Intravascular large B-cell lymphoma presenting with fever and refractory acidosis

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 smalland 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.

Intravascular Large B-Cell Lymphoma: A Review with a Focus on the Prognostic Value of Skin Involvement

“…A recent case of a Chinese patient, for example, showed both neurological involvement and hemophagocytic syndrome [17] (Table 1). Rather unusual and rare presentations of IVLBCL reported in the literature include refractory acidosis [18], spontaneous tumour lysis syndrome, dyspnoea with subsequent lactic acidosis, pulmonary embolism, affection of the cervix uteri [19], renal involvement with elevated serum anti-neutrophil cytoplasmic antibody (ANCA) titres [20], primary prostatic [21], or uveal [22] involvement. These various clinical presentations show that IVLBCL can affect virtually any organ system, depending on the location of the vasculopathy resulting from the intraluminal proliferation of neoplastic lymphoid cells.…”