E-Cadherin Expression and Blunted Interferon Response in Blastic Plasmacytoid Dendritic Cell Neoplasm

In this study, we investigated by immunohistochemical analysis the expression of E-cadherin (EC) on pDCs in reactive lymph nodes and tonsils, bone marrow, and in BPDCN. We compared the expression of EC in BPDCN to that in leukemia cutis (LC) and cutaneous lupus erythematosus (CLE), the latter typically featuring pDC activation. In BPDCN, we also assessed the immunomodulatory activity of malignant pDCs through the expression of several type I interferon (IFN-I) signaling effectors and downstream targets, PD-L1/CD274, and determined the extent of tumor infiltration by CD8-expressing T cells. In reactive lymph nodes and tonsils, pDCs expressed EC, whereas no reactivity was observed in bone marrow pDCs. BPDCN showed EC expression in the malignant pDCs in the vast majority of cutaneous (31/33 cases, 94%), nodal, and spleen localizations (3/3 cases, 100%), whereas it was more variable in the bone marrow (5/13, 38,5%), where tumor cells expressed EC similarly to the skin counterpart in 4 cases and differently in other 4. Notably, EC was undetectable in LC (n=30) and in juxta-epidermal pDCs in CLE (n=31). Contrary to CLE showing robust expression of IFN-I-induced proteins MX1 and ISG5 in 20/23 cases (87%), and STAT1 phosphorylation, BPDCN biopsies showed inconsistent levels of these proteins in most cases (85%). Expression of IFN-I-induced genes, IFI27, IFIT1, ISG15, RSAD2, and SIGLEC1, was also significantly (P
Source: The American Journal of Surgical Pathology - Category: Pathology Tags: Original Articles Source Type: research

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Two studies find that patients with either systemic or cutaneous disease have higher rates of multiple comorbidities, including those commonly reported before and those less frequently associated.Medscape Medical News
Source: Medscape Dermatology Headlines - Category: Dermatology Tags: Rheumatology News Source Type: news
ConclusionWe showed that different phenotypes of NPSLE were related to distinct patterns of underlying structural brain MRI changes. Especially the inflammatory phenotype of NPSLE was associated with the most pronounced brain volume changes, which might facilitate the diagnostic process in SLE patients with neuropsychiatric symptoms.Key Points• Neuropsychiatric systemic lupus erythematosus (NPSLE) patients showed a higher WMH volume compared to SLE patients with neuropsychiatric syndromes not attributed to SLE (non-NPSLE).• NPSLE patients with inflammatory phenotype showed a lower total brain and white matter vol...
Source: European Radiology - Category: Radiology Source Type: research
Conditions:   Chronic Myeloid Leukaemia Recurrent;   Chronic Myeloid Leukemia, in Relapse Intervention:   Diagnostic Test: Myeloid mutations measurement Sponsor:   Grupo Cooperativo de Hemopatías Malignas Recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
We present the case of an adolescent patient with pre-B-cell acute lymphoblastic leukemia (pre-B ALL) requiring ECMO support for septic shock and ARDS due to disseminated Legionella. To our knowledge, this is the first case describing an immunocompromised pediatric patient supported with ECMO for Legionnaires' disease.PMID:34658413 | PMC:PMC8499640 | DOI:10.1182/ject-2100020
Source: Journal of Extra-Corporeal Technology - Category: Cardiovascular & Thoracic Surgery Authors: Source Type: research
Am J Cancer Res. 2021 Sep 15;11(9):4470-4484. eCollection 2021.ABSTRACTPonatinib is a tyrosine kinase inhibitor (TKI) directed against BCR-ABL1 which is successfully used in patients with BCR-ABL1 T315I+ chronic myeloid leukemia (CML). However, BCR-ABL1 compound mutations may develop during therapy in these patients and may lead to drug resistance. Asciminib is a novel drug capable of targeting most BCR-ABL1 mutant-forms, including BCR-ABL1T315I, but remains ineffective against most BCR-ABL1T315I+ compound mutation-bearing sub-clones. We demonstrate that asciminib synergizes with ponatinib in inducing growth-arrest and apo...
Source: Cell Research - Category: Cytology Authors: Source Type: research
Source: Biologics: Targets and Therapy - Category: Biochemistry Tags: Biologics: Targets and Therapy Source Type: research
We report a 34-year-old Filipino female patient who received dasatinib as upfront therapy for the treatment of CP-CML who experienced chronic diarrhea for 2 months, which progressed to colitis.Case Rep Oncol 2021;14:1441 –1446
Source: Case Reports in Oncology - Category: Cancer & Oncology Source Type: research
Contributors : Steve Horvath ; Vera Gorbunova ; Andrei SeluanovSeries Type : Methylation profiling by arrayOrganism : Heterocephalus glaber ; Homo sapiens ; Mus musculus ; Rattus norvegicusAn Infinium microarray platform (GPL28271, HorvathMammalMethylChip40) was used to generate DNA methylation data from many tissues of the naked mole ratWe generated DNA methylation data from n=289 tissues. All tissues except for skin biopsies and blood were obtained from frozen tissue collection that were euthanized for other studies. Skin biopsies (2 mm punch) were collected from the backs of the animals under local anesthesia. Blood sam...
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Methylation profiling by array Heterocephalus glaber Homo sapiens Mus musculus Rattus norvegicus Source Type: research
In this study, we evaluated a single-center cohort of 44 patients with a diagnosis of myeloid and LPD focusing on clinical features, therapy requirement, and outcome. The two diagnoses were concomitant in 32% of patients, while myeloid disease preceded LPD in 52% of cases (after a median of 37 months, 6–318), and LPD preceded myeloid neoplasm in 16% (after a median of 41 months, 5–242). The most prevalent LPD was non-Hodgkin lymphoma (50%), particularly lymphoplasmacytic lymphoma (54.5%), followed by chronic lymphocytic leukemia (27%), plasma cell dyscrasias (18.2%), and rarer associations such as Hodgkin lymph...
Source: Frontiers in Oncology - Category: Cancer & Oncology Source Type: research
The objective of this study was to compare the contributions of genetic ancestry, socioenvironmental factors, and inflammation-related health conditions to ethnic differences in C-reactive protein levels. We used multivariable regression to compare CRP blood serum levels between Black and White ethnic groups from the United Kingdom Biobank (UKBB) prospective cohort study. CRP serum levels are significantly associated with ethnicity in an age and sex adjusted model. Study participants who identify as Black have higher average CRP than those who identify as White, CRP increases with age, and females have higher average CRP t...
Source: Frontiers in Genetics - Category: Genetics & Stem Cells Source Type: research
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