A case of intra-abdominal multiple lymphangiomas in an adult in whom the immunological evaluation supported the diagnosis.
A 60-year-old patient with intra-abdominal lymphangiomatosis is described. He presented with anaemia due to enteric haemorrhage, hypoproteinaemia with heavy hypogammaglobulinaemia and T-cell lymphopenia. Duodenal biopsy showed lymphangiectasia while a small bowel study revealed several filling defects in the terminal ileum. On exploratory laparotomy, numerous inoperable lymphangio-haemangiomata were found, involving the small and large intestine, appendix, mesenterium, gallbladder and main biliary tract. The importance of T-cell lymphopenia and hypogammaglobulinaemia in the diagnosis of intra-abdominal lymphangiomatosis with lymphangiectasia is stressed.
Amadori
G
G
Department of Clinical and Experimental Medicine, Padua University, Italy. Amadori@Protec.it
Micciolo
R
R
Poletti
A
A
D000008:Abdominal Neoplasms / Q000175:diagnosis* / Q000473:pathology; D000361:Agammaglobulinemia / Q000175:diagnosis*; D000740:Anemia / Q000209:etiology; D001063:Appendiceal Neoplasms / Q000473:pathology; D017809:Fatal Outcome; D005706:Gallbladder Neoplasms / Q000473:pathology; D006471:Gastrointestinal Hemorrhage / Q000209:etiology; D006391:Hemangioma / Q000473:pathology; D006801:Humans; D007019:Hypoproteinemia / Q000209:etiology; D007078:Ileal Neoplasms / Q000473:pathology; D007414:Intestinal Neoplasms / Q000473:pathology; D008200:Lymphangiectasis / Q000209:etiology; D008202:Lymphangioma / Q000175:diagnosis* / Q000473:pathology; D008231:Lymphopenia / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000473:pathology; D010534:Peritoneal Neoplasms / Q000473:pathology; D013601:T-Lymphocytes / Q000473:pathology*