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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.

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Department of Respiratory and Critical Care Medicine, The Second Hospital of Dalian Medical University, Dalian, Liaoning Province, China.

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  • Extramedullary plasmacytoma is a rare malignant tumor found in tissues outside of bone marrow, with primary pulmonary plasmacytoma (PPP) being particularly uncommon and diagnosed through biopsy.
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Imaging in multiple myeloma.

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Multiple myeloma (MM) is the second most common adult hematologic malignancy, characterized by clonal proliferation of malignant plasma cells mostly in the bone marrow. The presence of destructive changes of the mineralized bone is a hallmark feature of the condition and a sign of end-organ damage. Due to this, imaging plays an integral role in the diagnosis, prognostication, and treatment monitoring of patients undergoing therapy for MM as well as surveillance of patients with early-stage disease.

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Myeloproliferative neoplasms, polycythemia vera, essential thrombocytosis, and primary myelofibrosis are a unique group of clonal hematopoietic stem cell neoplasms that share somatic, gain-in-function driver mutations in 2, , and . As a consequence, these disorders exhibit similar phenotypic features, the most common of which are the ceaseless production of normal erythrocytes, myeloid cells, platelets alone or in combination, extramedullary hematopoiesis, myelofibrosis, and a potential for leukemic transformation. In the case of polycythemia vera and essential thrombocytosis, however, prolonged survival is possible.

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  • * The case study highlights a patient with relapsed-refractory EMD who initially presented with a para-osseous plasmacytoma that later evolved into a soft-tissue plasmacytoma with different immunoglobulin characteristics.
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