images in haematology
€ m macroglobulinaemia and intestinal Waldenstro lymphangiectasia
A 51-year-old man presented with lethargy, weight loss and 3 weeks of watery diarrhoea. He was underweight (body mass index, 197 kg/m2). Haemoglobin concentration was 87 g/l, reticulocytes 95 9 109/l, white cells 47 9 109/l, neutrophils 24 9 109/l, lymphocytes 19 9 109/l and platelets 93 9 109/l. The blood film showed occasional plasmacytoid lymphocytes. Erythrocyte sedimentation rate (ESR) was >140 mm in 1 h. Renal, liver and thyroid function tests and lactate dehydrogenase were normal. There was a serum monoclonal paraprotein, immunoglobulin (Ig) M kappa, of 17 g/l but no Bence-Jones proteinuria, and skeletal survey demonstrated no lytic lesions. There was lymphocytosis in the bone marrow, the lymphocytes being predominantly small cells with plasmacytoid features. These monoclonal B cells were kappa light chain-restricted and had the immunophenotype: CD19+, CD20+, CD22 weak+, CD23 variable, CD138+, HLA-DR+, l heavy chain+, and negative for CD5, CD10, CD38 and Cyclin D1. A computed tomography (CT) scan of neck, chest, abdomen and pelvis was unremarkable apart from several thick walled loops of small bowel (left, CT with contrast). Gastroscopy and colonoscopy demonstrated no macroscopic abnormality. Random biopsies of the terminal ileum showed lymphangiectasia – there was dilation and expansion of the villi due to aggregation of eosinophilic proteinaceous material within lacteals (right, Haematoxylin and Eosin). This material was periodic acid-Schiff-positive and Congo red-negative. Additionally, it stained positively for IgM and kappa light chains. Colonic biopsies showed mild patchy colitis only. A diagnosis of intestinal lymphangiectasia
First published online 21 July 2014 doi: 10.1111/bjh.13041
associated with Waldenstr€ om macroglobulinaemia was made. The patient was treated with cyclophosphamide, vincristine and prednisolone (CVP), and the diarrhoeal symptoms subsided after only one cycle. He completed six cycles of CVP (rituximab was added to the last two cycles) with the serum paraprotein falling to 5 g/l, and he gained 8 kg in weight. Treatment of the underlying lymphoma appeared to ameliorate symptoms from intestinal lymphangiectasia. Lymphangiectasia is rare and has been estimated to be present in 13% of cases of Waldenstr€ om macroglobulinaemia. Most patients with intestinal lymphangiectasia have had unexplained diarrhoea, some for up to 5 years before diagnosis. The hyaline material in the acellular intestinal deposits is IgM admixed with phospholipid; it is largely confined to villi tips where it is almost entirely extracellular, and accumulates in the interstitium and lacteals. Lymphangiectasia can occur even with modest elevations of IgM, suggesting that it is not only the concentration of IgM that is important, but also the physicochemical properties of the immunoglobulin. It has been postulated that the IgM deposition leads to increased viscosity within lymphatic channels (which normally clear IgM from the intestine) resulting in lymphatic stasis with eventual obstruction and dilation. Subsequent lymphangiectasia results in diarrhoea and malabsorption. Rachel E. Cooke1, Renate M. Kalnins2 and Wai Khoon Ho1 1
Department of Haematology, Austin Health, and 2Department of
Anatomical Pathology, Austin Health, Melbourne, Australia. E-mail: [email protected]
ª 2014 John Wiley & Sons Ltd British Journal of Haematology, 2014, 167, 292
€ m macroglobulinaemia and intestinal Waldenstro lymphangiectasia
A 51-year-old man presented with lethargy, weight loss and 3 weeks of watery diarrhoea. He was underweight (body mass index, 197 kg/m2). Haemoglobin concentration was 87 g/l, reticulocytes 95 9 109/l, white cells 47 9 109/l, neutrophils 24 9 109/l, lymphocytes 19 9 109/l and platelets 93 9 109/l. The blood film showed occasional plasmacytoid lymphocytes. Erythrocyte sedimentation rate (ESR) was >140 mm in 1 h. Renal, liver and thyroid function tests and lactate dehydrogenase were normal. There was a serum monoclonal paraprotein, immunoglobulin (Ig) M kappa, of 17 g/l but no Bence-Jones proteinuria, and skeletal survey demonstrated no lytic lesions. There was lymphocytosis in the bone marrow, the lymphocytes being predominantly small cells with plasmacytoid features. These monoclonal B cells were kappa light chain-restricted and had the immunophenotype: CD19+, CD20+, CD22 weak+, CD23 variable, CD138+, HLA-DR+, l heavy chain+, and negative for CD5, CD10, CD38 and Cyclin D1. A computed tomography (CT) scan of neck, chest, abdomen and pelvis was unremarkable apart from several thick walled loops of small bowel (left, CT with contrast). Gastroscopy and colonoscopy demonstrated no macroscopic abnormality. Random biopsies of the terminal ileum showed lymphangiectasia – there was dilation and expansion of the villi due to aggregation of eosinophilic proteinaceous material within lacteals (right, Haematoxylin and Eosin). This material was periodic acid-Schiff-positive and Congo red-negative. Additionally, it stained positively for IgM and kappa light chains. Colonic biopsies showed mild patchy colitis only. A diagnosis of intestinal lymphangiectasia
First published online 21 July 2014 doi: 10.1111/bjh.13041
associated with Waldenstr€ om macroglobulinaemia was made. The patient was treated with cyclophosphamide, vincristine and prednisolone (CVP), and the diarrhoeal symptoms subsided after only one cycle. He completed six cycles of CVP (rituximab was added to the last two cycles) with the serum paraprotein falling to 5 g/l, and he gained 8 kg in weight. Treatment of the underlying lymphoma appeared to ameliorate symptoms from intestinal lymphangiectasia. Lymphangiectasia is rare and has been estimated to be present in 13% of cases of Waldenstr€ om macroglobulinaemia. Most patients with intestinal lymphangiectasia have had unexplained diarrhoea, some for up to 5 years before diagnosis. The hyaline material in the acellular intestinal deposits is IgM admixed with phospholipid; it is largely confined to villi tips where it is almost entirely extracellular, and accumulates in the interstitium and lacteals. Lymphangiectasia can occur even with modest elevations of IgM, suggesting that it is not only the concentration of IgM that is important, but also the physicochemical properties of the immunoglobulin. It has been postulated that the IgM deposition leads to increased viscosity within lymphatic channels (which normally clear IgM from the intestine) resulting in lymphatic stasis with eventual obstruction and dilation. Subsequent lymphangiectasia results in diarrhoea and malabsorption. Rachel E. Cooke1, Renate M. Kalnins2 and Wai Khoon Ho1 1
Department of Haematology, Austin Health, and 2Department of
Anatomical Pathology, Austin Health, Melbourne, Australia. E-mail: [email protected]
ª 2014 John Wiley & Sons Ltd British Journal of Haematology, 2014, 167, 292
