Intestinal lymphangiectasia associated with angiofollicular lymph node hyperplasia (Castleman's disease)

Area of Science:

• Gastroenterology
• Hematology
• Oncology

Background:

• Castleman's disease is a rare lymphoproliferative disorder with distinct clinicopathological subtypes.
• The hyaline vascular type is the most common variant, typically presenting with localized lymphadenopathy and often asymptomatic.
• Systemic symptoms are generally uncommon in the hyaline vascular Castleman's disease.

Observation:

• A patient with a history of myocardial infarction presented with significant gastrointestinal symptoms.
• Clinical examination revealed ascites, hepatosplenomegaly, para-aortic lymphadenopathy, thrombocytosis, and paraproteinemia.
• Jejunal biopsy demonstrated lymphangiectasia, while spleen and lymph node histology confirmed angiofollicular hyperplasia consistent with hyaline vascular Castleman's disease.

Findings:

• This case represents a previously undescribed association between Castleman's disease (hyaline vascular type) and prominent gastrointestinal manifestations.
• The presence of systemic symptoms, including ascites and thrombocytosis, is atypical for this subtype of Castleman's disease.
• Lymphangiectasia observed in the jejunal biopsy may be linked to the underlying Castleman's disease pathology.

Implications:

• This case expands the known clinical spectrum of hyaline vascular Castleman's disease.
• It suggests that gastrointestinal symptoms and associated findings like lymphangiectasia should be considered in the differential diagnosis of unexplained ascites and hepatosplenomegaly, particularly in patients with thrombocytosis or paraproteinemia.
• Further research is warranted to understand the pathogenetic mechanisms linking Castleman's disease to these systemic and gastrointestinal manifestations.