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Lymphomatoid granulomatosis
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Lymphomatoid granulomatosis

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Contributors: Jason Birnbaum MD, Lowell A. Goldsmith MD, MPH, Art Papier MD, William Van Stoecker MD
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Synopsis

Lymphomatoid granulomatosis : Cough, Fever, Rash, Hypoxemia, Malaise, Mental status alteration, Respiratory alkalosis, Dyspnea
Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus (EBV)-related B-cell lymphoproliferative disorder of the lungs with variable involvement of skin, central nervous system, liver, and kidneys. It mainly affects middle-aged adults with a mean age of 48 years and a male-to-female ratio of 2:1; rarely, children are affected. The most common presenting symptoms are fever (60%), cough (56%), malaise (35%), weight loss (35%), dyspnea (29%), and chest pain (13%). Less common symptoms include arthralgias, myalgias, and gastrointestinal (GI) complaints. LYG causes greater than 50% mortality, with most deaths occurring within 2 years of diagnosis. Cause of death is usually extensive destruction of the pulmonary parenchyma.

Skin involvement is seen in approximately 40% of patients, and the rash consists of subcutaneous nodules (36%), dermal nodules (27%), maculopapular eruptions (15%), macular erythema (15%), and ulcers (7%). The rash is typically generalized but can be localized to the legs, trunk, face, or arms. The skin lesions can occur before, simultaneously, or after the lung lesions.

Central and peripheral nervous systems are involved in approximately one-third of patients. Signs of brain involvement include confusion, ataxia, hemiparesis, and seizures. Cranial nerve involvement can cause Bell palsy, diplopia, transient blindness, proptosis, deafness, and vertigo. Peripheral neuropathy is a common complaint and usually presents as symmetric paresthesias and weakness of the lower limbs.

LYG can also involve multiple organs: kidney, liver, brain, and spleen, and less commonly the adrenals, heart, pancreas, GI tract, and prostate.

Risk factors include a prior history of lymphoma or leukemia, especially acute lymphocytic or myeloid leukemia in remission, and various immunodeficiency states, including AIDS, variable immunodeficiency, X-linked agammaglobulinemia, hypogammaglobulinemia, and impaired T-cell function.

There have been patients with similar pathologies occurring after organ transplant or treatment with methotrexate for rheumatoid arthritis.

Codes

ICD10CM:
D47.Z9 – Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue

SNOMEDCT:
239940004 – Lymphomatoid granulomatosis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated:11/19/2019
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Lymphomatoid granulomatosis
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A medical illustration showing key findings of Lymphomatoid granulomatosis : Cough, Fever, Rash, Hypoxemia, Malaise, Mental status alteration, Respiratory alkalosis, Dyspnea
Clinical image of Lymphomatoid granulomatosis - imageId=5924209. Click to open in gallery. caption: 'Superficial ulcers and an atrophic scar in the axilla.'
Superficial ulcers and an atrophic scar in the axilla.

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