluate the clinical characteristics, MYD88^L265P mutation, CXCR4^WHIM mutation and prognosis in patients with Waldenström macroglobulinemia (WM). Methods: The clinical characteristics, International Prognostic Scoring System for symptomatic WM (WPSS) , and overall survival (OS) were retrospectively assayed in 93 patients with newly diagnosed WM at Peking Union Medical College Hospital during January 2000 to August 2016. The MYD88^L265P mutation and CXCR4^WHIM mutation were tested among 34 patients. Results: The median age of the 93 patients was 64 years (range, 33-85 years) with a male-to-female ratio of 2.44. According to WPSS, we included 16 (17.2%) low-risk, 44 (47.3%) intermediate-risk and 33 (35.5%) high-risk patients. Eight patients had secondary amyloidosis. With a median follow-up of 44 (1-201) months, the median OS was 84 months. Cox regression multifactor analysis showed WPSS risk group (HR=2.342, 95% CI 1.111-4.950, P=0.025) , whether patients had secondary amyloidosis (HR=5.538, 95% CI 1.958-15.662, P=0.001) and whether patients received new drugs (HR=3.392, 95% CI 1.531-7.513, P=0.003) were independent factors associated with OS. We have investigated the presence of the MYD88^L265P and CXCR4^WHIM mutation in 34 patients and found that MYD88^L265P mutation was occurred in 32 patients (94.1%) and CXCR4^WHIM mutation was occurred in 8 patients (23.5%). Seven of 8 patients who harbored CXCR4^WHIM-mutated also exhibited the MYD88^L265P mutation. Patients with MYD88^L265PCXCR4^WHIM vs MYD88^L265PCXCR4^WT presented with more severe anemia, lower platelet level, higher M protein level and more hyper-viscosity syndrome. Conclusion WPSS risk group, whether patients had secondary amyloidosis or received new drugs are independent factors for OS in WM. MYD88^L265P and CXCR4^WHIM mutation, the most common somatic variants in WM, often occur together and impact the clinical presentation.

Objective: To evaluate the response of oral melphalan plus high-dose dexamethasone (MDex) for patients with primary light chain amyloidosis (pAL). Methods: Clinical data, hematological and organ responses, and survival of 76 patients with pAL who had received MDex from January 2009 to July 2017 were retrospectively analyzed. Results: Of 76 patients (47 males and 29 females with the median age of 56 [range, 20-74] years old), 19.70% patients were defined as Mayo 2004 stage 3, involvement of more than or two organs was presented in 65 (85.53%) patients. Among 60 response evaluable patients, overall hematological response was 48.33% with complete response of 20.00% and very good partial response of 20.00%, respectively. The median time to the hematological response was 5 (range, 1-15) months. 36.67% patients achieved organ response. After the median follow up of 23(range, 1-113) months for surviving patients, median progression-free survival (PFS) and overall survival (OS) were 34 and 43 months, respectively. In a three months landmark analysis, the median rates of PFS and OS were 46 and 65 months, respectively. The median OS rates of patients with Mayo 2004 stage 3 and non Mayo 2004 stage 3 were 5 and 65 months (P=0.001), respectively. Conclusions MDex was an effective treatment for patients with early stage pAL, but was not suitable for those with severe cardiac involvement.

Objective:To demonstrate the type of CEBPA gene mutations among patients with acute myeloid leukemia (AML), clinical characteristics, and prognostic effect on patient outcomes.Methods:Demographic data, clinical features, laboratory characteristics, and data about treatment and follow-up of 57 patients with CEBPA mutated AML diagnosed at Peking Union Medical College Hospital between April 2016 and November 2022 were collected and analyzed.Results:In total, 57 patients with CEBPA mutation accounted for 16.1% of all the 353 patients with AML, among which 28 patients had CEBPA-bZIPinf and 29 had CEBPA-other. Compared with the CEBPA-other group, the CEBPA-bZIPinf group was younger (54 vs 64 years, P=0.010), de novo AML was more common ( P=0.001), and the level of bone marrow blast was higher (68.0% vs 36.3%, P=0.001). Moreover, 24 patients from the CEBPA-bZIPinf group and 19 from the CEBPA-other group received chemotherapy. The one-course complete remission (CR) rate of the CEBPA-bZIPinf group was significantly higher than that of the CEBPA-other (87.5% vs 47.4%, P=0.010) and CEBPA-wt (87.5% vs 50.3%, P=0.002) groups. After a median follow-up of 11 months, the median OS of the CEBPA-bZIPinf group was significantly longer than that of the CEBPA-wt group (not reached vs 22.1 months, P=0.012) . Conclusion:CEBPA-bZIPinf mutated AML is a unique clinical entity, with a younger age of diagnosis, better response to chemotherapy, and better prognosis.