Clinical characteristics of plasma cell leukemia and progress in its diagnosis and treatment / 白血病·淋巴瘤

Plasma cell leukemia (PCL) is a plasma cell proliferative disorder with strong invasiveness, rapid progression and poor prognosis. The incidence of PCL is about (0.04-0.05)/100 000 per year. According to the multiple myeloma (MM) history, PCL can be divided into primary plasma cell leukemia (PPCL) and secondary plasma cell leukemia (SPCL). PPCL accounts for about 60% of PCL, and it is in the stage of leukemia at diagnosis and has no history of MM. SPCL accounts for the remaining 40% of PCL, and mostly shows as the MM end-stage manifestation, but also can be secondary to Waldenstrom macroglobulinemia, B-cell lymphoma, chronic lymphoblastic leukemia, amyloidosis, etc. Patients who progress from MM to SPCL account for 2%-4% of all MM patients. Due to the low incidence and strong clinical heterogeneity of PCL, the evidence-based medicine about PCL is relatively lacking, this article reviews the clinical characteristics of PCL and progress in its diagnosis and treatment.

Screening, preparation and identification of a broad-spectrum monoclonal antibody against Strepto-coccus pneumoniae surface protein A / 中华微生物学和免疫学杂志

Objective To screen and prepare a broad-spectrum monoclonal antibody against Strep-tococcus pneumoniae ( S. pneumoniae) surface protein A ( PspA) and to evaluate its potential in clinical prac-tice. Methods Hybridoma cells were screened and inoculated into the abdominal cavities of BALB/c mice to prepare antibodies in ascites. Monoclonal antibodies were obtained by ammonium sulfate precipitation and protein A affinity chromatography and then identified by SDS-PAGE and Western blot. Their specificity, iso-forms and killing activities in vitro were analyzed. Results A broad-spectrum monoclonal antibody that rec-ognized PspA subclasses 2, 3 and 4 was obtained. Its in vitro killing rate against S. pneumoniae reached 40. 3％. Conclusions A broad-spectrum monoclonal antibody that could specifically bind to PspA was suc-cessfully prepared with a strong in vitro killing activity. This study provided reference for clinical diagnosis of S. pneumoniae-related diseases, quality assessment of S. pneumoniae vaccines and further research on mono-clonal antibody therapeutics.

Perioperative platelet transfusion in infantile with Kasabach-Merritt phenomenon insensitive to glucocorticoids / 中华整形外科杂志

Objective@#To assess the safety and effectiveness of sufficient, short-term platelet (PLT) transfusion for the surgery preparation of the infantile patients with Kasabach-Merritt phenomenon, who were insensitive to glucocorticoids.@*Methods@#The infantile cases were retrospectively analyzed during May 2011 to December 2016, who were clinically diagnosed as KMP and insensitive to glucocorticoids, received PLT transfusion and surgical resection. PLT transfusion in patients whose PLTC was less than 30×109/L, was 0.3 therapeutic dose(TD)/kg, and 0.2 TD/kg in PLTC≥30×109/L group. The maximum was 1 TD.Criteria of the PLT transrusion: 1 hour after the transfusion, the PLT count (PLTC) were tested and the corrected count increment of platelet (CCI) and practical platelet recovery (PPR) was calculated. PLTC ≥100×109/L, CCI>7.5×109/L and PPR>30% were defined as effective; while PLTC=(50-99)×109/L, CCI>7.5×109/L and PPR>30% as partial effective; PLTC<50×109/L, or CCI≤7.5×109/L, or PPR ≤30% were defined as ineffective. By reviewing the method and response of their PLT infusions, to figure out the most effective way in rising PLT, as a part of pre-operation treatment.@*Results@#There were 46 cases in the research. Based on the PLTC, CCI and PPR 1 hour after PLT transfusion, there were 44 effective transfusion, 2 patients with partial effectiveness, and no ineffective case. There was no allergic or heart failure happened in any cases. No critical potential complications of PLT transfusion occurred, including fluid and iron overload, alloimmunization to human leukocyte antigen and/or PLT antigen.@*Conclusions@#Pre-operative sufficient and short-term PLT infusions are more effective than low dose and long-term ones. They can create a more optimistic opportunity for surgical resections.

Diffusion tensor imaging study on white matter structure in children with autism spectrum disorder / 中华行为医学与脑科学杂志

Objective To explore the white matter microstructural change between autism spectrum disorder(ASD) children and the healthy controls and it's correlation with the age and the clinical symptoms.Methods Thirty-three patients with ASD and thirty-three healthy controls (HC) matched for sex-,age-and handedness underwent diffusion tensor imaging (DTI) scans.Use the Autism Diagnostic Observation Schedule(ADOS) and the Neuropsychological Development Inventory for Children Aged 0-6 Years Old (SLAS) to assess the clinical symptoms.Use tract-based spatial statistics (TBSS) based on fiber skeleton to analyze the fractional anisotropy (FA) of the whole brain DTI then to identify the differentiated brain regions.And analyze the correlation with age and clinical symptoms.Results (1) FA value of the body of corpus callosum (BCC) (x =-10,y =11,z =26;P=0.0012),left posterior limb of internal capsule (LPLI) (x =-23,y =-11,z =12;P＜0.01),left anterior corona radiata (LACR) (x =-28,y =28,z =16;P＜ 0.01),lift superior corona radiata(LSCR) (x=-27,y=-1,z=21;P=0.0017) was significantly lower in ASD group.(2) There was a positive correlation between the FA value of LPLI and the LACR,LSCR with age among the ASD group(r=0.436,P=0.012;r=0.443,P=0.010;r=0.475,P=0.005).There was a negative correlation between the FA value of the BCC,LPLI and the LACR,LSCR with the age among the HC children.(3) There was a correlation between the LPLI with the score of A DOS (communication:r=0.406,P=0.025;communication+social behavior:r=0.377,P=0.039;restrictive interests and repetitive behaviors:r=0.375,P=0.041) and SLAS (gross motor:r=-0.409,P=0.024),There was a correlation between the LSCR with the score of the SLAS (gross motor:r=-0.539,P=0.002,adaptive capacity:r=-0.373,P=0.041,mental age:r=-0.388,P=0.034).Conclusion The brain white matter microstructure change of BCC,LPLI,LACR,LSCR may be the pathophysiological basis of ASD.The development trend of brain white matter structure varied with age in ASD children is different from that of normal children.Brain white matter microstructure of the LPLI,LSCR is correlation with ASD symptoms.

Diffusion tensor imaging study on white matter structure in children with autism spectrum disorder / 中华行为医学与脑科学杂志

Objective To explore the white matter microstructural change between autism spectrum disorder(ASD) children and the healthy controls and it's correlation with the age and the clinical symptoms.Methods Thirty-three patients with ASD and thirty-three healthy controls (HC) matched for sex-,age-and handedness underwent diffusion tensor imaging (DTI) scans.Use the Autism Diagnostic Observation Schedule(ADOS) and the Neuropsychological Development Inventory for Children Aged 0-6 Years Old (SLAS) to assess the clinical symptoms.Use tract-based spatial statistics (TBSS) based on fiber skeleton to analyze the fractional anisotropy (FA) of the whole brain DTI then to identify the differentiated brain regions.And analyze the correlation with age and clinical symptoms.Results (1) FA value of the body of corpus callosum (BCC) (x =-10,y =11,z =26;P=0.0012),left posterior limb of internal capsule (LPLI) (x =-23,y =-11,z =12;P＜0.01),left anterior corona radiata (LACR) (x =-28,y =28,z =16;P＜ 0.01),lift superior corona radiata(LSCR) (x=-27,y=-1,z=21;P=0.0017) was significantly lower in ASD group.(2) There was a positive correlation between the FA value of LPLI and the LACR,LSCR with age among the ASD group(r=0.436,P=0.012;r=0.443,P=0.010;r=0.475,P=0.005).There was a negative correlation between the FA value of the BCC,LPLI and the LACR,LSCR with the age among the HC children.(3) There was a correlation between the LPLI with the score of A DOS (communication:r=0.406,P=0.025;communication+social behavior:r=0.377,P=0.039;restrictive interests and repetitive behaviors:r=0.375,P=0.041) and SLAS (gross motor:r=-0.409,P=0.024),There was a correlation between the LSCR with the score of the SLAS (gross motor:r=-0.539,P=0.002,adaptive capacity:r=-0.373,P=0.041,mental age:r=-0.388,P=0.034).Conclusion The brain white matter microstructure change of BCC,LPLI,LACR,LSCR may be the pathophysiological basis of ASD.The development trend of brain white matter structure varied with age in ASD children is different from that of normal children.Brain white matter microstructure of the LPLI,LSCR is correlation with ASD symptoms.

Analysis of the pain in extremities caused by intramuscular venous malformation and surgical treatments / 中华整形外科杂志

Objective@#To analyze the pain caused by intramuscular venous malformation, so as to avoid misdiagnosis.@*Methods@#We retrospectively analyzed 173 patients who received surgical treatments in our department between Jan.2012 to Dec.2014, with the main complaint of local pain and were diagnosed as intramuscular venous malformation. The mechanisms of the local pain, based on the image data, intra-operative findings, pathology reports and the comparison of the Visual Analogue Scale(VAS) data before and after operation were summarized. The surgical interventions included simple excision or excision + adhesiolysis or excision + adhesiolysis + nerve decompression.@*Results@#The reasons of local pain can be divided into 4 categories: ①lesion located in the tendon insertions; ②lesion involving the local nerve, inducing the thickening and tensing of its epineurium and the increasing of its diameter; ③lesion infiltrated to the periosteum; ④phlebolith in it. All the 173 patients received surgical treatments and got varying degrees of relieving from local pain. 63 patients got a decrease of the VAS by 5 or more, and 95 cases′ VAS number decreased by 3-4, the rest 15 patients′ VAS cut down by 1-2.@*Conclusions@#Intramuscular venous malformation is an important reason for local pain and should not be neglected. Surgical treatment can be an effective method to remove the lesion and relieve local pain.

Effects of allogeneic compact bone derived-mesenchymal stem cell on proliferation,differentiation and chemokine receptor expression of T cells / 中国免疫学杂志

Objective:To observe the effects of allogeneic compact bone derived-mesenchymal stem cells ( CB-MSCs) on pro-liferation and differentiation of T cells,and investigate the molecular mechanisms of the immunosuppressive ability.Methods:With an established co-culture system of CB-MSCs and mouse spleen lymphocytes ( SP) in vitro,we observed the effects of CB-MSCs on prolif-eration,apoptosis and cell cycle of SP by MTS/PES assay and flow cytometry.Also,we measured the effects of CB-MSCs on regulatory T cells ( Treg) ratio and expressions of CCR5,CCR7 and CXCR3 in SP.Results:CB-MSCs could obviously inhibit the PHA-stimulated SP proliferation with a dose-dependent manner;MSCs could significantly inhibit the spontaneous apoptosis of SP and induce SP cell cycle G0/G1 phase arrest.After co-culture with SP,CB-MSCs could obviously increase the proportion of Treg in SP,down-regulate the expression of CXCR3 and CCR5,as well as up-regulate the expression of CCR7.Conclusion: Allogeneic CB-MSCs can significantly inhibit cell proliferation of SP,the mechanisms mainly involved the G0/G1 cell cycle arrest rather than apoptosis induction.In addition, CB-MSCs can exert immunomodulatory effects by increasing the Treg ratio,regulating the expressions of chemokine receptors.

[Application of radiofrequency ablation for tongue venous malformation].

OBJECTIVE: To explore the therapeutic effect of radiofrequency ablation for tongue venous malformation( VM). METHODS: From July 2013 to July 2014, 30 cases with tongue VM (local or diffuse) were retrospectively analyzed. 23 cases underwent radiofrequency ablation treatment. The radiofrequency electrode tip(0. 5 mm in diameter) was inserted into the lesion 1 mm below the bottom with 25 W in power and 15-30 s of pulse. The treatment was repeated when the electrode tip was removed back every 1 mm. Multi-point treatment was performed. RESULTS: 15 cases with unilateral VM were completely healed after one-stage radiofrequency ablation. 8 cases with bilateral VM received two-stage radiofrequency ablation with a 3-6 months of interval. Among the 8 cases, completely healing was achieved in 5 cases, partial VM residue happened in 3 cases due to its diffuse lesion and reservation of tongue function. 23 cases were followed up for 3 month to 1.5 years. Good cosmetic and functional results was achieved in 20 cases with no relapse. Partial VM residue was left in 3 cases. CONCLUSIONS: Radiofrequency ablation can effectively treat tongue VM with minimal morbidity and good cosmetic appearance. It also avoids the disadvantages of surgery.

Surgical treatment of hemangioma of limbs associated with Kasabach-Merritt phenomenon / 中华皮肤科杂志

Objective To analyze the surgical treatment of hemangioma of limbs complicated by Kasabach-Mcrritt phenomenon in children.Methods A retrospective study was carried out on 31 children with hemangioma of limbs complicated by Kasabach-Merritt phenomenon.Lower limbs were affected in 19 patients,and upper limbs in 12 patients.Twenty-six patients showed decreased platelet count ((2.6-60) × 109/L) and fibrinogen level (＜ 1 g/L),and 21 prolonged partial prothrombin time (＞ 50 seconds).All the patients who had responded poorly to medications including glucocorticoids received surgical treatment.Results Of these patients,30 were cured,and one died of massive blood loss.After surgical treatment,the platelet count returned to normal in 1-3 days in 25 patients,3-7 days in 4 patients,and more than 10 days in one patient.Conclusions For pediatric hemangiomas of limbs complicated by Kasabach-Merritt phenomenon,surgical treatment should be given as early as possible so as to reduce mortality in patients.

Problems in the treatment of acute promyelocytic leukemia / 白血病·淋巴瘤

Acute promyelocytic leukemia (APL), a subtype of acute myeloid leukemia, is characterized by rapid progress, prone to developing DIC, and high mortality. Typical chromosome translocation with PMLRARα fusion gene occurs in more than 95% of APL cases. Since 1986, the outcome of APL has been significantly improved in our country by firstly using ATRA and ATO for treating APL, making APL of AML curable by chemotherapy only. Based on our limited experiences, we discussed the related problems in the treatment of APL.

Chemotherapy strategies in adult refractory/relapsed acute lymphoblastic leukemia / 白血病·淋巴瘤

The treatment for adult refractory /relapsed acute lymphoblastic leukemia is a major challenge in clinical practice. Therapeutic strategies including high-dose single agent,intensified induction,new drugs,targeted therapy,and immunotherapy etc. may be of benefit to some patients. The post-remission treatments remain to be further developed.

Skp2 and malignandes / 白血病·淋巴瘤

S-phase kinase associated protein (Skp2) assumes the high expression in the most of malignant tumors, with its occurrence, and the development and the prognosis is closely related with a latent diagnose and treat went value. This review will focus on unique feature, biology activity, the function mechanism and with malignant tumor relations of the Skp2.

Is it chronic eosinophilic leukemia or myeloid/lymphoid neoplasm with eosinophilia? / 白血病·淋巴瘤

In 2008, WHO classified chronic eosinophilic leukemia with rearranged PDGFRA、PDGFRB or FGFRI as myeloid / lymphoid neoplasm with eosinophilia and PDGFRA、PDGFRB or FGFR1rearrangement and CEL without these abnormalities but with other abnormal clonality as CEL not otherwise specified (CEL-NOS). The article expresses authors' opinion.