ObjectiveTo analyze the characters of POEMS syndrome, raise physician awareness of diagnosis and therapy,and explore the optimal treatment strategies.MethodsThe clinical features,laboratory examination and therapy of 24 patients with POEMS syndrome were analyzed,and relative literatures were reviewed. ResultsA strong predominance of male over female was found, 18 vs 6. All patients were over 40 years old,with a mean age 56.5 years old,indicating a common adult involvement.All presented with polyneuropathy,which was also the most common complaints when admitted,which reminded neurologists of underlying possibility of a rare plasma cell disorder.Organomegaly was found,including 19 cases with hepatomegaly,17 patients with splenomegaly.Endocrinal abnormalities were also found in 15 cases.18 patients were M protein positive. Skin pigmentation was recognized in 21 cases. Melphalan in combination with prednisone was applied and 100 ％ response was observed. One of the patients received peripheral blood stem cell transplantation(PBSCT)2 years ago and a durable response was observed with a continuous complete remission of polyneuropathy and the absence of M protein following 2 years post PBSCT.ConclusionPOEMS syndrome is a rare multisystem disorder, the combination of symptoms and signs is highly complex,which is easy to misdiagnose or missed diagnose.A polyneuropathy with either organomegaly,endocrinal abnormalities, skin disorder or serositis is required a further investigation of clonal M protein and bone marrow.Melphalan and prednisone probable are the optimal regimens.PBSCT provides a new choice for therapy and research of POEMS syndrome.

AIM: To study the characteristics of new corneal biomechanical parameters in different degrees of myopia and analyze the correlation of the new parameter stress-strain index(SSI).METHODS: A cross-sectional study was conducted on 366 adult patients(718 eyes)with different degrees of myopia who received treatment at the First Affiliated Hospital of Dali University from October 2021 to November 2021, aged 18-50 years, and the spherical equivalent(SE)was -0.50～-16.75D. The axial length(AL)of the eye was measured by IOL master, and the new corneal biomechanical parameters, central corneal thickness(CCT)and intraocular pressure(IOP)were measured by corneal visualization Scheimpflug technology(Corvis ST). The subjects were categorized into low myopia, moderate myopia and high myopia groups according to SE. The data were analyzed by ANOVA and Pearson correlation.RESULTS: The ratio of the thinnest corneal thickness to horizontal thickness change rate(ARTh)and SSI were statistically significant(P<0.001), while the remaining parameters were not statistically significant(P>0.05). SSI was positively correlated with age(r=0.102, P=0.006), SE(r=0.361, P<0.001), IOP(r=0.175, P<0.001), CCT(r=0.098, P=0.009), SPA1(r=0.182, P<0.001), negatively correlated with AL(r=-0.331, P<0.001), IR(r=-0.545, P<0.001)and had no correlation with other corneal biomechanical parameters(P>0.05).CONCLUSION: With the increase of myopia degree and the elongation of the axial length, the SSI value becomes smaller and the corneal hardness decreases. SSI may be a helpful corneal biomechanical indicator for future research on myopia.

Objective: To investigate the gene expression characteristics of peripheral blood mononuclear cells from patients with high altitude pulmonary hypertension (HAPH) in Naxi residents living in Lijiang, Yunnan, and to explore the underlying pathogenesis and value for potential drug selection. Methods: This is a case-control study. Six patients with HPAH (HPAH group) and 4 normal subjects (control group) were selected from the Naxi residents who originally lived in Lijiang, Yunnan Province. The general clinical data of the two groups were collected, and the related indexes of pulmonary artery pressure were collected. Peripheral blood mononuclear cells of the subjects were collected for RNA sequencing. The differences on gene expression, regulatory network of transcription factors and drug similarity between the two groups were compared. The results were compared with the public data of idiopathic pulmonary arterial hypertension (IPAH). Biological processes and signal pathways were analyzed and compared between HPAH and IPAH patients. Results: The age of 6 patients with HAPH was (68.1±8.3) years old, and there were 2 males (2/6). The age of 4 subjects in the control group was (62.3±10.9) years old, and there were 2 males (2/4). Tricuspid regurgitation velocity, tricuspid pressure gradient and pulmonary systolic pressure in HAPH group were significantly higher than those in control group (all P<0.05). The results of RNA sequencing showed that compared with the control group, 174 genes were significantly upregulated and 169 genes were downregulated in peripheral blood mononuclear cells of HAPH group. These differentially expressed genes were associated with 220 biological processes, 52 molecular functions and 23 cell components. A total of 21 biological processes and 2 signal pathways differed between HPAH and IPAH groups, most of which were related to inflammation and immune response. ZNF384, SP1 and STAT3 were selected as highly correlated transcription factors by transcription factor prediction analysis. Trichostatin A and vorinostat were screened out as potential drugs for the treatment of HAPH by drug similarity analysis. Conclusions: There are significant differences in gene expression in peripheral blood monocytes between HAPH patients and normal population, and inflammation and immune dysfunction are the main pathogenic factors. Trichostatin A and Vorinostat are potential drugs for the treatment of HAPH.
Aged ; Altitude ; Altitude Sickness/genetics* ; Case-Control Studies ; China ; Familial Primary Pulmonary Hypertension/genetics* ; Humans ; Hydroxamic Acids/therapeutic use* ; Hypertension, Pulmonary/genetics* ; Inflammation ; Leukocytes, Mononuclear/pathology* ; Male ; Middle Aged ; Transcription Factors ; Transcriptome/genetics* ; Vorinostat/therapeutic use*

Objective To analyze the clinical manifestation of diffusive alveolar hemorrhage in acute leukemia induction therapy.Methods Clinical data of two diagnosed cases of diffusive alveolar hemorrhage secondary to acute leukemia were collected.Clinical data of eight cases of diffusive alveolar hemorrhage secondary to acute leukemia which were published were also collected by searching in Medline database.The clinical manifestation,diagnosis,strategy of differential diagnosis and treatment of diffusive alveolar hemorrhage secondary to acute leukemia were analyzed.Results Diffusive alveolar hemorrhage was a rare but fatal complication of acute leukemia.The common clinical manifestations included hemoptysis,progressive dyspnea and progressive decrease in concentration of hemoglobin.The analysis of blood gas showed type Ⅰ respiratory failure.The manifestations of chest computed tomography included diffusive ground glass opacity and infiltration of parenchyma.The bronchoalveolar lavage fluid was bloody.And lung biopsy showed congestion of alveoli and capillaritis.The detection for pathogens,vasculitis related antibodies,brain natrium peptide were negative.The mortality of those cases was 40 ％ (4/10).Corticosteroids therapy was effective.The mortality of patients received corticosteroids therapy was 25 ％ (2/8).Conclusion Diffusive alveolar hemorrhage is a rare but fatal complication of acute leukemia.The mortality is high.The key points of therapy are early diagnosis and corticosteroids therapy.

Objective To establish a real-time quantitative polymerase chain reaction (qPCR) assay for B-cell lymphoblastic leukemia according to individualized and specific immunoglobulin gene rearrangements in leukemia cells, and to use it for the monitoring of minimal residual disease (MRD) of B-cell lymphocytic leukemia. Methods The immunoglobulin gene rearrangements of bone marrow samples from 15 cases of B-cell lymphoblastic leukemia were analyzed with a validated European BIOMED-2 system, and the individualized and specific qPCR-based quantification of leukemic immunoglobulin gene rearrangements was established. Results Unique and specific gene rearrangements of immunoglobulin light and heavy chains were identified in 14 cases and Ig-qPCR based on these gene rearrangements had a sensitivity of 10-5 and high specificity which met the international criteria in 10 patients. Leukemia MRD quantification with immunoglobulin gene rearrangement-based qPCR was similar as compared with other MRD detection methods. Conclusion Immunoglobulin gene rearrangement-based leukemia MRD quantification is feasible, sensitive, specific, precise and much valuable for clinical decision of treatments in B-cell lymphoblastic leukemia.

Objective To evaluate the efficacy and safety of HLA identical and haploidentical related allogeneic hematopoietic stem cell trans?plantation in the treatment of acute myeloid leukemia(AML)and myelodysplastic syndrome(MDS). Methods A total of 21 patients with AML and 8 patients with MDS who underwent allogeneic hematopoietic stem cell transplantation in our hospital from April 2011 to April 2016 were ana?lyzed retrospectively,including 16 cases of HLA?identical allogeneic HSCT,10 cases of haploidentical allogeneic HSCT,and 3 cases of syngeneic HSCT. BUCY2 or TBI plus CY ± chemotherapeutic agents was the regular conditioning regimen. No graft versus host disease(GVHD)prophylax?is was required for syngeneic HSCT,but cyclosporine in combination with methotraxate was essential for allogeneic HSCT,cyclosporine,methotrax?ate,antithymocyte globulin,mycophenolate mofetil and glucosteroids for haploidentical HSCT. Results All patients achieved fully donor?originat?ed hematopoiesis. Two patients died of severe acute GVHD within 100 days post HSCT. Acute GVHD with gradeⅡ?Ⅳoccurred in 23.1%(6/26) patients,chronic GVHD in 50%patients,therapy and relapse?relevant mortality was 4/29(13.8%)and 6/29(20.7%)cases within a median follow?up of 23(1?60)months,respectively. Two?year overall survival and leukemia free survival rates are 68.09%( 95%CI:45.77%?82.78%)and 60.22%(95%CI:38.19%?76.55%),respectively. High risk AML is still the main challenge to long?term leukemia free survival. Conclusion HLA identical and haploidentical allogeneic HSCT for AML and MDS is safe ,effective and feasible. Minimal residual disease monitoring and pre?ventative as well as preemptive intervention is necessary for improving prognosis of high risk AML.

Topical compound lidocaine cream is commonly used for pain relief before laser treatment for scar. In January, 2020, one 4-year-old girl with large area scar was admitted to the Department of Plastic Surgery, Taizhou Hospital of Zhejiang Province. Severe adverse drug reaction such as convulsions, cyanosis of the lips, loss of consciousness, and urination incontinence occurred, after 1.5 hours of topical application of compound lidocaine cream before laser treatment. She was recoved after emergency treatment.

Eyelid and surrounding tissue injury caused by trauma, burn, infection and other reasons will lead to different degrees of eyelid ectropion and incomplete eyelid closure after scar formation. At present, surgical treatment is the main treatment for cicatricial ectropion, but there is still the risk of scar formation again after operation. We used early ion beam drug introduction to treat 1 case of cicatricial ectropion after trauma, and achieved satisfactory result.

Topical compound lidocaine cream is commonly used for pain relief before laser treatment for scar. In January, 2020, one 4-year-old girl with large area scar was admitted to the Department of Plastic Surgery, Taizhou Hospital of Zhejiang Province. Severe adverse drug reaction such as convulsions, cyanosis of the lips, loss of consciousness, and urination incontinence occurred, after 1.5 hours of topical application of compound lidocaine cream before laser treatment. She was recoved after emergency treatment.

Eyelid and surrounding tissue injury caused by trauma, burn, infection and other reasons will lead to different degrees of eyelid ectropion and incomplete eyelid closure after scar formation. At present, surgical treatment is the main treatment for cicatricial ectropion, but there is still the risk of scar formation again after operation. We used early ion beam drug introduction to treat 1 case of cicatricial ectropion after trauma, and achieved satisfactory result.