Leukocytosis is a common finding in pediatric patients, and the differential diagnosis can be broad, including benign reactive leukocytosis and malignant myeloproliferative disorders. Transient abnormal myelopoiesis is a myeloproliferative disorder that occurs in young infants with constitutional trisomy 21 and somatic GATA1 mutations. Most patients are observed, but outcomes span the spectrum from spontaneous resolution to life-threatening complications. Juvenile myelomonocytic leukemia is a highly aggressive myeloproliferative disorder associated with altered RAS-pathway signaling that occurs in infants and young children. Treatment typically involves hematopoietic stem cell transplantation, but certain patients can be observed. Early recognition of these and other myeloproliferative disorders is important and requires a clinician to be aware of these diagnoses and have a clear understanding of their presentations. This paper discusses the presentation and evaluation of leukocytosis when myeloproliferative disorders are part of the differential and reviews different concepts regarding treatment strategies.
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http://dx.doi.org/10.1182/hematology.2023000464 | DOI Listing |
Acta Med Indones
October 2024
Department of Internal Medicine, Faculty of Medicine, Universitas Airlangga, Surabaya, Indonesia.
This report describes a rare case of anuric acute kidney injury related to suspected urate nephropathy in a 23-year-old male with chronic phase of Chronic Myeloid Leukemia (CML). The patient presented with anuria and limb edema, with a history of imatinib-treated CML. Investigations revealed probable urate crystals causing bilateral hydronephrosis and hydroureters.
View Article and Find Full Text PDFCancers (Basel)
January 2025
Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, MN 55455, USA.
CNL is a rare subtype of MPNs characterized by persistent neutrophilia, bone marrow hypercellularity, and specific genetic mutations, particularly in the gene. Advances in molecular diagnostics have greatly enhanced our understanding of CNL, distinguishing it from other myeloproliferative disorders and refining diagnostic criteria. This review provides an updated overview of CNL, focusing on breakthroughs in genetic profiling, including novel mutations with potential prognostic value and implications for targeted therapy.
View Article and Find Full Text PDFHematol Oncol
January 2025
Division of Hematology, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan.
Various prognostic scoring systems in myelofibrosis (MF) have been developed to guide clinical decision-making in MF. However, discrepancies between different scoring systems for individual patients remain poorly understood, which can result in conflicting treatment recommendations. Moreover, data regarding there applicability in Asian populations remain scarce.
View Article and Find Full Text PDFNat Commun
January 2025
Department of Osteology and Biomechanics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Myeloproliferative neoplasm-associated myelofibrosis is a clonal stem cell process characterized by pronounced bone marrow fibrosis associated with extramedullary hematopoiesis and splenomegaly. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) represents the only curative treatment leading to bone marrow fibrosis regression. Here we provide an in-depth skeletal characterization of myelofibrosis patients before and after allo-HSCT utilizing clinical high-resolution imaging, laboratory analyses, and bone biopsy studies.
View Article and Find Full Text PDFMedicine (Baltimore)
January 2025
Division of Hematology/Oncology, Changhua Christian Hospital, Changhua, Taiwan.
Rationale: Chylothorax is a rare adverse effect that is associated with dasatinib, a tyrosine kinase inhibitor administered for chronic myeloid leukemia (CML) treatment. Most reported cases have described standard dosing. In this case report, we described a 43-year-old male patient with CML who developed chylothorax after 4 years of low-dose dasatinib therapy.
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