We report the first case of systemic lupus erythematosus with hyperviscosity syndrome as the presenting manifestation. A polyclonal increase in all immunoglobulins was observed. Ultracentrifuge studies showed 17-19S intermediate immune complexes (IC) composed primarily of autoreactive IgG demonstrated at acid pH. This was confirmed by chromatographic fractionation of sera, since the intermediate IC eluted as did purified heat aggregated IgG. Small amounts of IgM and IgA in the intermediate IC were also demonstrated by more sensitive techniques, i.e., nephelometry, polyacrylamide gel electrophoresis, and electrophoretic blotting. The decrease of serum viscosity was associated with the decrease of circulating IC after plasmapheresis or combined treatment of prednisone and cyclophosphamide. Serum viscosity bore no relationship to the amount of dissociated immunoglobulins.
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Hum Pathol
December 2024
Departments of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, 77030, USA. Electronic address:
There has been remarkable progress over the past 80 years since Jan Waldenstrom first described patients with a hyperviscosity syndrome related to IgM paraprotein in 1944. The definition of Waldenstrom macroglobulinemia (WM) has evolved from a clinical syndrome to a distinct clinicopathologic entity with characteristic morphology, immunophenotype and molecular features. The landmark discovery of MYD88 mutation among most WM cases in 2012 marked the dawning of an era of molecular genomic exploration that led to a paradigm shift in clinical practice.
View Article and Find Full Text PDFAm J Emerg Med
November 2024
Department of Emergency Medicine, University of Ottawa, Ottawa, Canada. Electronic address:
Introduction: Multiple myeloma (MM) and its complications carry a high rate of morbidity and mortality.
Objective: This review evaluates MM and its complications, including presentation, diagnosis, and management in the emergency department (ED) based on current evidence.
Discussion: MM is the second most common hematologic cancer and associated with monoclonal plasma cell proliferation.
Am J Dermatopathol
December 2024
Pathologist, Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY.
Background: Microvascular thrombosis is key to the pathogenesis of calciphylaxis. C5b-9-mediated microvascular injury reflective of complement pathway activation could be a key pathophysiologic event.
Methods: We conducted a retrospective multicenter study of 24 patients who have had biopsy-supported calciphylaxis from the 2010-2022 data base from Emory where C5b-9 immunohistochemistry (IHC) had not been conducted and the 2019-2023 data base from Cornell where C5b-9 IHC was done as part of the routine calciphylaxis work up.
Front Vet Sci
November 2024
Department of Veterinary Science, University of Turin, Grugliasco, TO, Italy.
A 9-month-old female Jack Russell Terrier was referred because of two episodes of suspected syncope. Echocardiography revealed a large, isolated mid-muscular ventricular septal defect (7 mm) with bidirectional shunting. A diagnosis of Eisenmenger syndrome and erythrocytosis (68.
View Article and Find Full Text PDFCureus
October 2024
Rheumatology, Royal Free London NHS Foundation Trust, London, GBR.
Immunoglobulin G4 (IgG4)-related disease is a rare multi-system immune-mediated inflammatory condition. Pathologically, it is associated with an increase in plasma IgG4 levels and tissue infiltration of IgG4-secreting plasma cells of any organ. Clinical features vary, but it usually presents with the diffuse enlargement and dysfunction of the affected organ.
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