Lymphoreticular Syndrome

At a glance

It is characterized by an idiopathic enlargement of mesenteric lymph nodes associated with debilitating sprue.

Incidence

Extremely rare; only occurs in association with celiac sprue (or disease).

Genetic inheritance

Unknown.

Pathophysiology

Uncertain. Debilitated patients with celiac sprue develop mesenteric lymph node enlargement and cavitation anemia, and duodenal ulcerations. This leads to worsening of the failure to thrive most often associated with celiac sprue.

Diagnosis

Based on the clinical picture. Patients may have repeated negative biopsies for lymphoma, despite a highly suspicious presentation. Lymphoreticular Syndrome is diagnosed in the presence of celiac sprue.

Clinical aspects

Anemia, diarrhea, electrolyte derangement, hyposplenism duodenal ulceration, total parenteral nutrition are the most frequent features.

Precautions before anesthesia

Check volemia, baseline cell blood count, and electrolytes.

Anesthetic considerations

The anesthetic considerations must be adapted according to the procedure and underlying medical condition associated with the clinical presentation. For instance, discontinue total parenteral nutrition perioperatively and adapt intravenous glucose administration (10% solution might be necessary) to prevent hypoglycemia.

Pharmacological implications

No reported pharmacological implications.

Other condition to be considered

• Castleman’s Syndrome (Castleman’s Disease; Giant Lymph Node Hyperplasia; Lymphoid Hamartoma; Angiofollicular Lymph Node Hyperplasia Syndrome): Group of uncommon lymphoreticular proliferative disorders involving the lymph nodes histological features. It is characterized by waxing and waning mild lymph nodes enlargement, with intense inflammation, hepatomegaly, splenomegaly, ascites, pleural effusions, organ failure, and even death. Several subtypes have been defined under a classification called unicentric or multicentric. It is caused by excessive secretion of cytokines, particularly IL-6 which is the most commonly elevated cytokine one. It is named after Benjamin Castleman.

References