Angiotropic large cell lymphoma (ALCL): morphological, immunohistochemical and genotypic studies with analysis of previous reports.
The entity 'angioendotheliomatosis proliferans systemisata' was first described 28 years ago as a cutaneous small vessel neoplasm of presumed endothelial origin. Since then, 101 similar cases have been reported under a variety of different names, most with systemic as well as cutaneous lesions, and a lymphoid histogenesis of the tumour cell is now favoured. Review of these cases has shown a characteristic clinical presentation with predominant neurological and dermatological features, although the diagnosis was made at autopsy in 53 per cent of patients. Most therapeutic regimens have proved ineffective with a median survival of 5 months from date of clinical presentation. Aggressive combination chemotherapy can produce complete and lasting remission and a partial response to steroids is sometimes seen. We have examined a case of this condition showing unusual clinical features. Immunohistochemical studies confirm the lymphoid origin of the tumour cells with B cell phenotype. Antigen receptor gene rearrangement studies indicate the presence of the same clonal population of B cells in multiple sites. We suggest that the term 'angioendotheliomatosis proliferans systemisata' should be dropped and support the use of 'angiotropic large cell lymphoma' to describe this unusual condition.
Domizio
P
P
Department of Histopathology, St Bartholomew's Hospital, London, U.K.
Hall
P A
PA
Cotter
F
F
Amiel
S
S
Tucker
J
J
Besser
G M
GM
Levison
D A
DA
D001402:B-Lymphocytes / Q000473:pathology; D015322:Gene Rearrangement, B-Lymphocyte; D006801:Humans; D007150:Immunohistochemistry; D007672:Kidney Cortex / Q000098:blood supply / Q000473:pathology; D007680:Kidney Neoplasms / Q000032:analysis / Q000235:genetics / Q000473:pathology; D008228:Lymphoma, Non-Hodgkin / Q000032:analysis / Q000235:genetics / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009360:Neoplastic Cells, Circulating / Q000032:analysis / Q000473:pathology*