Necrotizing soft tissue infection (NSTI) remains associated with substantial morbidity and risk of death, especially in immunocompromised patients, such as those with multiple myeloma. Early diagnosis and treatment is crucial, yet particularly complicated in patients with atypical presentations and impaired host defenses.
A report of two cases of multiple myeloma and NSTI at a single institution. Review of pertinent international literature.
Necrotizing soft tissue infections are rare in patients with multiple myeloma. However, when they occur, the presentation may be atypical, and morbidity is high. Anti-tumor chemotherapy must be suspended, worsening an already-guarded prognosis.
A high index of suspicion for NSTI is necessary for immunocompromised patients, such as those with multiple myeloma, in view of the possibility of atypical presentation. Diagnosis may therefore be challenging, and a protracted course is possible because of host immunosuppression.
[Show abstract][Hide abstract] ABSTRACT: IgG4-related disease is a newly described systemic fibroinflammatory process, characterized by increase in IgG4-positive plasma cells. Its pathogenesis, including the role of IgG4, remains poorly understood. Plasma cell myeloma is typically associated with a large monoclonal serum spike, which is frequently of IgG isotype. We sought to identify and characterize a subset of IgG4-secreting myeloma, as it may provide a biological model of disease with high serum levels of IgG4. Six out of 158 bone marrow biopsies (4%) from patients with IgG myeloma expressed IgG4. Four patients were men and two were women, with a mean age of 64 (range 53-87) years. Imaging showed fullness of pancreatic head (1), small non-metabolic lymphadenopathy (1), and bone lytic lesions (6). Two patients developed necrotizing fasciitis. All had elevated serum M-protein (mean 2.4, range 0.5-4.2 g/dl), and none had definite signs or symptoms of IgG4-related disease. Four myelomas had plasmablastic morphology. Four had kappa and two had lambda light chain expression. Three cases expressed CD56. Two patients had a complex karyotype. In conclusion, the frequency of IgG4 myeloma correlates with the normal distribution of IgG4 isoform. The patients with IgG4 myeloma appear to have a high rate of plasmablastic morphology and could be predisposed to necrotizing fasciitis. Despite high serum levels of IgG4, none had evidence of IgG4-related disease. These findings suggest that the increased number of IgG4-positive plasma cells is not the primary etiologic agent in IgG4-related disease. Elevated serum levels of IgG4 is not sufficient to produce the typical disease presentation and should not be considered diagnostic of IgG4-related disease.Modern Pathology advance online publication, 13 September 2013; doi:10.1038/modpathol.2013.159.
Modern Pathology 09/2013; 27(3). DOI:10.1038/modpathol.2013.159 · 6.19 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Las infecciones necrosantes de tejidos blandos presentan altas tasas de mortalidad. Estas se han relacionado con diferentes causas, incluidas las enfermedades neoplásicas. En la literatura se ha descrito infección necrosante especialmente en pacientes con neoplasias del tracto gastrointestinal bajo. La presentación de este caso clínico tiene como objetivo recordar a los médicos de urgencias la pertinencia de considerar este diagnóstico en pacientes con neoplasias de colon y recto en estadio avanzado y presentar una breve revisión de la literatura.
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