[Clinical Analysis of Patients with Blastic Plasmacytoid Dendritic Cell Neoplasm].

OBJECTIVE: To explore the clinical characteristics, treatment, and prognosis of patients with blastic plasmacytoid dendritic cell neoplasm(BPDCN). METHODS: Clinical data of 5 patients diagnosed with BPDCN in Wuhan First Hospital and Wuhan Tongji Hospital from June 2016 to November 2021 were retrospectively analyzed. RESULTS: Among the 5 patients, 3 were male and 2 were female, with a median age of 28(10-52) years old. Four patients showed obvious skin damage at the initial diagnosis; the other one showed clinical manifestations of acute leukemia rather than obvious skin damage at the initial diagnosis, but infiltrated skin when the disease relapsed after treatment. Other infiltration sites of lesions included bone marrow (2/5), peripheral blood (2/5), lymph nodes (3/5), liver and spleen (2/5). All patients had no clinical manifestation of central nervous system infiltration. Tumor cell specific immune markers CD4, CD56, CD123 were all positive, and the median Ki-67 index was 70%. TET2, ASXL1 and NRAS gene mutations were found respectively in 3 patients by next-generation sequencing technique (NGS). ALL-like, AML-like and invasive NK/T cell lymphoma-like first-line induction chemotherapy regimens were used for the patients. One patient died of severe complications during the early stage of chemotherapy, 3 patients were evaluated as CR, and 1 patient was evaluated as PR. 2 patients were recurred and progressed after induction of chemotherapy, and one of them was evaluated as CR after re-treatment. One patient received autologous hematopoietic stem cell transplantation (auto-HSCT) and got long-term survival (OS 87 months). 3 patients received allogeneic hematopoietic stem cell transplantation (allo-HSCT), of which one died of transplantation related complications, and 2 cases survived. The median follow-up time of 4 patients with evaluable efficacy was 28.5(9-84) months, the median OS time was 31.5(10-87) months. CONCLUSION: BPDCN is a highly heterogeneous malignant tumor with a poor prognosis. HSCT, especially allo-HSCT can significantly improve the prognosis of BPDCN patients.

Hemoporfin-mediated photodynamic therapy with general anesthesia showed superior efficacy in the treatment of port-wine stains: a retrospective evaluation.

Background: Hemoporfin-mediated photodynamic therapy (PDT) is an effective treatment for port-wine stains (PWS), and pain is the main adverse effect of this therapy. General anesthesia is commonly used for pain management during PDT, but the effect of general anesthetics on the subsequent treatment efficacy of PDT in PWS has not been reported. Objectives: To assess the use of general anesthesia combined with PDT compared with PDT alone in 207 PWS patients, and to provide further safety and efficacy data on this combined therapy. Methods: Propensity score matching (PSM) was used at a 2:1 ratio to create a general anesthetic group (n = 138) and a highly comparable nonanesthetic group (n = 69). The clinical outcomes were evaluated, and the treatment reactions and adverse effects were recorded after one treatment with PDT. Results: After matching, there was no significant difference in the demographic data of the patients in the two groups (p > 0.05), while the treatment efficacy was significantly higher in the general anesthetic group than in the nonanesthetic group (76.81 vs. 56.52%, p < 0.05). Moreover, logistic regression analysis confirmed that patients receiving general anesthesia showed an association with a good response to PDT (OR = 3.06; 95% CI, 1.57-6.00; p = 0.0011). Purpura lasted longer in the general anesthetic group, but the other treatment reactions and adverse effects were similar in the two groups (p > 0.05). No serious systemic adverse reactions were observed. Conclusion: We recommend this combined therapy, which is associated with painless, as a high efficacy treatment option for PWS patients, especially for patients with a poor response to multiple PDT alone treatments.

Whole-Genome Sequencing Identified KCNJ12 and SLC25A5 Mutations in Port-Wine Stains.

Port-wine stains (PWSs) are a congenital capillary malformed disorder and are caused by a number of somatic mutations that disrupt vascular development. However, the underlying genetic mutations in the pathogenesis of PWS have not yet been fully elucidated. To understand PWS genetic variations and investigate novel genetic mutations, we extracted genomic DNA from four sporadic PWS patients and then performed whole-genome sequencing (WGS). Using Sorting Intolerant from Tolerant (SIFT), PolyPhen2, Mutation Assessor, MetaSVM to identify candidate genetic mutations and whole-exome sequencing (WES) to confirm the identified variants. We found a previously reported G protein subunit alpha q (GNAQ) mutation c.548G > A, p.Arg183Gln in one case, whereas no such mutation was found in the other three samples. Moreover, six novel somatic mutations in three genes, including KCNJ12, SLC25A5, POTEE, were found in these four samples. Importantly, WES also verified the KCNJ12 (c.433G > A, p.Gly145Ser) and SLC25A5 (c.413G > A, p.Arg138His) mutations in other five sporadic PWS patients, with the frequency of 60% (3 of 5) and 40% (2 of 5), respectively. Thus, we reveal in this study two novel somatic mutations, KCNJ12 and SLC25A5, in the sporadic PWS patients for the first time. These findings highlight the genetic polymorphism of PWS and provide potential clinical prediction targets for this disease.

Counterintuitive Lanthanide Hydrolysis-Induced Assembly Mechanism.

The understanding of the hydrolysis mechanism of lanthanide ions is limited by their elusive coordination configuration and undeveloped technology. A potential solution by high-resolution mass spectroscopy studies is hindered by the lack of a stable model under electrospray ionization (ESI) conditions and the complexity of the spectra. Herein, it is demonstrated that diketonate ligands can efficiently stabilize the hydrolyzed intermediate cluster of Ln3+ under ESI conditions, and an effective mass difference fingerprint of isomorphism (MDFI) method is proposed, which can allow the determination of the nuclearity-number of the species without depth resolution. Thus, the hydrolysis of Ln3+ into an atomically precise hydroxide cluster is observed at the level of precise formulae. The species evolution upon hydrolysis is along the dominant path of {Eu3}-{Eu4}-{Eu9}-{Eu10}-{Eu11}-{Eu15}-{Eu16} and a nondominant path of {Eu3}-{Eu4}-{Eu8-1}-{Eu8-2} under the investigated conditions. The crystal of the {Eu16} species was obtained via low-temperature crystallization, and single-crystal X-ray diffraction studies show that its structure contains three octahedral {o-Ln6} units. The contradiction between multiple {o-Ln6} units in the structure and the absence in the formation process indicates that the repetitive subunit observed in the structure does not necessarily correspond to the construction units of high-nuclearity clusters. Photophysical measurements indicate that Eu16 cluster has a high total emission quantum efficacy of 12.8% in the solid state. This study provides fundamental insights into the formation, evolution, and assembly of small lanthanide hydroxide units upon hydrolysis, which is vital for the goal of directional synthesis of lanthanide hydroxide clusters.

Doped Luminescent Lanthanide Coordination Polymers Exhibiting both White-Light Emission and Thermal Sensitivity.

Multifunctional lanthanide coordination polymers (CPs) have the advantages of acting in two or more fields simultaneously. Herein, two single lanthanide CPs, formulated as LnL(D/L-Hlac)(H2O)2·0.5H2O (Ln = Eu (1), Tb (2); H2L = 4,4'-(pyridine-3,5-diyl)dibenzoic acid) and their doped lanthanide analogue Tb0.9373Eu0.0627L(D/L-Hlac)(H2O)2·0.5H2O (3) were prepared through hydrothermal methods. Luminescence measurements reveal that 1 displays red photoluminescence and its Commission International ed'Eclairage (CIE) coordinates are almost invariant in the temperature range from 80 to 300 K, while the emission color of 2 changes from yellow to green and its CIE coordinates change from (0.36132, 0.56365) at 80 K to (0.30448, 0.45566) at 300 K. Significantly, 3 not only displays white-light emission with CIE coordinates of (0.32999, 0.33406) but also exhibits a thermal sensitivity of 2.27% K-1 at 230-300 K. The obviously larger thermal sensitivity in 3 in comparison to that of 1.07% K-1 for 2 demonstrates that lanthanide CPs with both a heat-sensitive fluorescent thermometer and high-efficiency white-light emission can be expected by doping Eu(III) ions into Tb(III)-based CPs.

The Effect on the Luminescent Properties in Lanthanide-Titanium OXO Clusters.

Two lanthanide-titanium oxo clusters (LTOCs), formulated as [Eu3Ti3(µ3-O)2(µ3-OH)(CH3O)2(Ac)2(CH3OH)(tbba)12]·CH3OH (1) and [Eu6Ti8(µ3-O)13(µ2-OH)(µ3-OCH3)2(µ2-OCH3)2(H2O)(CH3OH)2(tbba)19]·Htbba·10H2O (2), were synthesized through the solvothermal reaction of 4-tert-butylbenzoate ligand, Eu(Ac)3·3H2O, and Ti(OiPr)4 in methanol. Single-crystal structural analysis reveals that 1 crystallized in the orthorhombic space group Pnn2, and 2 crystallized in the triclinic space group P1. Structurally, the core of 1 can be viewed as a coplanar unit of [Eu3Ti3(µ3-O)2(µ3-OH)]16+ formed through each µ3-O2- and µ3-OH- bridging one Ti4+ and two Eu3+ ions, while that of 2 can be viewed as two units of [Eu3Ti3(µ3-O)3(µ2-O)4(µ2-OCH3)2(CH3OH)]5+ and [Eu3Ti5(µ3-O)6(µ2-OH)(µ3-OCH3)2(H2O)(CH3OH)]14+ connected through four µ2-O units from the [Eu3Ti3(µ3-O)3(µ2-O)4(µ2-OCH3)2(CH3OH)]5+ unit to respectively coordinate two Eu3+ and two Ti4+ ions from the [Eu3Ti5(µ3-O)6(µ2-OH)(µ3-OCH3)2(H2O)(CH3OH)]14+ unit. Measurement of their luminescence properties shows that the luminescence lifetime and quantum yield are 1212 µs and 69.6% for 1 and 857 µs and 56.2% for 2. When F- was introduced into 1 in a molar ratio of F- to 1 of 1:3, its quantum yield was increased 1.3 times, and the lifetime increased from 1.2 to 1.4 ms. However, no obvious enhancement in the emission intensity was observed in 2; even the molar ratio of F- to 2 is in the range from 2 to 1/4. Investigation on the structures of 1 and 2 reveals that the luminescence lifetime and quantum yield in 1 is significantly larger than that in 2 are attributed to the vibration of the nonradiative O-H vibration groups in 1 being significantly smaller than that in 2.

High-Nuclearity Chiral 3 d-4 f Heterometallic Clusters Ln6Cu24 and Ln6Cu12.

Based on the anion template and chiral ligand inducting role, two series of high-nuclearity 3 d-4 f heterometallic clusters with formulas [NO3@Ln6Cu24(µ3-OH)30(µ2-OH)3(OAc)6( R/ S-L)12(H2O)24](NO3)14· x(H2O) (Ln = Dy, x = 30 for 1a( R-L) and 1b( S-L); Ln = Tb, x = 40 for 2a( R-L) and 2b( S-L)) and (Et3NH)4[Ln6Cu12(µ3-OH)14(µ2-Cl)6Cl12( R/ S-L)12]Cl2· x(H2O) (Ln = Dy, x = 28 for 3a( R-L) and 3b( S-L); Ln = Tb, x = 33 for 4a ( R-L) and 4b( S-L); HL = ( R/ S)-1,2,3,4-tetrahydroisoquinoline-3-carboxylic acid), have been synthesized and characterized. Structural analysis reveals that the metal skeleton of compounds 1 and 2 display a Ln6Cu12 octahedral inner core encapsulated by six outer Cu2 units. In the Ln6Cu12 octahedron, 6 Ln3+ ions located at the six vertices and 12 inner Cu2+ ions located at the 12 edges of octahedron, and one NO3- locates in the center of the octahedron. The metal core of compounds 3 and 4 can be viewed as a Ln6 octahedron encapsulated by six Cu2 units. It is interesting that the different inorganic anions involved in the reaction result in the difference in the structures of 1 to 2 and 3 to 4. Circular dichroism spectra of 1-4 display obvious mirror symmetry effect at 600-800 nm of d-d transition of Cu2+, suggesting that the chirality transferred from chiral R- and S-ligand to Cu2+ ions in this system. Notably, the CD peak at the Cu2+ d-d transition position of Ln6Cu12 cluster is obviously blue-shifted compared with that of Ln6Cu24 due to the different coordinated environments of Cu2+. Magnetic studies indicate that 1a and 2a show weak ferromagnetic interactions, while 3a and 4a display antiferromagnetic interactions.

Structural Insights into the Broad Substrate Specificity of a Novel Endoglycoceramidase I Belonging to a New Subfamily of GH5 Glycosidases.

Endoglycoceramidases (EGCases) specifically hydrolyze the glycosidic linkage between the oligosaccharide and the ceramide moieties of various glycosphingolipids, and they have received substantial attention in the emerging field of glycosphingolipidology. However, the mechanism regulating the strict substrate specificity of these GH5 glycosidases has not been identified. In this study, we report a novel EGCase I from Rhodococcus equi 103S (103S_EGCase I) with remarkably broad substrate specificity. Based on phylogenetic analyses, the enzyme may represent a new subfamily of GH5 glycosidases. The X-ray crystal structures of 103S_EGCase I alone and in complex with its substrates monosialodihexosylganglioside (GM3) and monosialotetrahexosylganglioside (GM1) enabled us to identify several structural features that may account for its broad specificity. Compared with EGCase II from Rhodococcus sp. M-777 (M777_EGCase II), which possesses strict substrate specificity, 103S_EGCase I possesses a longer α7-helix and a shorter loop 4, which forms a larger substrate-binding pocket that could accommodate more extended oligosaccharides. In addition, loop 2 and loop 8 of the enzyme adopt a more open conformation, which also enlarges the oligosaccharide-binding cavity. Based on this knowledge, a rationally designed experiment was performed to examine the substrate specificity of EGCase II. The truncation of loop 4 in M777_EGCase II increased its activity toward GM1 (163%). Remarkably, the S63G mutant of M777_EGCase II showed a broader substrate spectra and significantly increased activity toward bulky substrates (up to >1370-fold for fucosyl-GM1). Collectively, the results presented here reveal the exquisite substrate recognition mechanism of EGCases and provide an opportunity for further engineering of these enzymes.

[Molecular structure and luminescent property of bis(2-(4-methyl-2-hydroxyphenyl)benzothiazolate) zinc].

Bis(2-(4-methyl-2-hydroxyphenyl)benzothiazolate) zinc(Zn(4-MeBTZ)2) was synthesized. Its molecular structure was confirmed by single-crystal x-ray diffraction. Single-crystal data are as follows: space group triclinic, P-1; a = 8.989 9(11) angstroms, b =12.161 7 (15) angstroms, c = 12.871 9 (16) angstroms, alpha = 63.492 (2) degrees, beta = 84.825 (2) degrees, gamma =71.187 (2) degrees. The steric hindrance provided by introduction methyl groups on phenoxide ring prohibited effectively the formation of pentacoordinate complex. There is distinct intermolecular pi-pi interaction between molecules. The dihedral angle between the phenol and benzothiazolate rings of Zn(4-MeBTZ)2 is 2.166 degrees. The HOMO energy, LUMO energy and optical gap are -5.84, -3.46 and 2.37 eV, respectively. The maximum wavelength peak of PL spectra located at 470 nm. The double-layer devices were employed using Zn(4-MeBTZ)2 as emitter and NPB as hole-transport material. The EL spectra split into two peaks located at 501 and 544 nm respectively. The broadened EL spectra were demonstrated to be originated from the exciplexes formed at the interface between NPB and Zn(4-MeBTZ)2.

[Characterization and photoluminescence properties of a blue-light-emitting material].

Tris (2-methyl-8-hydroxyquinoline) aluminum (AlMeq3) was synthesized and purified by vacuum sublimation. The structure of the complex was characterized by 1H NMR, FTIR spectra and elemental analysis techniques. AlMeq3 consists of three 2-methyl-8-hydroxyquinoline ligands and one aluminum atom. Its thermal stability was studied by TG and DSC analysis and the result shows that AlMeq3 is a thermally stable material, with decomposition and crystalline transition temperature being 357 and 158 degrees C, respectively. Energy band structure was investigated by UV-Vis absorption spectra and fluorescence emission spectra. Experimental results show that its UV absorption bands were at about 246 and 390 nm, and the absorption band at about 246 nm can be assigned to pi--pi* of phenyl ring. AlMeq2 displays 8-hydroxyquinoline-oriented photophysical properties. The optical gap of AlMeq3: was about 2.85 eV, as determined by intrinsic absorption band edge of the complex in ethanol solution. Under UV excitation at 365 nm, the complex in ethanol solution emitted intensive blue fluorescence with the maximum emission peak at 479 nm, while the effective energy-transfer from the ligand to the central AlP+ ion occurred in the complex. AlMeq3 with bright blue photoluminescence can be applied as luminescent material in OLEDs.

[Characterization and photoluminescence properties of an azomethin-zinc complex].

A Schiff base organic metal complex, Bis(salicylidene)-1,2-phenylenediam-ine Zinc(II) with high purity, was synthesized and purified by vacuum sublimation. Its structure, thermal stability and energy band structure were investigated by element analysis, FTIR spectra, TG-DTA curve, UV-Vis absorption spectra, fluorescece emission spectra and PL spectra. Experimental results showed that the complex is a thermally stable, polycrystalline material, with glass temperature and decomposition temperature being 183 and 449 degrees C, respectively. In its infrared spectrum, a high intensity band was at about 1 385 cm(-1). This band was typical of the conjugated C=N stretching vibration, which shifted to higher frequency in relation to the free ligand of salicylaldehyde with 1,2-phenylenediamine. The new bnd at 529 cm(-1) was assigned to Zn-O stretching vibration. Its UV absorption bands were at about 297 and 406 nm, and its tetrahydrofuran solution emitted intensive blue-green fluorescence at the peak wavelength of 508 nm. The absorption band at about 406 nm can be assigned to the intrinsic absorption of C=N. Its optical gap was about 2.62 eV, which was determined by the intrinsic absorption band edge of the complex in tetrahydrofuran solution. Under UV excitation at 365 nm, the complex in film emitted yellow-green fluorescence with the maximum emission peak at 562 nm and a full-width at half-maximum of 48.5 nm in PL spectra. Finally, yellow organic light-emitting devices using this complex as the emissive layer were fabricated and investigated.