2018年 · 第131卷第15期

Hemophagocytic lymphohistiocytosis (HLH) is a severe life-threatening clinical syndrome with hyperinflammation rather than an isolated disease. It is classified as primary HLH and secondary HLH. Primary HLH is associated with HLH-related gene mutations, whereas secondary HLH occurs after severe infections, tumors, connective tissue diseases, etc.^[1]Regardless of primary or secondary HLH, 30- 73% of patients have central nervous system (CNS) involvement.^[2]Among adults with HLH in China, 38.4% of patients have neurological symptoms at the onset, and there are up to 89.6% with neurological symptoms during the entire course of the disease.^[3]However, it has not been clearly reported about the CNS involvement in children with HLH.

Prospective real-life data on the safety and effectiveness of rituximab in Chinese patients with diffuse large B-cell lymphoma (DLBCL) or follicular lymphoma (FL) are limited. This real-world study aimed to evaluate long-term safety and effectiveness outcomes of rituximab plus chemotherapy (R-chemo) as first-line treatment in Chinese patients with DLBCL or FL. Hepatitis B virus (HBV) reactivation management was also investigated.

A prospective, multicenter, single-arm, noninterventional study of previously untreated CD20-positive DLBCL or FL patients receiving first-line R-chemo treatment at 24 centers in China was conducted between January 17, 2011 and October 31, 2016. Enrolled patients underwent safety and effectiveness assessments after the last rituximab dose and were followed up for 3 years. Effectiveness endpoints included progression-free survival (PFS) and overall survival (OS). Safety endpoints were adverse events (AEs), serious AEs, drug-related AEs, and AEs of special interest. We also reported data on the incidence of HBV reactivation.

In total, 283 previously untreated CD20-positive DLBCL and 31 FL patients from 24 centers were enrolled. Three-year PFS was 59% (95% confidence interval [CI]: 50-67%) for DLBCL patients and 46% (95% CI: 20- 69%) for FL patients. For DLBCL patients, multivariate analyses showed that PFS was not associated with international prognostic index, tumor maximum diameter, HBV infection status, or number of rituximab treatment cycles, and OS was only associated with age >60 years (P < 0.05). R-chemo was well tolerated. The incidence of HBV reactivation in hepatitis B surface antigen (HBsAg)-positive and HBsAg-negative/hepatitis B core antibody-positive patients was 13% (3/24) and 4% (3/69), respectively.

R-chemo is effective and safe in real-world clinical practice as first-line treatment for DLBCL and FL in China, and that HBV reactivation during R-chemo is manageable with preventive measures and treatment.

ClinicalTrials.gov, NCT01340443; https://clinicaltrials.gov/ct2/show/NCT01340443.

After the first examination of patients with lymphoma diagnosis, important laboratory tests such as complete blood count; albumin, kidney and liver function tests; uric acid; β2-microglobulin; C-reactive protein (CRP); erythrocyte sedimentation rate (ESR); and lactate dehydrogenase (LDH) examinations are recommended. In this study, our aim was to find the relationship between laboratory parameters and the maximum standard uptake value (SUV_max) of positron emission tomography/computed tomography (PET/CT) in patients with lymphoma at the diagnosis and after treatment.

Thirty-four lymphoma patients treated at Mustafa Kemal University Internal Medicine Clinic between 2014 and 2017 were included in this retrospective study. Results of CRP, ESR, LDH, albumin, and white blood cell (WBC) count were recorded before each PET scan test, and each parameter was analyzed for correlation with SUV_max measurements.

Spearman’s correlation test showed that the after-treatment SUV_max values were significantly correlated with the after-treatment LDH, ESR, and CRP values (for LDH, ESR, and CRP, R²: 0.453, 0.426, and 0.351; P= 0.007, 0.012, and 0.042, respectively). On the other hand, albumin and WBC count did not show a significant correlation with the after-treatment SUV_max values (all P> 0.05).

CRP, ESR, and LDH values may also be good predictors in patients for whom PET/CT imaging cannot be performed.

Although much attention has been paid to the pharmacokinetics (PKs) of different factor VIII (FVIII) concentrates in persons with hemophilia A (HA), limited information is available in young boys with severe HA. In this study, we aimed to assess the PK parameters of FVIII products in boys with severe HA in China.

A total of 36 boys (plasma-derived [pd]-FVIII, n = 15; recombinant [r] FVIII, n = 21) were enrolled between January 2015 and May 2016 in Beijing Children’s Hospital. PK characteristics of FVIII products were studied according to a reduced 4-sampling time point design (1 h, 9 h, 24 h, and 48 h postinfusion).

The mean FVIII half-life (t_1/2) was 10.99 ± 3 .45 h (range 5.52- 20.02 h), the mean in vivo recovery (IVR) was 2.01 ± 0.42 IU/dl per IU/kg (range 1.24–3.02 IU/dl per IU/kg) and mean clearance (CL) of FVIII is 4.34 ± 1.58 ml·kg⁻¹·h⁻¹ (range 2.29–7.90 ml·kg⁻¹·h⁻¹). We also analyzed the influence of several parameters that potentially modulate FVIII PK. The age was closely associated with FVIII half-life (R² = 0.32, P< 0.01). The t_1/2 of FVIII increased by 0.59 h per year. Besides age, von Willebrand factor antigen (VWF:Ag) also was associated with FVIII half-life (R² = 0.52, P< 0.01). Patients with blood Group O had a shorter FVIII half-life than patients with non-O blood group (9.40 ± 0.68 h vs. 12.3 ± 0.79 h, t = 2.70, P= 0.01). The FVIII IVR correlated with age (R² = 0.21, P< 0.01) and VWF:Ag level (R² = 0.28, P< 0.01). CL rates were faster in young patients and in those with low-VWF:Ag levels. CL rates of FVIII are higher in blood Group O versus non-blood Group O persons (5.02 ± 0.38 vs. 4.00 ± 0.32 ml·kg⁻¹·h⁻¹, t= 2.53, P= 0.02).

Chinese boys with severe HA have similar PK values to other ethnic groups and large differences in FVIII PK between individual patients. Age, blood group, and VWF:Ag levels are important determining factors for FVIII CL.

Central nervous system (CNS) involvement is found in many patients with hemophagocytic lymphohistiocytosis (HLH). In this study, we mainly analyzed neurological symptoms, imaging findings, cerebrospinal fluid (CSF), and their relationship with outcomes of HLH children.

Related data of 179 Chinese pediatric patients with HLH admitted to our center from January 2010 to December 2015 were analyzed retrospectively. Diagnosis and treatment were based on the HLH-2004 protocol. Two-tailed Chi-squared test was used to compare between different groups, and Kaplan-Meier survival curves were used to analyze the overall survival (OS) of patients with HLH.

In the present study, 21.2% (38/179) of total patients had neurological symptoms including seizure, irritability, somnolence, and unconsciousness. There were 80 (50.0%, excluding 19 patients without imaging data) patients with cranial imaging abnormalities. There were 14.7% (17/116, excluding 63 patients who did not accept lumbar puncture) of patients with abnormal CSF results. CNS involvement is defined as abnormalities in one or more of CNS symptoms, radiological findings, and CSF. Thus, 60.3% of them had CNS involvement. As for the prognosis, the median follow-up time was 3.2 years (17 lost to follow-up). The probable 3-year OS of children was higher without CNS involvement (86.0% ± 4.6%) than those with CNS involvement (68.9% ± 4.9%, hazard ratio [HR] = 2.286, P= 0.019). Among them, the probable 3-year OS of children without CNS symptoms was 76.0% ± 3.8%, higher than with CNS symptoms (59.5% ± 8.1 %, HR= 2.147, P= 0.047). The 3-year OS of children with abnormal CSF was 64.7% ± 11.6%, compared with normal CSF (85.1% ± 3.7%, HR= 0.255, P= 0.038).

HLH patients with CNS involvement might have worse outcomes compared with those without CNS involvement, and CNS symptoms and CSF changes are more important to access the prognosis than imaging abnormality.

Pulmonary Langerhans cell histiocytosis (PLCH) is an interstitial primary pulmonary disease, characterized by Langerhans cell proliferation. It is easily misdiagnosed in children. This study aimed to characterize the clinical manifestations and features of PLCH by retrospective analysis.

A retrospective analysis was performed in 117 PLCH patients out of 338 LCH patients who were admitted in our center from November 2006 to October 2013. Variables between two groups were compared by Mann-Whitney U-test and Chi-square test. Kaplan-Meier curves were constructed to compare the survival rates and Cox regression to evaluate the effect of risk factors.

The median age of PLCH group was significantly lower than that of non-PLCH group (18.63 months vs. 43.4 months, P< 0.001). All PLCH children had other organ involvement and only 11 cases (9.4%) had respiratory symptoms. The most common radiologic finding was cystic lesions (29 cases, 24.8%). Pulmonary function abnormalities were dominated by obstructive ventilatory dysfunction (63 cases, 82.9%). The 5-year overall survival (OS) of PLCH children was 93.6% ± 2.3% and the event-free survival (EFS) was 55.7% ± 5.2%. Among the 38 cases with progressed or relapsed disease, five cases (13.2%) were due to progression or recurrence of lung damage. The 5-year OS of PLCH children with "risk organ" involvement was significantly lower than those without "risk organ" involvement (86.0% ± 4.9% vs. 100%, χ²= 8.793, P= 0.003). The difference of EFS between two groups was also significant (43.7% ± 7.7% vs. 66.3% ± 6.5%, χ² = 5.399, P= 0.020). The "risk organ" involvement had a significant impact on survival (hazard ratio = 1.9, P= 0.039).

PLCH mainly occurs in young children, and only a small percentage of patients have respiratory symptoms. They generally have other organ involvement. Most of PLCH children have a good prognosis and most lung lesions could have improved or stabilized. Management of "risk organ" involvement is the key point to improving EFS.

It is known that short sleep duration adversely affects children’s behavior and physical development. This study aimed to investigate the status of sleep duration in 3–14-year-old children in Beijing and explore the related factors of sleep loss with them.

In this study, a cross-sectional study of random stratified cluster sampling was conducted on 3–14-year-old children and adolescents in Beijing. According to the proportion of children in each district and school, the final cohort included a total of 11 kindergartens, 7 primary schools, and 8 junior high schools from 7 districts of Beijing. Children of sampled classes were included, and their parents were invited to fill a series of questionnaires including the simplified Chinese version of Pediatric Sleep Questionnaire, Sleep Questionnaire Scale, and Hong Kong-Children Sleep Questionnaire about the performance of the last 6 months.

Out of the total 11,420 questionnaires, 9198 questionnaires were valid and effective with the response rate of 80.54%. The age of the investigated children was 8.8 ± 3.8 years, including 4736 males and 4462 females. The daily sleep duration of children in Beijing was 9.7 ± 0.7 h. The prevalence of sleep loss (<9 h/day) of children in Beijing was 11.8%. The daily sleep duration of children aged <6, 6 ≤ age <11, and ≥11 years was 9.7 ± 0.6 h, 9.6 ± 0.6 h, and 9.5 ± 0.8 h, respectively. The sleep duration reduced significantly in children aged ≥11 years as compared to younger children in Beijing which was mainly contributed by the variation tendency of sleep duration on weekdays. The multivariate logistic regression analysis identified factors associated with sleep loss (P < 0.05): male (odds ratio [OR] = 1.32, 95% confidence interval [CI ]: 1.15–1.51), age ≥11 years (OR = 2.37, 95% CI: 1.92- 2.93), overweight (OR = 1.34, 95% CI: 1.17–1.54), family history of snoring (OR = 1.35, 95% CI: 1.13–1.61) and activities before bedtime with watching TV (OR = 1.24, 95% CI: 1.08–1.43), sports (OR= 1.22, 95% CI: 1.01–1.48), playing cellphone (OR = 1.91, 95% CI: 1.31- 2.73) and surfing the Internet (OR = 1.27, 95% CI: 1.06–1.52) and among them age ≥11 years and playing cellphone before bedtime had greater impact on children’s short sleep duration than that of other factors.

Sleep loss was common among 3–14-year-old children in Beijing. Sleep duration decreased with age, especially among children over 11 years old. Factors associated with sleep loss covered sociodemographic characteristics, family sleep habits and routine activities before bedtime, and among those variables, age ≥11 years and playing with cellphones before bedtime had a greater impact on sleep duration, indicating that existing sleep loss in 3–14-year-old children could be, at least partly, improved by paying more attention to children aged of 11 years or entering Grade 5 and Grade 6 and to children with a family history of snoring; by reducing the use of electronic products before bedtime, especially cellphones; by managing weight and keeping fit; and by improving the bedtime routine.

The 47,XYY syndrome could result in fertility problems. However, seldom studies reported comprehensive researches on the embryonic development and pregnancy outcomes of these patients. This study aimed to evaluate the clinical outcomes of nonmosaic 47,XYY patients performed with fluorescent in situ hybridization (FISH) and preimplantation genetic diagnosis (PGD) treatment.

This was a retrospective study. Between January 2012 and May 2017, 51 infertile males with nonmosaic 47,XYY syndrome underwent FISH-PGD were included in the study. According to sex chromosomal FISH results, embryos were classified as normal signal, no nuclei fixed, no signal in fixed nuclei, suspensive signal, and abnormal signal groups, respectively. The incidence of each group, the fixation rate, and hybridization rate were calculated. Embryonic development and pregnancy outcomes were also analyzed. The measurement data were analyzed with Student’s t-test. The comparison of categorical data was analyzed with the Chi-square test and Fisher’s exact test when expected cell count was <5.

The 53 PGD cycles with 433 embryos were analyzed. The fixation rate was 89.6%, while the hybridization rate was 96.4%. There were 283 embryos with two sex chromosomal signals with clear diagnosis (65.4%). The numbers of no nuclei fixed, no signal in fixed nuclei, suspensive signal, and abnormal signal groups were 45 (10.4%), 14 (3.2%), 24 (5.5%), and 67 (15.5%), respectively. Embryos with abnormal signals were abandoned. The number of good-quality embryos was 210 (57.4%), including implanted embryos on day 4/day 5 and cryopreserved. The rates of good-quality embryos in the no nuclei fixed (22.2%), no signal in fixed nuclei (28.6%), and suspensive signal groups (33.3%) were comparable (P > 0.05), and were significantly lower than the normal signal group (66.4%, P< 0.001). The clinical pregnancy rates of fresh and frozen embryos transferred cycles were 70.6% and 85.7%, respectively.

Among embryos with a clear diagnosis of sex chromosome, about one-fifth showed abnormal signals. Embryos with two sex chromosomal signals are more likely to develop into good-quality ones. The application of the PGD by FISH may help to improve the clinical outcomes.

Cytokines play an important role in occurrence and recovery of hepatitis B virus (HBV) infection. The aim of this study was to investigate the changes of cytokines concentration and its correlation to alanine aminotransferase (ALT), HBV deoxyribonucleic acid (HBV-DNA), hepatitis B envelope antigen (HBeAg), and HBV surface antigen (HBsAg) in the development of chronic hepatitis B (CHB).

Thirteen healthy individuals (HI), 30 chronic HBV-infected patients in immune tolerant (IT) phase, and 55 CHB patients were enrolled between August 2015 and May 2017. The peripheral blood samples were collected from all individuals. The levels of interferon (IFN)-α2, interleukin (IL)-10, transforming growth factor (TGF)-β1, HBV-DNA, HBsAg, and HBeAg and liver function were measured. The quantitative determinations of cytokines levels, including IFN-α2, IL-10, and TGF-β1 were performed using Luminex multiplex technology. The correlation of cytokines to ALT, HBV-DNA, HBsAg, and HBeAg was analyzed by linear regression analysis.

IFN-α2 levels were similar between HI and IT groups (15.35 [5.70, 67.65] pg/ml vs. 15.24 [4.07, 30.73] pg/ml, Z= −0.610, P= 0.542), while it elevated significantly in CHB group (35.29 [15.94, 70.15] pg/ml vs. 15.24 [4.07, 30.73] pg/ml; Z= −2.522, P= 0.012). Compared with HI group (3.73 [2.98, 11.92] pg/ml), IL-10 concentrations in IT group (5.02 [2.98, 10.11] pg/ml), and CHB group (7.48 [3.10, 18.00] pg/ml) slightly increased (χ² = 2.015, P= 0.365), and there was no significant difference between IT and CHB group (Z= −1.419, P= 0.156). The TGF-β1 levels among HI (3.59 ± 0.20 pg/ml), IT (3.62 ± 0.55 pg/ml), and CHB groups (3.64 ± 0.30 pg/ml) were similar (χ² = 2.739, P= 0.254). In all chronic HBV-infected patients (including patients in IT and CHB groups), the elevation of IFN-α2 level was significantly associated with ALT level (β = 0.389, t = 2.423, P= 0.018), and was also negatively correlated to HBV-DNA load (β= −0.358, t= −2.308, P= 0.024), HBsAg (β= −0.359, t= −2.288, P= 0.025), and HBeAg contents (β= −0.355, t= −2.258, P= 0.027). However, when both ALT level and cytokines were included as independent variable, HBV-DNA load, HBsAg, and HBeAg contents were only correlated to ALT level (β= −0.459, t= −4.225, P= 0.000; β= −0.616, t= −6.334, P= 0.000; and β= −0.290, t= −2.433, P= 0.018; respectively).

IFN-α2 elevation was associated with ALT level in patients with chronic HBV infection. However, in CHB patients, only ALT level was correlated to HBV-DNA, HBsAg and HBeAg contents.

Currently, spectral-domain optical coherence tomography (SD-OCT) appears to be a new type of glaucoma diagnostic tool. Thus, this study aimed to evaluate the effectiveness of glaucoma diagnostic parameters from SD-OCT of patients with different severities of myopia.

This was a cross-sectional study. A total of 248 participants (248 eyes) were enrolled, including 51 cases in the early primary open-angle glaucoma group, 79 cases in the control group (0.50 D to −0.50 D, excluding −0.50 D), 47 cases in the low-myopic group (−0.50 to −3.00 D, excluding −3.00 D), 43 cases in the moderate-myopic group (−3.00 to −6.00 D, excluding −6.00 D), and 28 cases in the high-myopic group (≤−6.00 D). All participants were examined using the Humphrey visual field test and SD-OCT. The SD-OCT parameters of the retinal nerve fiber layer (RNFL) and ganglion cell complex were analyzed statistically using the receiver operating characteristic curve and area under the curve (AUC).

The AUC showed that the best parameters for the control and low-myopic groups were the inferior and inferior temporal RNFL thicknesses (AUC >0.94), respectively; for the moderate- and high-myopic groups, the best parameter was the temporal low RNFL thickness (AUC, 0.926 and 0.896, respectively). The AUC of the inferior parameters of the moderate-myopic group (0.864) was lower, ranked 15^th among all RNFL parameters. When the sensitivity was fixed at 85%, the specificity of the inferior, superior, inferior temporal, and superior temporal quadrants was higher (>80%) in the control and low-myopic groups, while they were lower (20–60%) for the moderate- and high-myopia groups. The green color based on the OCT database was also less for the high-myopic group compared with that of other groups (P < 0.05).

Glaucoma diagnostic parameters from SD-OCT were not clinically effective for the moderate- and high-myopic groups. The specificities were low. The moderate- and high-myopic groups require comprehensive analyses for the diagnoses of glaucoma. The SD-OCT database should be improved to better indicate the level of myopia based on the corresponding diopter readings.

Technical aspects of the correct placement of medial support locking screws in the locking plate for proximal humerus fractures remain incompletely understood. This study was to evaluate the clinical relationship between the number of medial support screws and the maintenance of fracture reduction after locked plating of proximal humerus fractures.

We retrospectively evaluated 181 patients who had been surgically treated for proximal humeral fractures (PHFs) with a locking plate between September 2007 and June 2013. All cases were then subdivided into one of four groups as follows: 75 patients in the medial cortical support (MCS) group, 26 patients in the medial multiscrew support (MMSS) group, 29 patients in the medial single screw support (MSSS) group, and 51 patients in the no medial support (NMS) group. Clinical and radiographic evaluations included the Constant-Murley score (CM), visual analogue scale (VAS), complications, and revision surgeries. The neck-shaft angle (NSA) was measured in a true anteroposterior radiograph immediately postoperation and at final follow-up. One-way analysis of variance or Kruskal-Wallis test was used for statistical analysis of measurement data, and Chi-square test or Fisher’s exact test was used for categorical data.

The mean postoperative NSAs were 133.46°± 6.01°, 132.39°± 7.77°, 135.17°± 10.15°, and 132.41°± 7.16° in the MCS, MMSS, MSSS, and NMS groups, respectively, and no significant differences were found (F = 1.02, P= 0.387). In the final follow-up, the NSAs were 132.79°± 6.02°, 130.19°± 9.25°, 131.28°± 12.85°, and 127.35°± 8.50° in the MCS, MMSS, MSSS, and NMS groups, respectively (F = 4.40, P= 0.008). There were marked differences in the NSA at the final follow-up between the MCS and NMS groups (P = 0.004). The median (interquartile range [IQR]) NSA losses were 0.0° (0.0–1.0)°, 1.3° (0.0–3.1)°, 1.5° (1.0–5.2)°, and 4.0°(1.2–7.1)° in the MCS, MMSS, MSSS, and NMS groups, respectively (H = 60.66, P< 0.001). There were marked differences in NSA loss between the MCS and the other three groups (MCS vs. MMSS, Z = 3.16, P= 0.002; MCS vs. MSSS, Z = 4.78, P< 0.001; and MCS vs. NMS, Z = 7.34, P< 0.001). There was also significantly less NSA loss observed in the MMSS group compared to the NMS group (Z= −3.16, P= 0.002). However, there were no significant differences between the MMSS and MSSS groups (Z= −1.65, P= 0.225) or the MSSS and NMS groups (Z= −1.21, P= 0.099). The average CM scores were 81.35 ± 9.79, 78.04 ± 8.97, 72.76 ± 10.98, and 67.33 ± 12.31 points in the MCS, MMSS, MSSS, and NMS groups, respectively (F = 18.68, P< 0.001). The rates of excellent and good CM scores were 86.67%, 80.77%, 65.52%, and 43.14% in the MCS, MMSS, MSSS, and NMS groups, respectively (χ² = 29.25, P< 0.001). The median (IQR) VAS scores were 1 (0- 2), 1 (0- 2), 2 (1–3), and 3 (1–5) points in the MCS, MMSS, MSSS, and NMS groups, respectively (H = 27.80, P< 0.001). Functional recovery was markedly better and VAS values were lower in the MCS and MMSS groups (for CM scores: MCS vs. MSSS, P< 0.001; MCS vs. NMS, P< 0.001; MMSS vs. MSSS, P= 0.031; and MMSS vs. NMS, P< 0.001 and for VAS values: MCS vs. MSSS, Z = 3.31, P= 0.001; MCS vs. NMS, Z = 4.64, P< 0.001; MMSS vs. MSSS, Z= −2.09, P= 0.037; and MMSS vs. NMS, Z= −3.16, P= 0.003).

Medial support screws might help enhance mechanical stability and maintain fracture reduction when used to treat PHFs with medial metaphyseal comminution or malreduction.

Estrogen is one of the most important reproductive steroidal hormones and plays a critical role in the maintenance of pregnancy, and its function is mediated by estrogen receptor 1(ESR1 ). The polymorphisms of ESR1 were involved in recurrent spontaneous abortion (RSA); however, the association between ESR1 polymorphisms and RSA remains controversial. The present meta-analysis was aimed to clarify the association between ESR1 PvuII (-397C/T, rs2234693) and XbaI (-351A/G, rs9340799) polymorphisms and the risk of RSA.

All the included articles were retrieved from PubMed, Embase, Cochrane Library, China National Knowledge Infrastructure, and Wanfang Med Online Database up to January 3, 2018. Data were processed in the Stata 12.0 software. The odds ratios (ORs) and 95% confidence intervals (95% CIs) were calculated using fixed-effects models (FEM)/random-effects models (REM).

Seven case- control studies with 836 cases and 1164 controls were included in the study. Generally, the ESR1 polymorphisms were not associated with RSA in any of the genetic analysis models. However, it was found that as rs9340799 polymorphism was related to increased risk of RSA in non-Asian group in the homozygous genetic model (OR = 2.40, 95% CI = 1.05–5.50, P= 0.039). Moreover, in Asian group, rs9340799 polymorphism was found to be related to decreased RSA risk in both the heterozygous model (OR = 0.53, 95% CI = 0.33- 0.85, P= 0.009) and the dominant genetic model (OR = 0.55, 95% CI = 0.30- 0.98, P= 0.042).

Generally, there was no significant association between the polymorphisms of ESR1 and the risk of RSA. However, subgroup analysis indicated that ESR1 rs9340799 polymorphism was related to increased RSA risk in the non-Asian group while associated with decreased RSA risk in Asian group.

To the Editor: Plasmacytoid dendritic cells (pDCs) represent a subset of dendritic cells. There are two distinct forms of neoplasms derived from pDCs; the one is blastic plasmacytoid dendritic cell neoplasm (BPDCN) and the other is mature pDCs proliferation coexisted with myeloid tumors. The mature pDCs in myeloid tumor are characterized by a lineage-negative human leukocyte antigen (HLA)-DR⁺CD56⁻CD123⁺CD11c⁻immunophenotype. Here, we describe an 83-year-old man who developed acute myeloid leukemia (AML) transferred from primary myelofibrosis (PMF) coexisted with monoclonal gammopathy of undetermined significance (MGUS) and mature pDCs proliferation; however, the immunophenotype results of bone marrow (BM) showed positive CD56 expression .

To the Editor: Castleman’s disease (CD) describes a group of lymphoproliferative disorders with heterogeneous clinical manifestations.^[1]CD comprises at least two distinct diseases (unicentric CD [UCD] and multicentric CD [MCD]) with respective clinical features and prognoses: UCD often involves one lymph node area with favorable outcome; MCD is a systemic disease with relatively poor prognosis which involves multiple lymph node areas with constitutional symptoms (e.g. fever, night sweats, and weakness) and organ dysfunction. According to pathogenesis, MCD can be further divided into different subtypes based on the status of human herpes virus-8 (HHV-8) infection. Of the HHV-8 negative MCD (also named as idiopathic MCD [iMCD] ), there is a peculiar subtype named TAFRO syndrome which constitutes a constellation of unique clinical manifestations (T: Thrombocytopenia, A: Anasarca, F: myelofibrosis, R: Renal dysfunction, and O: Organomegaly). This syndrome, first reported in Japan,^[2]has been reported mostly in the Japanese population and occasionally in Caucasians. There is no such case reported in China. We herein report the first patient with TAFRO syndrome in China with confirmed myelofibrosis.

To the Editor: Excessive weight loss usually contributes to apparent abdominal contour deformity. Abdominoplasty is the optimal solution for wall laxity, excess skin, and diastasis of the rectus abdominis.^[1]Reverse abdominoplasty, a partial procedure, plays an irreplaceable role in correcting upper epigastric skin laxity and is usually performed after prior liposuction or conventional abdominoplasty.^[2]In cases with apparent skin laxity, residual contour deformity of the upper abdominal wall also remains.

To the Editor: According to the Chinese National Bureau of Statistics in October 1999, the population aged ≥60 years reached 10% of the total population, indicating that China was entering into an aging society. By the end of 2017, the population aged ≥60 years was 240,900,000, accounting for 17.3% of the total population. With the rapidly aging population, biliary diseases in elderly patients have become frequent in China, with a morbidity rate of 8–11%. Due to lowered stress response, defense ability, and immunity, biliary diseases in elderly patients are characterized by an increase in coexisting diseases, rapid progression, poor surgical tolerance, high surgical risk, frequent postoperative complications, and high mortality. Thus, it is important to explore effective treatment methods in elderly patients with biliary diseases. Based on our clinical experience in the treatment of elderly patients with biliary diseases,^[1]along with previous studies, this report presented the current status of surgical treatment of elderly patients with biliary diseases in China.^[2]

To the Editor: Percutaneous nephrolithotomy (PCNL) is a surgical procedure commonly used to treat large kidney stones. It is usually accompanied by severe pain and discomfort postoperatively. Intravenous opioids and local anesthetic infiltration have been conventionally used to control postoperative pain. In addition, regional blocks, for example, the paravertebral block and intercostal block, have been attempted. However, the efficacy of these blocks for analgesia has not been established yet.^[1]Erector spinae plane block (ESPB) reportedly provides effective analgesia after thoracic and abdominal surgery.^[2,3]We describe a patient in whom continuous ESPB was used to provide highly effective analgesia after PCNL. This study was approved by our hospital’s institutional review board. A 67-year-old man (weight 61 kg and height 171 cm) was admitted to undergo PCNL for a right kidney stone. He had controlled hypertension and diabetes mellitus, and his laboratory test values were unremarkable. PCNL was performed under general anesthesia in the lateral position.

To the Editor: Pseudohypoaldosteronism Type II (PHAII), also known as Gordon syndrome, is a rare autosomal disease, caused by mutations inWNK1,WNK4,CUL3, orKLHL3genes. Hitherto, about 200 individuals and families have been reported with PHAII; of these, 26 were caused byCUL3mutation.^[1,2]In pediatrics, hyperkalemia-complicated hypertension is rare, and PHAII should be considered in this condition.

To the Editor: A 43-year-old man diagnosed with dilated cardiomyopathy 6 years prior visited our institution recently because of worsened chest congestion with shortness of breath and abdominal distension. The patient had a history of diabetes. His electrocardiography (ECG) demonstrated a sinus rhythm with an intraventricular conduction disturbance, an atypical left bundle branch block, and a QRS interval of 160 ms. Echocardiographic findings were consistent with dilated cardiomyopathy [bicentric type,Figure 1a], in which the left atrial diameter was 44 mm, the left ventricular end-diastolic diameter was 78 mm, and the left ventricular ejection fraction was 30%. The final diagnosis was New York Heart Association Grade III dilated cardiomyopathy. To achieve a good prognosis, improve exercise tolerance, and prevent sudden death, cardiac resynchronization therapy (CRT) with defibrillator implantation was advised. However, coronary sinus (CS) abnormalities were observed during the implantation operation. Therefore, it was forced to stop the operation and a cardiac computed tomography angiography (CTA) evaluation of the abnormalities was arranged.

To the Editor: Despite the numerous morphological and biomechanical studies on the anterior cruciate ligament (ACL), this anatomical structure is still under investigation; in particular, there is a discussion about its anatomical composition in relation to biomechanics. At present, in general, it is assumed that ACL comprises a structure of two bundles: anteromedial and posterolateral. Sometimes, even three bundles are distinguished; however, the presumption that ACL is an integral and continuous structure is constantly mentioned in the literature. It is known that ACL is not isometric, and the length of ACL fibers varies along with knee flexion and extension: in flexion, the length of anteromedial bundle increases (tightens), while posterolateral bundle decreases (slackens). At the same time, during knee flexion and extension, femoral part of ACL rotates, while its tibial part moves minutely.^[1]It might be considered that the foreign body embedded at the distal part of ACL should have less potency for migration.

To the Editor: Hydroxychloroquine (HCQ) is commonly used in systemic lupus erythematosus (SLE), rheumatoid arthritis, and other autoimmune disorders. We report a case of psoriasis-form erythroderma induced by HCQ in an SLE patient. Thus, rheumatologists and dermatologists should be aware of the possibility of HCQ-induced psoriasis-form lesions.

To the Editor: Drug-induced pancreatitis (DIP) is a rare cause. According to a German retrospective study, the incidence of pancreatitis caused by drugs was 1.4%.^[1]A nationwide survey conducted by Japan showed that 1.2% of all cases of acute pancreatitis were caused by drugs.^[2]The DIP is caused due to the drug itself or its metabolites, or organism-specific reactions caused by the introduction of hypersensitivity during pancreatic injury. Despite appropriate treatment, it demonstrated severe complications and high mortality.^[3]Valproate (sodium valproate and divalproex sodium forms) is a commonly used medication that is approved by the U.S. Food and Drug Administration for the treatment of epilepsy, migraine, and bipolar disorders. Adverse effects associated with valproate are typically benign, but there are more serious effects that are less frequently observed. These effects include hepatotoxicity, teratogenicity, possible polycystic ovaries with a potential sterile effect, and acute pancreatitis.^[4]Even though acute pancreatitis is an adverse effect of very low frequency, it is very important to be noted due to a high mortality rate of patients with acute pancreatitis as a consequence of the use of valproate. A 26-year-old Chinese man was diagnosed with mania and received outpatient care at another mental hospital, and his positive symptoms included hallucinations and delusions. His condition has been well controlled by quetiapine. In June 2016, he met with a car accident, leading to multiple bone fractures in his foot, and he was given azithromycin for treating foot infection. After few days of medication, the patient had severe abdominal pain and diarrhea and so the antibiotics and quetiapine were suspended. Three days after the withdrawal, the mania condition relapsed, and then he was admitted in our hospital. The patient had a medical history of being never smoked, no alcohol intake, and no intake of any illegal drugs. After admission, the blood and urine routine examinations, hepatic and renal functions tests were performed and showed normal results. Electroencephalograms, electrocardiograms, and abdominal B ultrasonography showed normal results. Then, he had been taking quetiapine fumarate tablets (Hunan Dongting Pharmaceutical Co., Ltd.,) 400 mg/d and magnesium valproate sustained-release tablets (Hunan Xiangzhong Pharmaceutical Co., Ltd.,) 1000 mg/d. Six days after treatment, he developed abdominal tenderness, abdominal swelling, and vomitings. The beginning of the vomit included the stomach content, which was gradually turned into a dark green liquid. Blood tests indicated acute inflammation with a leukocytosis (white blood cell count 13.99; normal range 4–11 × 10⁹/L) and neutrophilia (neutrophil count 12.72 and normal range 2.5–7.5 × 10⁹/L). Amylase showed elevated levels at 368, which was about 3.0 times the normal value (normal range <122 U/L). Creatine kinase was within the normal range. Blood pressure was stable and within the normal range. Elevated serum amylase levels combined with severe abdominal pain often triggered the initial diagnosis of acute pancreatitis. Although results of the abdominal ultrasonography were negative, the results of digital radiography were positive for acute pancreatitis. A "sentinel loop" as a sign of intestinal obstruction and colonic truncation sign appeared in the plain abdominal X-ray film [Figure 1]. He was managed conservatively with intravenous fluid 2500 ml/d and moxifloxacin 400 mg/d, but the general condition of the patient was not improved. A chief psychiatrist has been consulted for this case. Factors such as alcohol use, obstruction, and trauma were excluded from the study. The possibility of drug-induced acute pancreatitis was considered. Through literature review, it was found that valproate was highly correlated with pancreatitis, and hence was withdrawn from his drug regimen. The patient continued treatment with fluid 2500 ml/d and moxifloxacin 400 mg/d, and he showed a substantial recovery within 1 week. Although rare, acute pancreatitis has been reported with the long-term use of valproate. The clinical features of acute pancreatitis are obvious but subtle clinical features made the diagnosis difficult in our patient. One may argue that the incrimination of valproate as an etiological cause of pancreatitis is not justifiable without reintroducing the drug to see the recurrence of pancreatitis. This was not done as we considered it to be unethical.

In the article titled, "Effect of Dandelion Extracts on the Proliferation of Ovarian Granulosa Cells and Expression of Hormone Receptors" , published on pages 1694-1701, Issue 14, Vol 131 ofChinese Medical Journal,^[1]the financial disclosure section is missing. It should be written as following: