Intravascular Large B-cell Lymphoma Associated with Systemic and Central Nervous System Hemophagocytic Lymphohistiocytosis: A Case Report

Perm J. 2020:24:19.105. doi: 10.7812/TPP/19.105. Epub 2019 Dec 6.

Abstract

Introduction: We present a case of an Asian variant of intravascular large B-cell lymphoma associated with systemic and central nervous system hemophagocytic lymphohistiocytosis (HLH) with multiple neurologic manifestations.

Case presentation: A 68-year-old, previously healthy, Korean woman presented with a 4-week history of fever and weight loss. She had pancytopenia with neutropenia, a ferritin level of 5030 μg/L, and elevated liver enzyme levels. Bone marrow, liver biopsy specimens, and cerebrospinal fluid demonstrated hemophagocytosis. The patient was treated with the HLH-2004 protocol, but her disease relapsed 3 months later. A repeated liver biopsy specimen confirmed the initially missed diagnosis of intravascular large B-cell lymphoma, a known driver of HLH in patients of Asian origin. She was then treated with lymphoma-directed therapy and had a prompt resolution of fever and neurologic symptoms as well as normalization of her blood cell counts and ferritin level.

Discussion: This case serves as a reminder that patients with an Asian variant of intravascular large B-cell lymphoma frequently present with HLH and neurologic manifestations, including seizures, strokes, and cognitive deficits. A high index of suspicion is needed for prompt diagnosis and initiation of lymphoma-directed therapy.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Antineoplastic Agents / therapeutic use
  • Brain / diagnostic imaging
  • Central Nervous System Diseases / complications*
  • Diagnosis, Differential
  • Female
  • Humans
  • Lymphohistiocytosis, Hemophagocytic / complications*
  • Lymphohistiocytosis, Hemophagocytic / diagnostic imaging
  • Lymphohistiocytosis, Hemophagocytic / drug therapy
  • Lymphoma, B-Cell / complications*
  • Lymphoma, B-Cell / diagnostic imaging
  • Lymphoma, B-Cell / drug therapy
  • Magnetic Resonance Imaging / methods

Substances

  • Antineoplastic Agents