Unusual spontaneous improvement in Asian variant of intravascular large B-cell lymphoma

Takashi Ninomiya,Toru Nakamura,Atsuko Shirakawa,Shigeki Umemura,Nobuharu Fujii,Akio Hiraki,Haruhito Kamei,Hiromichi Yamane

doi:10.4236/ojim.2012.21004

Takashi Ninomiya, Toru Nakamura + Show 6 more

Open Access

https://doi.org/10.4236/ojim.2012.21004

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Abstract

The clinical course of the Asian variant of intravascular large B-cell lymphoma (AIVL) is generally very aggressive. We describe a case of AIVL demonstrating an unusual clinical course, with spontaneous improvement. An 81-year-old man with high-grade fever and thrombocytopenia was admitted to our hospital. Although we could not confirm the origin of his symptoms, they disappeared completely without intervention within 2 weeks. Three months later, however, thrombocytopenia reappeared and progressed. Finally, he was readmitted due to a subdural hemorrhage with high fever and he finally died of rapidly progressive multiple organ failure. Autopsy findings revealed the presence of B-cell lymphoma cells in microscopic vessels of many organs as well as hemophagocytosis in the bone marrow. He was diagnosed with AIVL with an unusual indolent clinical course with spontaneous improvement.

Highlights

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of extranodal diffuse large B-cell lymphoma (DLBCL)
IVLBCL is a rare subtype of extranodal DLBCL characterized by massive proliferation of large tumor cells within the lumina of small-to-medium-sized vessels [2]
Murase et al reported that most Japanese cases were associated with hemophagocytic syndrome and these cases were recognized as Asian variant of intravascular large B-cell lymphoma (AIVL)

Summary

CASE REPORT

An 81-year-old man was admitted to our hospital owing to high fever > 39 ̊C in August 2006. Physical examination revealed hepatosplenomegaly with no other abnormal findings, such as lymph node swelling or skin rash. Laboratory data showed pancytopenia (white blood cell count (WBC), 2870/μL; hemoglobin (Hgb), 10.7 g/dL; platelet count (Plt), 21,000/μL), and elevation of serum lactate dehydrogenase (LDH, 779 IU/L), C-reactive protein (CRP, 5.0 mg/dL), and soluble interleukin-2 receptor (sIL-2 receptor, 10,800 U/mL). We performed chest and abdominal computed tomography (CT) (Figure 1) or magnetic resonance imaging of the brain; no evidence was found for any abnormality except hepatosplenomegaly. We suspected hemophagocytic syndrome, due to viral infection or malignant lymphoma, but neither malignant cells nor hemophagocytosis were detected after two individual aspirations and biopsies of the bone marrow, respectively.

INTRODUCTION

DISCUSSION