A CD30-positive variant of intravascular large B-cell lymphoma presenting as diffuse interstitial lung disease and generalized lymphadenopathy: a case report

TL;DR

A rare case of a CD30-positive variant of intravascular large B-cell lymphoma presented with lung disease and swollen lymph nodes, expanding the known clinical features of this aggressive cancer.

Contribution

This case report identifies a CD30-positive variant of IVLBCL with a unique clinical presentation involving diffuse lung disease and lymphadenopathy.

Findings

The patient's lymph node biopsy confirmed intravascular and intrasinusoidal lymphoma cell infiltration with preserved nodal architecture.

Tumor cells exhibited strong CD30 expression (~70%), a rare feature potentially linked to the unusual clinical presentation.

The case expands the recognized phenotypic spectrum of IVLBCL to include diffuse interstitial lung disease and generalized lymphadenopathy.

Abstract

Intravascular large B-cell lymphoma (IVLBCL) is a rare and aggressive malignancy defined by the proliferation of neoplastic B-cells within the vascular lumen. While the disease can affect multiple organs, commonly manifesting as skin lesions, neurological deficits, or hepatosplenomegaly, it usually spares the lymph nodes and rarely presents as diffuse interstitial lung disease (DILD). We report a 62-year-old man admitted with recurrent fever and progressive dyspnea. Chest computed tomography revealed extensive bilateral interstitial lung disease, with ultrasonography showing generalized lymphadenopathy with preserved architecture. Although initial therapy led to a favorable response, the patient subsequently relapsed. Lymph node biopsy confirmed IVLBCL, characterized by intravascular and intrasinusoidal lymphoma cell infiltration with well-preserved nodal architecture. Notably, the tumor cells showed diffuse strong CD30 expression (~70%), a rare finding potentially linked to the unusual clinical presentation.

This case demonstrates that IVLBCL can present with diffuse interstitial lung disease and generalized lymphadenopathy, expanding its recognized phenotypic spectrum. It may represent a variant with CD30-mediated nodal homing and immune activation.

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