We report the case of a rather rare form of K-light chain deposition disease (LCDD) in a 61-year-old man with hypertension and rapidly progressing nephropathy. Laboratory findings prompted suspicion of the diagnosis which was confirmed by light-microscopic and immunofluorescent studies of samples taken by percutaneous renal and liver biopsy. Hepatic and urinary K-light chains were present; no circulating light chains were detected. Bone marrow examination evidenced mild infiltration of lymphoid cells, all positive for K-light chain staining. Plasma cells were within normal ranges. LCDD appeared as nodular glomerulosclerosis with rare crescents and extensive tubular involvement with K-light chain deposits. There was no evidence of altered liver function, nor was amyloid found in the bone marrow, kidney or liver. After one year of continuous therapy with melphalan and prednisone, the patient's renal function has not worsened. We conclude with a review of the clinical and physiopathological features of the light chain subgroup of monoclonal immunoglobulin deposition diseases (MIDD).
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Medicine (Baltimore)
November 2024
Hematologic Disease Center, The First Affiliated Hospital of Xinjiang Medical University, Xinjiang Uygur Autonomous Region Research Institute of Hematology, Urumqi, China.
Rationale: Primary plasma cell leukemia is a rare and highly aggressive malignancy of the blood system, with rapid disease progression and a high early mortality rate. Currently, there is no recognized therapeutic regimen, leading to the adoption of strategies typically utilized for multiple myeloma, which, however, exhibit limited efficacy. Selinexor is considered effective in treating relapsed/refractory multiple myeloma, but there are currently no reports on its application in primary plasma cell leukemia.
View Article and Find Full Text PDFKey Clinical Message: Plasma cell myeloma is a rare entity in the pediatric population. The peak incidence is in the seventh decade, with less than 2% of cases occurring in patients under the age of 40. It is worth noting that any destructive bony lesion in a child should be investigated.
View Article and Find Full Text PDFCells
March 2024
Neonatal Brain Injury Laboratory, Division of Biomedical Research, Nemours Children's Health, Wilmington, DE 19803, USA.
The NF-κB (nuclear factor K-light-chain-enhancer of activated B cells) transcription factor family is critical for modulating the immune proinflammatory response throughout the body. During the resting state, inactive NF-κB is sequestered by IκB in the cytoplasm. The proteasomal degradation of IκB activates NF-κB, mediating its translocation into the nucleus to act as a nuclear transcription factor in the upregulation of proinflammatory genes.
View Article and Find Full Text PDFClin Immunol
April 2024
Université Paris Cité, INSERM UMR1163, Imagine Institute, Paris, France; Study Center for Primary Immunodeficiencies, Necker-Enfants Malades Hospital - Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Pediatric Immuno-Hematology and Rheumatology Unit, Necker Hospital for Sick Children - AP-HP, Paris, France; French National Reference Center for Primary Immune Deficiencies CEREDIH, Necker University, Hospital for Sick Children - AP-HP, Paris, France.
Math Biosci
January 2023
Department Mathematical Sciences, Politecnico di Torino, Corso Duca degli Abruzzi 24, Torino, Italy. Electronic address:
Cells respond to hypoxia via the activation of three isoforms of Hypoxia Inducible Factors (HIFs), that are characterized by different activation times. HIF overexpression has many effects on cell behavior, such as change in metabolism, promotion of angiogenic processes and elicitation of a pro-inflammatory response. These effects are driving forces of malignant progression in cancer cells.
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