Myeloproliferative Neoplasm-Unclassifiable (MPN-U) is defined as an MPN that fails to meet the diagnostic criteria for any of the other defined classical or 'non-classical' MPNs. The reported incidence is variable, dependent on appropriate recognition, and the clinical course can be highly variable ranging from an indolent disorder through to an aggressive disease course with a significant risk of thrombosis, bulky splenomegaly, and debilitating symptom burden. Clinicians frequently manage these conditions according to the clinical concerns e.g. splenomegaly akin to the phenotype, but no evidence base exists. Currently, there are no widely accepted guidelines to deal with both the diagnostic work up and subsequent clinical management of this entity. We hence surveyed major International MPN centres to gain an understanding of common challenges in MPN-U management in 2021 and aid identification of considerable practice variations where harmonisation would be desirable. Such information is vital to support future consensus guidelines in addition to informing further collaborative studies.
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| http://dx.doi.org/10.1016/j.retram.2022.103338 | DOI Listing |
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Abstract: Adrenal infarction, a rare ailment, has been sporadically linked to hematopoietic neoplasms.
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BMC Endocr Disord
June 2023
Division of Nephrology and Endocrinology, The University of Tokyo Hospital, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan.
Article Synopsis
- Adrenal infarction is a rare condition where parts of the adrenal glands get damaged, and it's often caused by blood conditions or diseases.
- An 81-year-old man came to the hospital with severe back pain, and doctors found he had this condition linked to a blood disease called MDS/MPN.
- This case is special because it's the first time someone has had adrenal infarction twice, and it shows how important it is to investigate the reasons behind these health issues.
Advances in molecular evaluation of myeloproliferative neoplasms.
Semin Diagn Pathol
May 2023
Department of Hematopathology, University of Texas MD Anderson Cancer Center, Houston, TX, United States. Electronic address:
Myeloproliferative neoplasms (MPN) are a group of clonal hematopoietic stem cell disorders with uncontrolled proliferation of one or more hematopoietic cell types, including myeloid, erythroid and megakaryocytic lineages, and minimal defect in maturation. Most MPN are associated with well-defined molecular abnormalities involving genes that encode protein tyrosine kinases that lead to constitutive activation of the downstream signal transduction pathways and confer cells proliferative and survival advantage. Genome-wide sequencing analyses have discovered secondary cooperating mutations that are shared by most of the MPN subtypes as well as other myeloid neoplasms and play a major role in disease progression.
View Article and Find Full Text PDF[Perforated upper gastrointestinal ulcers potentially attributable to mycophenolate mofetil after allogeneic hematopoietic stem cell transplantation].
Rinsho Ketsueki
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Department of Hematology, Nagano Red Cross Hospital.
A 46-year-old man with myelodysplastic syndrome/myeloproliferative neoplasm-unclassifiable underwent myeloablative bone marrow transplantation from an HLA-DR-1-antigen-mismatched related donor while receiving tacrolimus and mycophenolate mofetil (MMF) for graft-versus-host disease (GVHD) prophylaxis. However, grade III acute GVHD of the gut occurred on day 20 and was treated with prednisolone (PSL) and oral beclomethasone dipropionate while continuing MMF. Subsequently, he presented with progressive epigastric pain.
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