Pre-Fibrotic Myelofibrosis is a frequently under-recognised entity that has distinct features separate to those of both Essential Thrombocythaemia and overt Primary Myelofibrosis. Misdiagnosis is relatively common due to subtle differences in bone marrow trephine morphology and multidisciplinary approaches are required. The clinical phenotype and disease course is heterogeneous and hence management approaches tend to vary widely. Although patients may initially be asymptomatic, disease-related complications can include troublesome symptom burdens, increased incidence of both arterial and venous thromboses, haemorrhage, anaemia and an inherent risk of disease evolution to either overt myelofibrosis or blastic phase disease. Specific prognostic tools with high discriminatory power are lacking. Within this review we use case-based approaches to review the current literature, highlight challenges in both diagnostics and disease management and suggest contemporary approaches to improve patient outcomes.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.beha.2022.101378 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Spleen volume assessment in Ph-negative chronic myeloproliferative neoplasms: a real-life study comparing ultrasonography vs. magnetic resonance imaging scans.
Ann Hematol
January 2025
Hematology and Hematopoietic Stem Cell Transplant Center, Department of Medicine and Surgery, University of Naples Federico II, Via S. Pansini 5, Naples, 80131, Italy.
Splenomegaly is a quite common clinical feature of Philadelphia (Ph) negative chronic myeloproliferative neoplasms (MPNs) and its presence may, in some cases, drives treatment decision. Most importantly, palpable splenomegaly is a minor criterion for both pre-fibrotic/early primary myelofibrosis and primary myelofibrosis (PMF) diagnosis, even if clinical assessment by physical examination is poorly reliable and accurate. On the other hand, despite the International Working Group-Myeloproliferative Neoplasms Research and Treatment and European LeukemiaNet guidelines defined spleen response criteria by palpation, they also recognized the highly subjective nature of spleen size assessment by physical examination, and recommended objective confirmation of volume reduction via computed tomography or magnetic resonance imaging (MRI).
View Article and Find Full Text PDFWarm Autoimmune Hemolytic Anemia as a Rare Presentation of Pre-fibrotic Primary Myelofibrosis.
Cureus
December 2024
Hematology and Oncology, Olive View University of California Los Angeles (UCLA) Medical Center, Sylmar, USA.
Primary myelofibrosis (PMF) is an uncommon chronic myeloproliferative disorder that is commonly associated with Janus kinase 2 (JAK-2), calreticulin (CALR), or thrombopoietin receptor (MPL) mutations. Pre-fibrotic PMF (also known as pre-PMF or early PMF) is a subtype of PMF that is defined by a lower grade of fibrosis. In this report, we present a rare case of warm autoimmune hemolytic anemia (wAIHA) associated with pre-PMF.
View Article and Find Full Text PDFA comparative retrospective study of pre-fibrotic primary myelofibrosis overtly fibrotic stage in Qatar: clinicopathological, genetic landscape, risk stratification and survival data (2008-2021) - a single center experience.
Hematology
December 2024
Department of Haematology and Bone Marrow Transplant, National Center for Cancer Care and Research (NCCCR), Hamad Medical Corporation, Doha, Qatar.
Article Synopsis
- This study presents the first extensive report on Primary Myelofibrosis (PMF) in Qatar, covering data collected over 13 years, as there is a significant lack of information about PMF in the MENA region.
- Findings show that pre-PMF patients differ from overt PMF patients in terms of blood counts and genetic features, with overt PMF having a higher risk category and worse disease progression.
- The research provides important insights into the importance of the DIPSS plus scoring system, highlighting its effectiveness in identifying high-risk patients who face worse outcomes and increased treatment needs.
Different inflammatory, fibrotic, and immunologic signatures between pre-fibrotic and overt primary myelofibrosis.
Haematologica
October 2024
Departments of Medical Sciences; Departments of Cancer Evolution Research Center; Departments of Pathology, College of Medicine, The Catholic University of Korea, Seoul.
Primary myelofibrosis (PMF) is a myeloid proliferative neoplasm (MPN) characterized by bone marrow (BM) fibrosis. Pre-fibrotic PMF (pre-PMF) progresses to overt PMF. Megakaryocytes (MKs) play a primary role in PMF; however, the functions of MK subsets and those of other hematopoietic cells during PMF progression remain unclarified.
View Article and Find Full Text PDF[Recent advances in bone marrow pathology for myeloproliferative neoplasms diagnosis].
Rinsho Ketsueki
September 2024
ITO. MD Pathology Laboratory.
Identification of driver genes and pathological bone marrow diagnosis were considered essential for subclassification of MPN in the revised 4th edition of the WHO classification, and this remained nearly unchanged in the 5th edition. Pathological diagnosis of primary myelofibrosis/pre-fibrotic stage by biopsy is now feasible and is becoming standardized. Progressive myelofibrosis and blast transformation are the advanced forms of MPN, and various therapeutic interventions for these forms have been devised.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!