Background: The role of natural killer (NK) cells in plasma cell diseases has not yet been fully characterized.
Case Report: We present the case of a 47-year-old man with an extremely aggressive extramedullary plasmacytoma of the lung with associated cutaneous lesions, whose disease was accompanied by a significantly decreased number of NK cells (CD56+, CD16+, CD3-) in the peripheral blood, very low NK cell activity levels, and a decreased interleukin-2 production from cultured cells in vitro. Histology and immunohistochemistry of the lung and cutaneous lesions identified that the tumor was composed of clonal plasma cells which were CD38+++, CD138+++, lambda chain+, kappa chain-, and cytokeratin-. Bone marrow histology and cytology were initially normal. The disease progressed rapidly despite local radiotherapy and systemic chemotherapy, and the patient died shortly after diagnosis.
Conclusions: Cutaneous involvement in extramedullary plasmacytoma represents a clinically aggressive variant of plasma cell tumor, which runs a rapid course and has associated devastating effects on the patient's innate immune system.
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http://dx.doi.org/10.1159/000278713 | DOI Listing |
Multiple myeloma is a disease related to the proliferation of malignant plasma cells; in most patients, the disease is confined to the level of bone marrow. However, in a minority of patients, the malignant plasma cells are also localized outside the bone marrow, either at the level of peripheral blood (plasma cell leukemia) or at the level of soft tissues (extramedullary multiple myeloma). These two rare forms of aggressive MM (ultrahigh-risk (uHR) MM as MM leading to death within 24-36 months) are both associated with some molecular features and with a limited response to current treatments.
View Article and Find Full Text PDFOncol Ther
January 2025
Hematology, Department of Translational and Precision Medicine, Policlinico Umberto I-Sapienza University, Via Benevento 6, 00161, Rome, Italy.
Introduction: Myelofibrosis (MF) is often characterized by a multifactorial anemia determined, in part, by bone marrow (BM) fibrosis, extramedullary erythropoiesis and splenomegaly. Ruxolitinib (RUX) is the first-in-class janus kinase 2 (JAK2) inhibitor approved for treatment of MF, proved to reduce spleen volume and decrease symptom burden. The red cell distribution width (RDW) is the measure of erythrocyte volume variability (anisocytosis).
View Article and Find Full Text PDFLeukemia
January 2025
Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria.
Expression of CD2, CD25 and/or CD30 in extracutaneous mast cells (MC) is a minor diagnostic criterion for systemic mastocytosis (SM) in the classification of the World Health Organization and International Consensus Classification. So far, it remains unknown whether expression of these antigens on MC is of prognostic significance in SM. We performed a retrospective multi-center study of patients with SM using the data set of the registry of the European Competence Network on Mastocytosis, including 5034 patients with various MC disorders.
View Article and Find Full Text PDFHematol Oncol
January 2025
Babak Myeloma Group, Department of Pathophysiology, Faculty of Medicine, Masaryk University, Brno, Czech Republic.
Multiple myeloma is a plasma cell malignancy characterized by an abnormal increase in monoclonal immunoglobulins. Despite significant advances in treatment, some patients progress to more aggressive forms of multiple myeloma, including extramedullary disease or plasma cell leukemia. Although the exact molecular mechanisms are not known, several studies have confirmed the involvement of small extracellular vesicle-enriched microRNAs in multiple myeloma progression.
View Article and Find Full Text PDFJ Neurosurg Case Lessons
January 2025
Department of Radiology and Biomedical Imaging, University of California, San Francisco, California.
Background: Spinal ependymomas are typically slow-growing tumors with a favorable prognosis. Recently, a new aggressive subtype has emerged with its own distinct histopathological and molecular features characterized by MYCN amplification. However, this subtype of spinal ependymoma is rare, and studies on its imaging characteristics are limited.
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