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An 80-year-old woman with myelofibrosis sought evaluation for progressive dyspnea. Her past medical history included essential thrombocytosis, which transformed to myelofibrosis. Inspiratory computed tomography of chest showed diffuse mosaic attenuation with lymphadenopathy. Flexible bronchoscopy with lymph node and pulmonary parenchymal cryo biopsy revealed nodular deposits of extramedullary hematopoiesis in lung parenchyma and moderate to severe vascular medial and intimal thickening of pulmonary vasculature consistent with pulmonary parenchymal extramedullary hematopoiesis associated with pulmonary hypertension (a rare compensatory mechanism in myeloproliferative disorders). In this report, we explore the manifestations, pathogenesis, treatment, and prognosis of pulmonary extramedullary hematopoiesis reported in the literature.
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http://dx.doi.org/10.1016/j.rmcr.2022.101705 | DOI Listing |
J Pathol Clin Res
September 2025
Institute of Pathology and Neuropathology, University Hospital Tuebingen and Comprehensive Cancer Center South West, Tuebingen, Germany.
Myeloid sarcoma (MS) is a mass-forming extramedullary manifestation of myeloid blasts, either in relation to an underlying acute myeloid leukemia (AML), another myeloid neoplasm (MN) or as a de novo occurrence. Data on the genetic profile of MS are sparse. In this study, 41 MS of 34 patients, including 7 de novo cases and 24 patients with antecedent or synchronous MN, were analyzed with targeted next-generation sequencing (NGS), RNA-based fusion detection, and gene expression profiling (GEP).
View Article and Find Full Text PDFAm J Surg Pathol
September 2025
Department of Pathology.
Perinephric myxoid pseudotumor of fat (PMPTF) is a recently characterized lesion typically associated with non-neoplastic renal disease. Its pathogenesis is thought to result from chronic renal "irritation," either due to mass effect from renal carcinoma or inflammation related to benign renal conditions. Prompted by several cases arising in the absence of underlying renal pathology, we conducted a multi-institutional study of 29 mass-forming cases with detailed clinical, histologic, and molecular characterization.
View Article and Find Full Text PDFSci Adv
August 2025
Mouse Cancer Genetics Program, Center For Cancer Research, National Cancer Insitute-Frederick, NIH, Frederick, MD 21702, USA.
Hematopoietic malignancies emerge through the acquisition of genetic mutations within hematopoietic stem and progenitor cells (HSPCs). Some mutations impart a selective growth advantage to HSPCs, which expand and contribute to mature blood cells. This expansion is termed clonal hematopoiesis (CH).
View Article and Find Full Text PDFBlood Adv
August 2025
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, SC Medicina ad Indirizzo Metabolico, Italy.
Chronic anemia due to non-transfusion-dependent β-thalassemia (NTDT) can result in clinical morbidities, particularly with inadequate management. Luspatercept was previously shown to improve hemoglobin levels in patients with NTDT in the phase 2, randomized, double-blind, placebo-controlled BEYOND trial (ClinicalTrials.gov, NCT03342404).
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