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 |