Category Ranking
98%
Total Visits
921
Avg Visit Duration
2 minutes
Citations
20
Article Abstract
Essential thrombocythemia (ET) is a myeloproliferative neoplasm characterised by sustained thrombocytosis. Paradoxically, bleeding complications remain an under-recognised clinical challenge. Compared with CALR-mutated patients, those harbouring the JAK2-V617F variant appear more prone to haemorrhage. This may be secondary to acquired von Willebrand syndrome (AvWS) and intrinsic platelet dysfunction. AvWS in ET arises from extreme platelet counts driving the adsorption and proteolysis of high-molecular-weight von Willebrand factor (VWF) multimers, producing a qualitative VWF defect akin to type 2A von Willebrand disease. However, the platelet count threshold for AvWS is variable, and patients with platelet counts below 1000 × 10/L may still exhibit clinically significant VWF anomalies. Diagnosis relies on tests such as VWF activity and antigen levels. Perioperative management of patients with AvWS focuses on correcting the VWF defect and/or lowering platelet counts. Ultimately, clinicians must balance between preventing thrombosis and bleeding by tailoring management to each patient. The authors have confirmed clinical trial registration is not needed for this submission.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC12231194 | PMC |
| http://dx.doi.org/10.1002/jha2.70088 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
LRIG1 is a positive prognostic marker in Merkel cell carcinoma and Merkel cell carcinoma expresses epithelial stem cell markers.
Virchows Arch
December 2021
Department of Plastic Surgery, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine malignancy of the skin. The cell of origin of MCC is thus far unknown and proposed cells of origin include Merkel cells, pro-/pre- or pre-B cells, epithelial stem cells, and dermal stem cells. In this study, we aimed to shed further light on the possibility that a subset of MCC tumors arise from epithelial stem cells of the skin by examining the expression of hair follicle and epidermal stem cell markers in MCC and normal human skin.
View Article and Find Full Text PDFA novel MPLKIP-variant in three Finnish patients with non-photosensitive trichothiodystrophy type 4.
Am J Med Genet A
June 2021
The Folkhaelsan Department of Medical Genetics, The Folkhaelsan Institute of Genetics and the Department of Medical and Clinical Genetics, Medicum, University of Helsinki, Helsinki, Finland.
Article Synopsis
- - Trichothiodystrophy is a rare genetic disorder characterized by abnormal hair development and affects multiple body systems; this study focuses on two Finnish families with this condition.
- - The researchers identified a new mutation in the MPLKIP gene through whole-exome sequencing, confirming the diagnosis of non-photosensitive trichothiodystrophy type 4 (TTD4) in three patients.
- - This report enhances understanding of TTD4 by detailing the patients' unique physical traits and comparing their clinical features with previously documented cases.
Quantitative sensory phenotyping in chronic neuropathic pain patients treated with unilateral L4-dorsal root ganglion stimulation.
J Transl Med
October 2020
Institute of Neurophysiology, Medical Faculty Mannheim, University Heidelberg, Heidelberg, Germany.
Article Synopsis
- A study looked at a treatment called L4-DRG stimulation for patients with a painful condition called CRPS.
- The researchers tested how patients felt different sensations like pain and touch before and after 3 months of treatment.
- They found that the treatment helped reduce pain for the patients, but it did not change how they felt warmth or touch.
Lichen planus pigmentosus-inversus in a Finnish man.
J Eur Acad Dermatol Venereol
February 2019
Department of Dermatology, Allergology and Venereology, Helsinki University Central Hospital, Helsinki, Finland.
Oral Platelet-Derived Growth Factor and Vascular Endothelial Growth Factor Inhibitor Sunitinib Prevents Chronic Allograft Injury in Experimental Kidney Transplantation Model.
Transplantation
January 2016
1 Transplantation Laboratory, University of Helsinki, Helsinki, Finland. 2 Department of surgery, Oulu University Central Hospital, Oulu, Finland. 3 Transplantation and Liver Surgery Unit, Helsinki University Central Hospital, Helsinki, Finland. 4 Division of Nephrology, Department of Medicine, Hels
Article Synopsis
- Increased expression of PDGF and VEGF is linked to chronic rejection in kidney transplants, which can lead to allograft loss.
- Sunitinib, a tyrosine kinase inhibitor, was tested in a rat model and shown to significantly reduce neointimal formation, smooth muscle cell activity, and chronic rejection signs while improving kidney function.
- The findings suggest that targeting both PDGF and VEGF with sunitinib may offer a promising new approach for preventing chronic rejection in kidney transplant patients.