Lethal midline granuloma (polymorphic reticulosis) and lymphomatoid granulomatosis. Evidence for a monoclonal T-cell lymphoproliferative disorder.
Lymphomatoid granulomatosis (LG) and polymorphic reticulosis (PR), originally described as distinct entities, now are considered as a single disease process. Common histopathologic features include necrosis, vasculitis, and a granulomatous infiltrate. Such features have led to consider lymphomatoid granulomatosis as a systemic vasculitis; alternatively the possible emergence of an overt lymphoma has suggested that it could be a lymphoproliferative process. To investigate this later hypothesis, the authors analyzed the cellular infiltrate of tissue specimens from two patients with histologic features of LG. The analysis included the study of T-cell antigen expression and DNA rearrangement of the beta T-cell receptor gene. In one patient, the T-cell phenotype of infiltrating cells was abnormal because of antigen loss. In both patients, the cells contained rearranged DNA indicating the presence of a clonal T-cell proliferation. It is concluded that some cases of LG and PR, if not all, are related to a neoplastic T-cell lymphoproliferative disorder.
Gaulard
P
P
Département de Pathologie Tissulaire et Cellulaire, CHU Henri Mondor, Creteil, France.
Henni
T
T
Marolleau
J P
JP
Haioun
C
C
Henni
Z
Z
Voisin
M C
MC
Divine
M
M
Goossens
M
M
Farcet
J P
JP
Reyes
F
F
D000328:Adult; D000368:Aged; D000941:Antigens / Q000032:analysis*; D004247:DNA / Q000032:analysis; D003937:Diagnosis, Differential; D006103:Granuloma, Lethal Midline / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008230:Lymphomatoid Granulomatosis / Q000473:pathology*; D008297:Male; D009336:Necrosis / Q000473:pathology; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology* / Q000648:ultrastructure; D014657:Vasculitis / Q000473:pathology
D000941:Antigens; D004247:DNA