Published online Dec 24, 2019. doi: 10.5306/wjco.v10.i12.402
Peer-review started: March 8, 2019
First decision: April 18, 2019
Revised: August 21, 2019
Accepted: November 17, 2019
Article in press: November 17, 2019
Published online: December 24, 2019
Intravascular large B-cell lymphoma (IVLBCL) is a rare and aggressive subtype of non-Hodgkin lymphoma with a varied presentation and no pathognomonic findings. Early diagnosis is critical to altering the disease course as early treatment with chemoimmunotherapy is required to prevent a rapidly fatal outcome. Strategies including improved awareness of this clinical entity through publication of cases with unique presentations are essential to prompt consideration of IVLBCL early in the disease workup. Here, we present a case of IVLBCL presenting with altered mental status and systemic organ dysfunction.
A 61-year-old male patient presented with flu-like symptoms and a high fever. He experienced rapid clinical deterioration with liver, kidney failure, and shock despite rapid antibiotic administration and supportive care. A broad infectious workup was negative. Intracranial imaging revealed nonspecific changes to the corpus callosum suspicious for vasculitis. Renal biopsy was non-diagnostic. After further progression of his symptoms, the family elected to withdraw care and the patient died shortly thereafter. Post-mortem analysis revealed clear multi-organ involvement by IVLBCL, prompting re-examination of the ante-mortem renal biopsy that also identified IVLBCL involvement.
IVLBCL is a rare disease. Communication with specialties and early biopsy is critical to establishing the diagnosis and initiating therapy.
Core tip: Early diagnosis is critical to altering the disease course of intravascular large B-cell lymphoma (IVLBCL). A triad of B symptoms, organ dysfunction, and an elevated lactate dehydrogenase should prompt consideration of IVLBCL and early biopsy of affected tissues for rapid diagnosis and treatment. Close communication with pathology in cases of suspected IVLBCL is critical in evaluating for this rare and evasive diagnosis.