[Immunoproliferative small intestinal disease (IPSID): IPSID-like lymphomas, IPSID equivalents?, in 12 Spanish patients].
BACKGROUND
Immunoproliferative small intestine disease (IPSID) encompasses a primary intestinal lymphoma of underpriviliged populations of North Africa, Middle East, etc. This epidemiological feature strongly implicates environmental and host (genetic) factors in its pathogenesis. IPSID can be distinguished on clinicopathological grounds from "Western-type" intestinal lymphomas. "IPSID-like" lymphomas had been sporadically identified, i.e., patients with original clinico-analytical data of IPSID (chronic diarrhoea, malabsortion, clubbing of fingers, diffuse intestinal involvement, etc.) without its conventional histopathologic (lymphoplasmocytic or plasmocytic infiltration) and immunological (alpha-heavy-chain paraprotein) background.


OBJECTIVE
The aim of this study has been: 1) to identify, in a series of small intestine lymphomas, a group of patients with a long-lasting history of chronic diarrhoea and a clinico-biologic pattern of "IPSID-like" lymphoma; 2) to analyze its clinicopathological profile; 3) to search for differences with the pattern of the remaining cases ("Western-type" lymphomas) and 4) To suggest a possible epidemiological significance.


METHODS
Patients considered were 12 Spanish caucasians with primary intestinal lymphoma and a long-lasting history of chronic diarrhoea vs 31 cases of "Western-type" intestinal lymphomas admitted in our Hospital over a 33-year period. Statistical significance of differences in clinico-biological features (symptoms/signs, analytical data, patterns of involvement, histopathology, immunophenotype and tumor staging) between these two groups was evaluated using X2 test.


RESULTS
The results of this retrospective study allow us to delineate a relatively homogeneous "IPSID-like" group (12 cases) among 43 cases of primary small intestine lymphoma diagnosed between 1960 and 1993. The clinico-pathological behavior of these patients was significantly different from that exhibited by the 31 cases of so-called "Western-type" lymphomas.


CONCLUSIONS
It is suggested that they may represent a group of patients suffering an evanescent "IPSID-equivalent" disorder (last case diagnosed in 1975), that probably has evolved in similar but not identical epidemiological circumstances to those present in the "Third World" countries of our Mediterranean area.
Sánchez-Fayos Calabuig
P
P
Servicio de Aparato Digestivo, Fundación Jiménez Díaz, Madrid.
Hernández Guío
C
C
Porres Cubero
J C
JC
Olavarría López-Aróstegui
E
E
Rivas Manga
C
C
Outeiriño Hernanz
J
J
Sánchez Fayos
J
J
D000293:Adolescent; D000328:Adult; D000368:Aged; D002675:Child, Preschool; D002908:Chronic Disease; D003906:Developing Countries; D003937:Diagnosis, Differential; D003967:Diarrhea / Q000209:etiology; D004198:Disease Susceptibility / Q000208:ethnology; D004777:Environment; D005260:Female; D006680:HLA Antigens / Q000032:analysis; D006801:Humans; D007161:Immunoproliferative Small Intestinal Disease / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology*; D007414:Intestinal Neoplasms / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology*; D018442:Lymphoma, B-Cell, Marginal Zone / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology; D008228:Lymphoma, Non-Hodgkin / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology*; D008297:Male; D019083:Mediterranean Region / Q000453:epidemiology; D008875:Middle Aged; D012189:Retrospective Studies; D012959:Socioeconomic Factors; D013030:Spain / Q000453:epidemiology; D044465:White People
D006680:HLA Antigens