DOI: 10.12890/2026_007411
Intravascular large B-cell lymphoma presenting with rhabdomyolysis and inflammatory myositis: a diagnostic challenge
Authors
Queen.Okereke@health.qld.gov.au
Keywords
Intravascular large B cell Lymphoma, random skin biopsies, myositisAbstract
Introduction: Intravascular large B-cell lymphoma (IVLBCL) is a rare and aggressive extranodal lymphoma characterised by the proliferation of lymphoma cells within small blood vessels. Distinctively, IVBCL typically lacks the lymphadenopathy, discrete tumour masses, or circulating lymphoma cells characteristic of other lymphomas. This results in substantial diagnostic delays and historically high rates of postmortem diagnosis. Common clinical features of IVLBCL include fever of unknown origin, cytopenias, elevated lactate dehydrogenase, and neurological dysfunction. This case highlights the diagnostic challenges of IVLBCL and an unusual presentation with rhabdomyolysis and inflammatory myositis
Case description: An 80-year-old man presented with recurrent falls, progressive lower limb weakness, fever, and a left foot drop. Investigations revealed bicytopenia, acute kidney injury, and markedly elevated creatine kinase (peaking at 11,200 U/l). Despite extensive negative investigations including positron emission tomography scans and bone marrow biopsy, his condition deteriorated with progressive neurological impairment and evidence of myositis on magnetic resonance imaging (MRI). The diagnosis was established by random deep skin biopsies obtained from clinically normal-appearing skin.
Conclusion: IVLBCL should be considered in patients with unexplained fever, cytopenias, and neurological decline. Rhabdomyolysis and myositis-like MRI findings are rare but important manifestations. Random incisional skin biopsy is a key diagnostic tool, offering a high diagnostic yield even in the absence of cutaneous lesions. Early recognition of this clinical constellation and timely random skin biopsy can facilitate diagnosis and enable potentially life-prolonging therapy.
References
- Ponzoni M, Ferreri AJ, Campo E, Facchetti F, Mazzucchelli L, Yoshino T, et al. Definition, diagnosis, and management of intravascular large B-cell lymphoma: proposals and perspectives from an international consensus meeting. J Clin Oncol. 2007;25(21):3168-73. doi: 10.1200/JCO.2006.08.2313.
- Liu Y, Ma Y, Zhou H, Zhou X, Shao J. Analysis of clinicopathological features and prognostic factors of non-Hodgkin’s intravascular large B-cell lymphoma. Oncol Lett. 2020;20(4):43. doi: 10.3892/ol.2020.11908.
- Ponzoni M, Campo E, Nakamura S. Intravascular large B-cell lymphoma: a chameleon with multiple faces and many masks. Blood. 2018;132(15):1561-1567. doi: 10.1182/blood-2017-04-737445.
- Henter JI. Hemophagocytic Lymphohistiocytosis. N Engl J Med. 2025;392(6):584-598. doi: 10.1056/NEJMra2314005.
- Marini-Bettolo C, Lane R, Charles P, Naresh K, Nicholas R, et al. Myopathy secondary to intravascular large B-cell lymphoma. Neuromuscul Disord. 2009;19(12):856-9. doi: 10.1016/j.nmd.2009.09.003.
- Shimada K, Kosugi H, Shimada S, Narimatsu H, Koyama Y, Suzuki N, et al. Evaluation of organ involvement in intravascular large B-cell lymphoma by 18F-fluorodeoxyglucose positron emission tomography. Int J Hematol. 2008;88(2):149-153. doi: 10.1007/s12185-008-0102-7.
- Asada N, Odawara J, Kimura S, Aoki T, Yamakura M, Takeuchi M, et al. Use of random skin biopsy for diagnosis of intravascular large B-cell lymphoma. Mayo Clin Proc. 2007;82(12):1525-7. doi: 10.1016/s0025-6196(11)61097-5.
- Enzan N, Kitadate A, Tanaka A, Matsue K. Incisional random skin biopsy, not punch biopsy, is an appropriate method for diagnosis of intravascular large B-cell lymphoma: a clinicopathological study of 25 patients. Br J Dermatol. 2019;181(1):200-201. doi: 10.1111/bjd.17603.
- Shimada K, Matsue K, Yamamoto K, Murase T, Ichikawa N, Okamoto M, et al. Retrospective analysis of intravascular large B-cell lymphoma treated with rituximab-containing chemotherapy as reported by the IVL study group in Japan. J Clin Oncol. 2008;126(19):3189-95. doi: 10.1200/JCO.2007.15.4278.
- Ferreri AJ, Dognini GP, Govi S, Crocchiolo R, Bouzani M, Bollinger CR, et al. Can rituximab change the usually dismal prognosis of patients with intravascular large B-cell lymphoma? J Clin Oncol. 2008;26(31):5134-6; author reply 5136-7. doi: 10.1200/JCO.2008.19.1841.
