Cellular Therapy and Transplantation

Proteasome-associated autoinflammatory syndromes (PRAAS) are rare genetic diseases which are characterized by various phenotypes and are classified as primary immunodeficiencies.PRAAS consists of 4 common syndrome variants: chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature syndrome; joint contractures, muscle atrophy, hepatomegaly, splenomegaly, microcytic anemia and panniculitis-induced lipodystrophy syndrome; Nakajo-Nishimura syndrome and Japanese autoinflammatory syndrome with lipodystrophy.Symptoms include periodic fever, skin rash, partial lipomuscular atrophy, and joint contracture.We presented a case of CANDLE syndrome with homozygous mutation on exon 2 (c.274G > A) PSMB8 gene.

Tatiana Bykova

Anna Zvyagintseva

Anna Osipova

Andrey Kozlov

Anna Botina

Vadim Baykov

Ludmila Zubarovskaya

CANDLE syndrome: chronic atypical neutrophilic dermatosis with lipodystrophy and fever

CANDLE综合征：慢性非典型中性粒细胞性皮肤病伴脂肪营养不良和发热

The aim of our study was to compare different regimes of hematopoietic stem cell (HSC) mobilization, i.e., cyclophosphamide combined with granulocyte colony-stimulating factor (Cy/G-CSF), versus cytarabine with G-CSF (Ara-C/G-CSF).Efficiency and toxicity of these treatment regimens were assessed.Achievement of partial remission of the disease was sufficient for successful mobilization.CD34+ cell numbers in the blood showed a significant correlation with total number of CD34+ yielded by apheresis on the same day.When comparing the two groups of mobilization we have revealed that the patients treated with AraC + G-CSF yielded higher amounts of CD34+ cells in peripheral blood on the first apheresis day.Moreover, lesser occurrence of toxicity was revealed in group of patients treated with AraC-G-CSF.

Diana Babenetskaya

Dmitry Motorin

Elza Lomaia

George Baratashvili

Renat Badaev

Alexey Petrov

Julia Alekseeva

Darina Zammoeva

Dmitry Pevcov

Maria Sharapova

Andrey Zaritskey

Cyclophosphamide versus cytarabine mobilization of hematopoietic stem cells with G-CSF stimulation in myeloma patients subjected to autologous stem cell transplantation

环磷酰胺与阿糖胞苷动员骨髓瘤患者自体造血干细胞移植中的造血干细胞，并联合G-CSF刺激

Dear colleagues and friends,Th is brief note concerns my refl ections on the international meetings which I had the pleasure to attend in 2022, as well

Rüdiger Hehlmann

Remarkable events in oncohematology 2022

2022年肿瘤血液学领域的重大事件

Nadezhda Orlova

Alexander Muravyov

Anna Gorelova

Anna Remezova

Tatiana Vinogradova

Natalia Yudintсeva

Yulia Nashchekina

Alexander Gorbunov

Alexander Lebedev

Andrei Gorelov

Petr Yablonsky

Mesenchymal stem cells in complex treatment of urinary tract diseases: a literature review and our own experience

复杂治疗泌尿系统疾病中的间充质干细胞：文献综述与我们的经验

Children with Down syndrome (DS, OMIM #190685) have an increased risk for a hematopoietic malignancy.Down syndrome-associated AML (DS-AML) is diagnosed in children under 4 years, being often characterized as acute megakaryoblastic leukemia (AMegL), with somatic mutations in the GATA1 gene, and a relatively favorable course of the disease.This disease is commonly preceded by a stage of bone marrow failure with morphological myelodysplasia of erythroid and megakaryocytic cells.Additional chromosomal abnormalities in the DS-MDS/AML karyotypes are described in 20-35% of patients.To date the published data on the cytogenetic changes that occur during the evolution of DS-AML with an assessment of their prognostic impact are still insufficient. Case presentationsWe present three clinical observations of DS-MDS/AML in children under 4 years old.In all patients, DS-AML was preceded by DS-MDS.One patient was diagnosed with AMegL with a non-mutated GATA1 gene.In two others, the M0-AML and M4-AML FAB variants were diagnosed, and in both, the pathogenic GATA1 mutations were found.In all patients, cytogenetic and molecular cytogenetic analyzes, in addition to constitutive trisomy 21, revealed unbalanced translocations that led to the formation of one or two additional copies of duplicated segments 1q25-q44 / 1q32-q44, 11q13-q25 in the genome and trisomy of chromosome 11 thus resulting in dismal outcomes of the disease. ConclusionOur findings suggest that partial trisomy of the chromosome 1 with a duplication of segments 1q25-1q44 or 1q32-q44 in combination with complete or partial (11q13-q25) trisomy of the chromosome 11 may be indicative for a poor prognosis in DS-MDS/AML.

Tatiana Gindina

Polina Kozhokar

Sabina Ryabenko

Artem Gusak

Sergey Riumin

Tatiana Gracheva

Ildar Barkhatov

Elena Babenko

Olesya Paina

Elena Semenova

Nikolay Mamaev

Alexander Kulagin

Nonrandom extra copies of 1q and 11q in the karyotypes of three new cases of acute myeloid leukemia associated with Down syndrome

与唐氏综合征相关的三例急性髓系白血病染色体核型中1q和11q非随机额外拷贝

Jaleel Mohammed

Alice Volkova

Hadeel Bakhsh

Zahid Asghar

The 4‘I’s for a holistic musculoskeletal rehabilitation approach following hematopoietic stem cell transplantation

造血干细胞移植后整体骨骼肌肉康复方法的四个“I”

Nina Gurgenidze

Oleg Goloshapov

Alexander Shvetco

Vladimir Zavarukhin

Alisa Volkova

Zhemal Rakhmanova

Julia Rodneva

Irina Baranova

Tatyana Bykova

Ivan Moiseev

Successful skin alloplasty and allogeneic finger phalanx transplantation from haploidentical donor in ALL patient with severe chronic GvHD

成功在严重慢性移植物抗宿主病ALL患者中进行单倍体供者皮肤移植及异体指骨移植

Ruba Joujeh

Dima Joujeh

The effect of plant extracts and plant-derived compounds on mesenchymal stromal cell differentiation

植物提取物和植物源化合物对间充质基质细胞分化的影响

Alexey Polushin

Maria Chernaya

Anastasia Orlovskaya

Ekaterina Krasnova

Evgenia Lopatina

Alexander Tsynchenko

Yuri Zalyalov

Anna Lisker

Anna Volkova

Yuri Khalimov

Endocrine complications of high-dose immunosuppressive therapy with autologous hematopoietic stem cell transplantation in autoimmune diseases

自身免疫病自体造血干细胞移植高剂量免疫抑制治疗中的内分泌并发症

Uliana Karpunina

Airat Sadykov

First experience with BAALC-expressing leukemia stem cell fraction in juvenile myelomonocytic leukemia patients treated by hematopoietic stem cell transplantation

首次体验BAALC表达的白血病干细胞在青少年骨髓单核细胞白血病患者造血干细胞移植中的作用

Publication Information	Publisher: Universitatsklinikum Hamburg - Eppendorf，Publishing cycle: ，Journal Type: journal，Open Access Journals: No
Basic data	Year of publication: 2015，Proportion of original research papers: 0.00%，Self Citation Rate:N.A.， Gold OA Rate: 0.00%

Cellular Therapy and Transplantation

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