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Table 1 Clinical characteristics of patients with PIL-associated lympho

From: A case of primary intestinal lymphangiectasia with non-Hodgkin lymphoma

References Sex PIL symptoms Age at PIL onset (years) Age at PIL Dx (years) Lymphoma symptoms Time to lymphoma* (years) Lymphoma site Lymphoma type Stage Treatment Outcome
Strober et al. [15] 22 Small bowel
Waldmann et al. [3] 3 Breast
Waldmann et al. [3] 25 Stomach Reticulum cell sarcoma
Ward et al. [8] M Peripheral edema 12 29 Acute small bowel obstruction 27 Jejunum Differentiated lymphocytic lymphoma I Surgery, radiotherapy Died of septicemia
Border et al. [9] F Cachexia, diarrhea, vomiting anasarca 24 26 Breast mass 15 Breast Small non-cleaved cell (B-cell) lymphoma IV CVP Remission of PIL and lymphoma
Massachusetts Hospital. [10] F Abdominal pain and vomiting 57 57 Dyspnea 13 GI tract, lung, epicardium, mesentery, omentum, pancreas, liver, lymph node, spleen, bone marrow B-immuno-blastic lymphoma (diffuse histiocytic lymphoma) IV VCP Died of acute broncho-pneumonia
Herait et al. [11] F 3 15 Retro-peritoneum, mediastinum Not stated, large cells IV AVmCP Remission of lymphoma, PIL outcome unknown
Shpilberg et al. [12] F Diarrhea, vomiting weight loss 6 6 Soft-tissue mass in left thigh 13 Bone DLBCL IEB CHOP, radiotherapy Remission of PIL and lymphoma
Gumà et al. [5] F Tetany, edema 34 34 Abdominal colic, vomiting 20 Jejunum DLBCL IE Surgery, CHOP Remission of lymphoma, but not PIL
Bouhnik et al. [2] F Diarrhea, steatorrhea, abdominal distension 5 11 Abdominal pain 45 Small intestine B-cell, centroblastic lymphoma I Surgery, AVmCP Remission of lymphoma, but not PIL
Bouhnik et al. [2] F Diarrhea, edema 18 58 Abdominal pain 40 Small intestine B-cell, centroblastic lymphoma I PACOB Remission of lymphoma, but not PIL
Laharie et al. [13] F Diarrhea, edema 20 20 Left cervical lymphadeno-pathy 19 Nodal DLBCL I CHOP, radiotherapy Remission of PIL and lymphoma
Prasad et al. [14] F Diarrhea, anasarca 11 17 Abdominal pain and lump 8 Mesenteric DLBCL IIIA R-CHOP, radiotherapy Remission of PIL and lymphoma
Present case M Abdominal distension 34 54 Abdominal pain 23 Colon DLBCL IVA R-CHOP Remission of lymphoma, but not PIL
  1. PIL primary intestinal lymphangiectasia, Dx diagnosis, CVP cyclophosphamide, vincristine, and prednisone, VCP vincristine, chlorambucil, and prednisone, AVmCP, adriamycin, teniposide, cyclophosphamide, and prednisone, CHOP, cyclophosphamide, adriamycin, vincristine, and prednisone, PACOB prednisolone, adriamycin, cyclophosphamide, vincristine, and bleomycin, R-CHOP rituximab, cyclophosphamide, doxorubicine, vincristine, and prednisone, GI gastrointestinal, DLBCL diffuse large B-cell lymphoma
  2. *Time to lymphoma is expressed in terms of years after the onset of PIL