Macroglobulinaemia and intestinal lymphangiectasia: a rare association

Abstract

Two cases in which macroglobulinaemia was associated with intestinal lymphangiectasia are recorded. Immunoperoxidase stains demonstrated a high content of monoclonal IgM in the intestinal lymph. The seven previously recorded examples of this association are reviewed. It is concluded that the concurrence of these two conditions is not merely fortuitous, and that increased viscosity of the lymph consequent on its high IgM content may be important in the pathogenesis of the intestinal lymphangiectasia. Since the original description of macroglobulinaemia' a small number of cases has been recorded in which the macroglobulinaemia was associated with intestinal lymphangiectasia (IL). We wish to record two further examples of this association and to present observations on the pathogenesis of the intestinal changes. Case reports CASE An 89-year-old woman was admitted to another hospital in May 1977 when she was found to have anaemia and splenomegaly. Her haemoglobin was 6-1 g/dl, WCC 5 6 x 109/1 (neutrophils 60%, lymphocytes 34%); the bone marrow contained 20% lymphocytes. She was treated with blood transfusion alone. After this admission she became increasingly weak and lost 4 stones (25 4 kg) in weight. In January 1978 she developed vomiting 1-2 hours after meals and there was intermittent diarrhoea with pale, loose stools being passed 5-6 times daily. She had complained of abdominal distension and feeling bloated for many years. She was admitted to Withington Hospital in February 1978. On examination she was pale with a malar flush. The liver and spleen were enlarged to 4 cm and 13 cm below the costal margins respectively and the abdomen was diffusely tender. There was no palpable lymphadenopathy. An aortic systolic murmur was heard and there was moderate ankle, sacral and abdominal wall oedema. The jugular venous pressure was normal.

Multiple Myeloma Presenting as Chronic Diarrhea

“… 5 , 6 This diffuse intestinal lymphangiectasia has been associated with protein-losing enteropathy and malabsorption in several reports. 5 , 6 …”

“…Case reports have highlighted the role of macroglobulin deposition, typically seen with WM, in causing increased interstitial viscosity and thus the development of secondary lymphangiectasia. 5,6 This diffuse intestinal lymphangiectasia has been associated with protein-losing enteropathy and malabsorption in several reports. 5,6 In the presence of a known plasma cell neoplasm, unexplained GI symptoms could be a result of coexisting amyloidosis.…”

“…5,6 This diffuse intestinal lymphangiectasia has been associated with protein-losing enteropathy and malabsorption in several reports. 5,6 In the presence of a known plasma cell neoplasm, unexplained GI symptoms could be a result of coexisting amyloidosis. 7 Over 10% of patients with MM have concomitant amyloidosis.…”