Enfermedad de Castleman multicéntrica en sida. Su relación con el VHH-8 o virus herpes asociadoal sarcoma de Kaposi. Estudio de dos casos / Multicenter Castleman disease in AIDS. Its relationship with HHV-8 or hespes virus associated to Kaposi's sarcoma. Study of two cases

La enfermedad de Castleman está considerada como un cuadro linfadenopático reactivo con dos formas clínicas: una localizada, frecuente en pacientes inmunocompetentes, y otra multicéntrica, más característica en los enfermos inmunodeprimidos. Se presentan dos casos de enfermedad de Castleman multicéntrica en pacientes VIH positivos con sarcoma de Kaposi. Ambos pacientes muestran adenopatías múltiples, hepatomegalia y síntomas B al diagnóstico. Uno de ellos presenta respuesta favorable al tratamiento quimioterápico y el otro fallece. Se realiza una revisión del concepto de enfermedad de Castleman multicéntrica, así como su relación patogénica con el virus herpes humano-8

Castleman disease is considered a reactive lymphadenopathic picture with two clinical forms: one localized, frequent in immunocompetent patients and another multicenter one that is more characteristic in immunodepressed patients. Two cases of Castleman disease multicenter in HIV positive patients with Kaposi's sarcoma are presented. Both patients have multiple adenopathies, hepatomegaly and symptoms B on diagnosis. One of them had a favorable response to chemotherapy treatment and another died. A review of the concept of multicenter Castleman disease and its pathogenic relationship to human herpes virus 8 (HHV-8) is done

[Multicenter Castleman disease in AIDS. Its relationship with HHV-8 or herpes virus associated to Kaposi's sarcoma. Study of two cases]. / Enfermedad de Castleman multicéntrica en sida. Su relación con el VHH-8 o virus herpes asociado al sarcoma de Kaposi. Estudio de dos casos.

Castleman disease is considered a reactive lymphadenopathic picture with two clinical forms: one localized, frequent in immunocompetent patients and another multicenter one that is more characteristic in immunodepressed patients. Two cases of Castleman disease multicenter in HIV positive patients with Kaposi's sarcoma are presented. Both patients have multiple adenopathies, hepatomegaly and symptoms B on diagnosis. One of them had a favorable response to chemotherapy treatment and another died. A review of the concept of multicenter Castleman disease and its pathogenic relationship to human herpes virus 8 (HHV-8) is done.

[Immunoproliferative small intestinal disease (IPSID): IPSID-like lymphomas, IPSID equivalents?, in 12 Spanish patients]. / Enfermedad inmunoproliferativa del intestino delgado (EIPID): linfomas "EIPID-símiles"--EIPID-equivalentes?--en doce pacientes españoles.

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. PURPOSE: 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. PATIENTS AND 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.

[Adult T-cell lymphoblastic lymphoma associated with quiescent multiple myeloma]. / Linfoma linfoblástico T del adulto asociado a mieloma múltiple quiescente.

We report the case history of a female patient suffering of a T lymphoblastic lymphoma with an atypical presentation and evolution, consisting of pleuropericardiac effusion without mediastinic masses that coexists with a plasma cell dyscrasia that fulfils the criteria for stationary multiple myeloma. The possible pathogenic relationship between both processes is discussed, emphasizing the functional evolutive dissociation between the cellular constituents of both neoplasias, immature T lymphoblasts and plasma cells, respectively; this constitutes the first reported case in literature.