Lymphomatoid granulomatosis

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.

Lymphomatoid granulomatosis is a rare Epstein–Barr virus (EBV)-associated B-cell lymphoproliferative disorder that most commonly affects the lungs but can also involve the central nervous system, skin, kidneys, and liver. A distinctive presentation may include pulmonary nodules or infiltrates accompanied by neuropsychiatric and cutaneous manifestations.

It can present with psychiatric features such as personality or behavioural change, depression, irritability, cognitive impairment, and, rarely, psychotic symptoms. Acute psychosis has been reported as a presenting manifestation of CNS lymphomatoid granulomatosis.

Other characteristic features include headache, seizures, altered consciousness, ataxia, focal neurological deficits, cranial neuropathies, and progressive cognitive or behavioural changes. Constitutional symptoms such as fever, fatigue, weight loss, and malaise are also common. Renal and hepatic involvement may occur but can be clinically silent, with routine renal function, urinalysis, and liver biochemistry often remaining normal in the absence of substantial organ involvement.

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

  • Respiratory: Pulmonary involvement is characteristic and may cause persistent cough, dyspnoea, chest tightness or discomfort, and, less commonly, haemoptysis. Imaging typically demonstrates multiple pulmonary nodules or infiltrates, often bilateral.

  • Hematologic: Anaemia is relatively common; leukopenia or leukocytosis and thrombocytopenia may also occur.

  • Dermatologic: Cutaneous involvement commonly produces erythematous macules, papules, plaques, or subcutaneous nodules, which may be disseminated and can ulcerate; lesions may be tender or pruritic.

Early diagnosis is important to identify the extent of extranodal and central nervous system involvement, initiate grade-appropriate treatment, reduce the risk of progressive organ damage and transformation to an aggressive B-cell lymphoma, and improve prognosis and quality of life.

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.