Table 1

Characteristics of reported 11 patients with primitive intestinal lymphangiectasia (PIL) and lymphoma

ReferenceSexAge at first symptoms of
PIL (y)
Lymphocyte
counts (μl)
Cutaneous
warts
Time from PIL
to lymphoma onset (y)
LymphomaOutcome
LocationTypeTreatmentLymphomaEnteropathy
Waldman 19724 (3 cases)NSNSNSNS3, 22 and 25Breast,NSNS??
small bowel,NSNS??
stomachNSNS??
Ward 19776 M121000+27JejunumNS (lymphocytic)Surgery, radiotherapyDeath 
Broder 19817 1-a F24600NS15BreastB cell (small cells)CVP1-b Complete remissionImproved
Case record 19848 F57798NS13GI tractB cell (immunoblasts)VCP1-c Death 
Herait 19859 F3860NS15Retroperitoneum, mediastinumNS (large cells)AVmCP1-d Complete remissionUnchanged
Shpilberg 199310 F6640NS13BoneB cell (large cells)CHOP1-e, radiotherapyComplete remissionImproved
Guma 199811 F34NS+20JejunumB cell (large cells)Surgery, CHOPComplete remissionUnchanged
Present case 1F11700+39MidgutB cell (centoblastic)Surgery, AVmCPComplete remissionUnchanged
Present case 2F18990+40IleumB cell (centoblastic)PACOB1-f Complete remissionUnchanged
  • 1-a There is some evidence that this case is one of the initial three cases quoted by Waldman and colleagues4;

  • 1-b cyclophosphamide, vincristine, prednisone;

  • 1-c vincristine, chlorambucil, prednisone;

  • 1-d adriamycin, teniposide, cyclophosphamide, prednisone;

  • 1-e cyclophosphamide, adriamycin, vincristine, prednisone;

  • 1-f prednisolone, adriamycin, cyclophosphamide, vincristine, bleomycin.

  • +, present; NS, not stated; M, male; F, female.