Intravascular Large B-Cell Lymphoma Detected by Cutaneous Biopsy with Pancytopenia and Negative Bone Marrow Biopsy

Case Report

J Blood Disord. 2023; 10(2): 1078.

Intravascular Large B-Cell Lymphoma Detected by Cutaneous Biopsy with Pancytopenia and Negative Bone Marrow Biopsy

Battis N¹*; Gardeen S²; Kozlowski A²; Furda-Raine L¹

¹Department of Dermatology, Park Nicollet Health System, USA

²Department of Dermatology, Health Partners Institute, USA

*Corresponding author: Battis N Department of Dermatology, Park Nicollet Health System, 7550 34th Ave. S., Suite 101, Minneapolis, MN 55450, USA. Tel: (+1) 952-220-1064; Fax: 952-883-9746 Email: nicholas.battis@parknicollet.com

Received: June 23, 2023 Accepted: July 25, 2023 Published: August 01, 2023

Abstract

Intravascular large B-cell lymphoma is a B-cell lineage neoplasm, which is typically recognized as a high-grade neoplasm with poor outcomes. Though rare, the cutaneous variant can present with multiple lesions of the skin and negative systemic staging. We present a case of intravascular large B-cell lymphoma first identified on cutaneous biopsy, with pancytopenia, in addition to negative bone marrow biopsy and serum protein electrophoresis. The patient is a 71-year-old male who presented with upper body pruritis and pancytopenia, as well as weight loss and fatigue. A full body skin exam incidentally revealed a well-demarcated, firm, bound down atrophic lichenified plaque on the right anterior thigh. A punch biopsy revealed large, atypical lymphoid cells exclusively localized within the lumina of variably sized blood vessels, immunoreactive for CD20, BCL6, MUM1, and CD5, but negative for CD3, CD10, cyclin D1, and TdT. A diagnosis of intravascular large B-cell lymphoma was made, and he was referred to oncology for further treatment. Full body skin exam is a mainstay of dermatologic practice, and it is essential to pursue biopsy with uncharacteristic lesions, regardless of the presenting concern.

Keywords: IVLBCL; Lymphoma; Intravascular; Cutaneous biopsy; Intravascular large B-cell lymphoma

Abbreviations

CRP: C-Reactive Protein; RBC: Red Blood Cell; WBC: White Blood Cell; PLT: Platelet; SPEP: Serum Protein Electrophoresis; IVLBCL: Intravascular Large B-cell Lymphoma; DIF: Direct Immunofluorescence; FISH: Fluorescence in Situ Hybridization; HLH: Hemophagocytic Lymphohistiocytosis; R-HD-MTX-ARA-C: Rituximab with High-Dose Methotrexate and Cytarabine; R-CHOP: Rituximab, Cyclophosphamide, Doxorubicin Hydrochloride, Vincristine, and Prednisone; WHO: World Health Organization

Case Presentation

We present a case of intravascular large B-cell lymphoma first identified on cutaneous biopsy, with pancytopenia, in addition to negative bone marrow biopsy and serum protein electrophoresis.

A 71-year-old male presented to dermatology clinic on referral from oncology due upper body pruritus with pancytopenia, weight loss, and fatigue. On exam, he had well-defined, eroded, and crusted papules in various stages of healing on the bilateral arms and upper back with ill-defined, subtle, thin, pink papules coalescing into thin plaques on the neck. On the right anterior thigh, a well-demarcated, firm, bound down atrophic lichenified plaque was incidentally noted. The patient was adamant that this was due to crossing his legs while watching television, however when having the patient cross his legs, there was no appreciable pressure in the location of the plaque.

Laboratory evaluation was notable for an elevated Anti-Nuclear Antibody (ANA) of 1:640, copper of 153.4 μg/dL, ferritin of 2,124 ng/mL, and C-Reactive Protein (CRP) of 4.1 mg/dL. Also notable was his pancytopenia with a Red Blood Cell (RBC) count of 3.18*1012/L, White Blood Cell (WBC) count of 3.4*109/L, and Platelet (PLT) count of 55*109/L.

Prior to presentation in dermatology, a bone marrow biopsy was hypocellular without increase in blasts, nor morphologic evidence of lymphoma. Serum Protein Electrophoresis (SPEP) was within normal limits. Given his presentation without unifying diagnosis, skin biopsies were performed (Figure 1).