Intravascular large B-cell lymphoma presenting as unilateral lymphedema in a patient with rheumatoid arthritis and sarcoidosis : A case report
- 1Division of Respirology, Rheumatology, Infectious Diseases, and Neurology, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan
- 2Section of Oncopathology and Morphological Pathology, Department of Pathology, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan.
Abstract
Rationale:: Intravascular large B-cell lymphoma (IVLBCL) is an uncommon subtype of diffuse large B-cell lymphoma, typically presenting with nonspecific symptoms and lacking lymphadenopathy or visible tumor lesions, which makes early diagnosis challenging.
Patient concerns:: A 66-year-old female developed progressive unilateral lymphedema despite ongoing treatment for rheumatoid arthritis and sarcoidosis. Sarcoidosis-related lymph node lesions were also considered as a potential cause, and the prednisolone dose was increased. Despite these measures, the edema worsened, and a fever of around 38°C developed. At the initial evaluation, the serum soluble interleukin-2 receptor level was already elevated at 9985 U/mL.
Diagnoses:: Initial investigations, including imaging, bone marrow aspiration, and random skin biopsies, failed to reveal malignancy. Subsequent monitoring revealed that the serum level of serum soluble interleukin-2 receptor had risen further to 19,835 U/mL and that thrombocytopenia was present, prompting a repeat random skin biopsy that ultimately confirmed IVLBCL through the identification of intravascular cluster of differentiation 20-positive lymphoma cells.
Interventions:: Chemotherapy with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone was initiated. However, the patient developed peripheral neuropathy, suspected to be an adverse drug reaction. Consequently, the regimen was switched to polatuzumab vedotin, rituximab, cyclophosphamide, doxorubicin, and prednisolone.
Outcomes:: After completing 8 courses, the patient achieved remission. The left lower-limb lymphedema improved significantly.
Lessons:: This case highlights several clinically significant points: first, IVLBCL can present solely as unilateral lymphedema without detectable lymph node enlargement or tumorous lesions, underscoring the diagnostic difficulty. Second, repeated random skin biopsies, including adipose-rich vascular tissue, are crucial in establishing a definitive diagnosis when initial biopsies are inconclusive. Third, patients with underlying autoimmune conditions such as rheumatoid arthritis and sarcoidosis, particularly those receiving immunosuppressive therapies, may have an increased susceptibility to lymphoma, suggesting that systemic immune dysregulation could contribute to disease onset.
Related
- Pulmonary Sarcoidosis Presenting as a Solitary Pulmonary Nodule With Probable Neurosarcoidosis: A Case Report
- Recurrent Sarcoidosis Presenting With Bilateral Leg Edema: A Case Report
- Intravascular large B-cell lymphoma (IVLBCL) presenting with exclusive cutaneous involvement, no B-symptoms and negative PET-CT: A Case Study.
- Combined Vitamin B12 and Folate Deficiency Presenting As Pseudo-Thrombotic Microangiopathy With a Disseminated Intravascular Coagulation (DIC)-Like Coagulation Profile: A Case Report
- Overlap syndrome between sarcoidosis and Sjögren’s disease presenting as hypopituitarism: case report and review of literature
BibTeX
@article{kimura2026intravascular,
title = {{Intravascular large B-cell lymphoma presenting as unilateral lymphedema in a patient with rheumatoid arthritis and sarcoidosis : A case report}},
author = {Masatoshi Kimura and Haruka Sasaki and Risa Kudo and Masayuki Murai and Yuki Rikitake and Chihiro Iwao and Kousho Iwao and Ayako Aizawa and Takeshi Kawaguchi and Motohiro Matsuda and Shunichi Miyauchi and Ichiro Takajo and Takumi Kiwaki and Kunihiko Umekita and Taiga Miyazaki},
journal = {Medicine},
year = {2026},
month = oct,
doi = {10.1097/md.0000000000051323},
url = {https://europepmc.org/article/PMC/PMC13727228},
note = {CC BY},
}
RIS
TY - JOUR
TI - Intravascular large B-cell lymphoma presenting as unilateral lymphedema in a patient with rheumatoid arthritis and sarcoidosis : A case report
AU - Masatoshi Kimura
AU - Haruka Sasaki
AU - Risa Kudo
AU - Masayuki Murai
AU - Yuki Rikitake
AU - Chihiro Iwao
AU - Kousho Iwao
AU - Ayako Aizawa
AU - Takeshi Kawaguchi
AU - Motohiro Matsuda
AU - Shunichi Miyauchi
AU - Ichiro Takajo
AU - Takumi Kiwaki
AU - Kunihiko Umekita
AU - Taiga Miyazaki
PY - 2026
DA - 2026/10/30
JO - Medicine
DO - 10.1097/md.0000000000051323
UR - https://europepmc.org/article/PMC/PMC13727228
AB - Rationale:: Intravascular large B-cell lymphoma (IVLBCL) is an uncommon subtype of diffuse large B-cell lymphoma, typically presenting with nonspecific symptoms and lacking lymphadenopathy or visible tumor lesions, which makes early diagnosis challenging. Patient concerns:: A 66-year-old female developed progressive unilateral lymphedema despite ongoing treatment for rheumatoid arthritis and sarcoidosis. Sarcoidosis-related lymph node lesions were also considered as a potential cause, and the prednisolone dose was increased. Despite these measures, the edema worsened, and a fever of around 38°C developed. At the initial evaluation, the serum soluble interleukin-2 receptor level was already elevated at 9985 U/mL. Diagnoses:: Initial investigations, including imaging, bone marrow aspiration, and random skin biopsies, failed to reveal malignancy. Subsequent monitoring revealed that the serum level of serum soluble interleukin-2 receptor had risen further to 19,835 U/mL and that thrombocytopenia was present, prompting a repeat random skin biopsy that ultimately confirmed IVLBCL through the identification of intravascular cluster of differentiation 20-positive lymphoma cells. Interventions:: Chemotherapy with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone was initiated. However, the patient developed peripheral neuropathy, suspected to be an adverse drug reaction. Consequently, the regimen was switched to polatuzumab vedotin, rituximab, cyclophosphamide, doxorubicin, and prednisolone. Outcomes:: After completing 8 courses, the patient achieved remission. The left lower-limb lymphedema improved significantly. Lessons:: This case highlights several clinically significant points: first, IVLBCL can present solely as unilateral lymphedema without detectable lymph node enlargement or tumorous lesions, underscoring the diagnostic difficulty. Second, repeated random skin biopsies, including adipose-rich vascular tissue, are crucial in establishing a definitive diagnosis when initial biopsies are inconclusive. Third, patients with underlying autoimmune conditions such as rheumatoid arthritis and sarcoidosis, particularly those receiving immunosuppressive therapies, may have an increased susceptibility to lymphoma, suggesting that systemic immune dysregulation could contribute to disease onset.
SN - 0304-5412
SN - 1536-5964
SN - 0025-7974
ER -