A Rare Case of Intravascular Large B-Cell Lymphoma. (21st September 2018)

• Record Type: Journal Article
• Authors: Raza, Roshan; Singh, Rajendra
• Is Part Of: American journal of clinical pathology. Volume 150(2018)Supplement 1
• Page Start: S5
• Page End: S6
• Publication Date: 2018-09-21
• Subjects: Diagnosis, Laboratory -- Periodicals; Pathology -- Periodicals; 616.07
• DOI: 10.1093/ajcp/aqy090.012
• Languages: English
• ISSNs: 0002-9173

Abstract

Abstract: First reported in the literature in 1959, intravascular large B-cell lymphoma (IVLBCL) is a very rare subtype of large cell lymphoma characterized by the proliferation of clonal atypical lymphocytes within the lumina of small blood vessels, without any obvious extravascular tumor mass or readily observable circulating lymphoma cells in the peripheral blood. The surprising degree of sparing of the surrounding tissue and the absence of lymphoma cells in the lymph nodes and reticuloendothelial system are a hallmark of the disease. Here were present a case of a 63-year-old female, without any significant medical history, presenting with a macule on the left breast skin. A punch biopsy was performed, which on histology revealed atypical lymphoid cells (large cells with vesicular nuclear chromatin and prominent nucleoli) within the smaller blood vessels in the reticular dermis. There was no infiltration of these atypical cells in the surrounding dermis. With a strong suspicion of lymphoma, the first round of immunohistochemistry was performed, which showed negative results for CK7, CK20, mammaglobin, CD31, and CD68 and strong positivity for CK20, suggestive of IVLBCL. Second round of immunostains was positive for CD79a, MUM1, and BCL-2; CD10 was negative while CD3 showed admixed lymphocytes, supportive of the diagnosis. IVLBCL is a rare disease with an estimated incidence of less than one per million. Median age of incidence is 70 years (34–90 years). Patients present with a variety of constitutional and systemic symptoms (especially CNS involvement) caused by the occlusion of small vessels, but our patient did not present with any. Skin involvement can manifest as nodules/plaques (49%), macules (22.5%), telangiectatic patches (20%), and cellulitis. We present this rare and interesting case to highlight the existence of this entity since it carries a poor prognosis and requires a timely rituximab-containing chemotherapy plus CNS-oriented therapy.