Intravascular large B-cell lymphoma

Page most recently updated 7 September 2026

This page focuses on the reported psychiatric associations, which may be the initial presenting manifestation, and on other clinical features that may assist the psychiatrist or primary care physician in identifying the underlying diagnosis, considered through the lenses of the diagnostic lenses framework.

Intravascular large B-cell lymphoma (IVLBCL) is a rare, aggressive form of non-Hodgkin lymphoma that can involve multiple organ systems, with particularly important manifestations involving the central nervous system, skin, and hematologic system. A distinctive presentation may include the combination of rapidly progressive neuropsychiatric symptoms, fever or other constitutional symptoms, characteristic cutaneous lesions, cytopenias, and markedly elevated LDH, particularly in the absence of prominent lymphadenopathy.

It can present with psychiatric features such as depressive symptoms, delusions, hallucinations, and behavioural or personality change, which may occur alongside or precede more overt cognitive or neurological abnormalities. Psychotic symptoms are uncommon but can occur without an overt delirium syndrome.

Other characteristic features includerapidly progressive or fluctuating neurological syndromes, particularly cognitive impairment or encephalopathy, multifocal ischaemic events or stroke-like presentations, seizures, myelopathy, and focal neurological deficits. Neurological involvement may be multifocal and evolve over time, with brain or spinal MRI abnormalities sometimes mimicking cerebral vasculitis or recurrent infarction. A haemophagocytic syndrome-associated phenotype can present with persistent fever, cytopenias, hepatosplenomegaly and systemic inflammation.

From a multisystemic lens perspective, clinical features in other body systems include (but are not limited to):

  • Endocrine: Pituitary, adrenal or thyroid involvement may cause endocrine dysfunction, including hypopituitarism, adrenal insufficiency, central hypothyroidism or diabetes insipidus.

  • Hematologic: Anaemia and other cytopenias. Markedly elevated lactate dehydrogenase (LDH) is an important laboratory clue.

  • Dermatologic: Painful, indurated erythematous or violaceous plaques, nodules, or other infiltrative skin lesions, particularly affecting the trunk or extremities. Cutaneous involvement can provide an important diagnostic clue and an accessible site for biopsy.

Early diagnosis is important to allow prompt initiation of potentially disease-modifying chemoimmunotherapy and CNS-directed treatment where indicated, thereby reducing the risk of irreversible neurological injury, progressive multiorgan dysfunction, and other serious complications. Because IVLBCL has highly heterogeneous presentations, substantial delays in diagnosis may occur; maintaining clinical suspicion and obtaining timely tissue biopsy are particularly important.

Selected references and further reading — Multisystemic lens

Cardinal RN, Bullmore ET. The diagnosis of psychosis. Cambridge: Cambridge University Press; 2011.

Sachdev PS, Keshavan MS, editors. Secondary schizophrenia. Cambridge: Cambridge University Press; 2010.

Levenson JL, editor. The American Psychiatric Association Publishing textbook of psychosomatic medicine and consultation-liaison psychiatry. 3rd ed. Washington (DC): American Psychiatric Association Publishing; 2019.

Stern TA, Beach SR, Smith FA, Freudenreich O, Vranceau AM, Fava M, editors. Massachusetts General Hospital handbook of general hospital psychiatry. 8th ed. Philadelphia: Elsevier; 2025.

Arciniegas DB, Yudofsky SC, Hales RE, editors. The American Psychiatric Association Publishing textbook of neuropsychiatry and clinical neurosciences. 6th ed. Washington (DC): American Psychiatric Association Publishing; 2018.

Agrawal N, Faruqui R, Bodani M, editors. Oxford textbook of neuropsychiatry. Oxford: Oxford University Press; 2020.

Boland R, Verduin M, editors. Kaplan and Sadock's comprehensive textbook of psychiatry. 11th ed. Philadelphia: Wolters Kluwer; 2024.

Loscalzo J, Fauci AS, Kasper DL, Hauser SL, Longo DL, Jameson JL, editors. Harrison's principles of internal medicine. 22nd ed. New York: McGraw Hill; 2025.