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Intravascular large B-cell lymphoma presenting with fever and refractory acidosis
Department of PathologyStorandt, Michael Harrison; Department of PathologyKoponen, Mark Alan.
  • Department of PathologyStorandt, Michael Harrison; University of North Dakota. School of Medicine and Health Sciences. Department of PathologyStorandt, Michael Harrison. Grand Forks. US
  • Department of PathologyKoponen, Mark Alan; University of North Dakota. School of Medicine and Health Sciences. Department of PathologyKoponen, Mark Alan. Grand Forks. US
Autops. Case Rep ; 11: e2021324, 2021. graf
Artigo em Inglês | LILACS | ID: biblio-1285406
ABSTRACT
Intravascular large B-cell lymphoma (IVLBCL) is a rare form of diffuse large B-cell lymphoma, characterized by malignant B-cells primarily localized to the lumina of small- and medium-sized blood vessels without lymphadenopathy. Two patients initially presented with fever of unknown origin and persistent lactic acidosis without evidence of tissue hypoxia. Neither patient had an identifiable source of infection and both underwent peripheral blood smear demonstrating leukocytosis with a neutrophilic predominance and thrombocytopenia without evidence of hematologic malignancy. One had previously had a bone marrow biopsy which was unremarkable. Both patients' condition deteriorated rapidly, progressing to multiorgan failure requiring pressors and mechanical ventilation, which ultimately resulted in cardiopulmonary arrest. At autopsy, each patient demonstrated malignant lymphocytoid cells, staining positive for CD20, localized to the lumina of small- and medium-sized vessels in multiple organs, including the lungs, liver, spleen, and kidneys, among others, allowing for the diagnosis of IVLBCL. IVLBCL is exceedingly rare, which in combination with significant variability in presentation, can make identification and diagnosis challenging. Diagnosis requires biopsy, therefore a high index of suspicion is needed to obtain an adequate tissue sample, whether pre- or postmortem. In the presented cases, both patients exhibited type B lactic acidosis with an unknown etiology that was ultimately determined at autopsy.
Assuntos


Texto completo: DisponíveL Índice: LILACS (Américas) Assunto principal: Acidose Láctica / Linfoma não Hodgkin / Linfoma Difuso de Grandes Células B / Neoplasias Hematológicas Tipo de estudo: Estudo prognóstico Limite: Idoso / Aged80 / Feminino / Humanos / Masculino Idioma: Inglês Revista: Autops. Case Rep Assunto da revista: Anatomia / Patologia Cl¡nica / Patologia Legal Ano de publicação: 2021 Tipo de documento: Artigo País de afiliação: Estados Unidos Instituição/País de afiliação: University of North Dakota/US

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Texto completo: DisponíveL Índice: LILACS (Américas) Assunto principal: Acidose Láctica / Linfoma não Hodgkin / Linfoma Difuso de Grandes Células B / Neoplasias Hematológicas Tipo de estudo: Estudo prognóstico Limite: Idoso / Aged80 / Feminino / Humanos / Masculino Idioma: Inglês Revista: Autops. Case Rep Assunto da revista: Anatomia / Patologia Cl¡nica / Patologia Legal Ano de publicação: 2021 Tipo de documento: Artigo País de afiliação: Estados Unidos Instituição/País de afiliação: University of North Dakota/US