54. Lymphomatoid Granulomatosis
Definition
Lymphomatoid granulomatosis (LYG) is a rare angiodestructive, lymphoproliferative disease with significant pulmonary involvement in the form of pulmonary angiitis and granulomatosis. LYG mimics Wegner’s granulomatosis (see p. 345), both in clinical and radiographic presentation.
Incidence
The frequency of LYG occurrence is not known. Males are affected more than females at a 2:1 rate.
Etiology
The true cause of LYG is not known. Recent evidence strongly suggests it is a distinct type of malignant lymphoma associated with immunosuppression. LYG is distinguished from Wegner’s granulomatosis by the involvement of B-cells.
Signs and Symptoms
Definitive diagnosis of LYG is based on the histologic triad of (1) granulomatosis, (2) polymorphic lymphocytic infiltrate, and (3) angiitis.
Symptoms include:
• Ataxia
• Cough
• Distal sensory neuropathy
• Dyspnea
• Erythema
• Fever
• Hemiparesis
• Hemoptysis
• Hepatomegaly
• Lymphocytic infiltration of meninges, cerebral vessels, and peripheral nerves
• Malaise
• Mental status changes
• Mononeuritis multiplex
• Patchy, painful erythematous macules, papules, and plaques
• Pneumonia
• Seizures
• Weight loss
Medical Management
A consensus for treatment of LYG has not been established. Regimens cover the spectrum from observation and occasional spontaneous remission, to prednisone administration with or without chemotherapy using agents such as cyclophosphamide. Antilymphoma regimens are employed if or when LYG progresses to a high-grade form of lymphoma, but response typically has been poor.
Localized or isolated pulmonary tumors or masses may be surgically excised and followed by chemotherapy. Interferon alfa-2b or ganciclovir are currently being used experimentally to treat LYG.
Complications
• Diabetes insipidus
• Hemoptysis
• Hemorrhage
• Mental status changes
• Mononeuropathy
• Pneumothorax
• Progressive respiratory disease
• Pulmonary infection
• Respiratory failure
• Seizures
• Sepsis
• Transformation to a high-grade lymphoma
Anesthesia Implications
The primary organs targeted by LYG are the lungs. Preoperatively the patient should receive both pulmonary function testing and arterial blood gas analysis to evaluate the extent of lung dysfunction present. Ventilatory support intraoperatively, and potentially postoperatively, will need to be individualized based on the results of the pulmonary function tests and ABGs. Postoperative ventilatory support should be discussed with the patient and/or family before surgery.
Hypoadrenalism, hypercalcemia, hypothyroidism, and hypogonadism have all been reported in association with LYG. Treatment of any of these associated conditions should be assessed and initiated before surgery and anesthesia. Supplemental steroids may be needed for patients receiving prednisone.
Neurologic sequelae of LYG do not occur very often. Typically any central nervous system (CNS) involvement results from cranial nerve palsies. Therefore the patient with LYG should undergo a good neurologic examination preoperatively.
The patient receiving chemotherapy agents, whether cyclophosphamide or vincristine, should be assessed for potential complications associated with the treatment regimen. Cyclophosphamide is associated with myelosuppression; vincristine is associated with the development of peripheral neuropathy. Any condition associated with the ongoing chemotherapy regimen should be documented during the preanesthesia interview/assessment.
