title: [Extramedullary manifestations among adult patients with acute lymphoblastic leukemia (ALL)].
abstract: In 56 adult acute lymphoblastic leukemia (ALL) patients, 71% presented with extramedullary leukemic infiltration in lymphoid tissues (EML- spleen, liver, lymphnodes or thymus). EML was seen most often in patients with T-ALL (0.05 greater than p greater than 0.02) and in patients with high white blood counts (0.1 greater than p greater than 0.05). Surprisingly, these more often achieved complete remissions (0.1 greater than p greater than 0.05), of which both the duration and disease-free survival were longer. Thus, EML at diagnosis seems to be a favourable prognostic factor. At diagnosis, 23% of the patients had extramedullary leukemic infiltration in non-lymphoid tissue (EMIL-CNS, testis, skin, pleural cavities or gingiva). While more of these patients were of T-cell origin (61%, p less than 0.01), they were less likely to achieve CR, both the duration of remissions (p less than 0.01) as well as the disease free survival were shorter. Not unexpectedly, during the course of disease, the incidence of relapse localised at EMIL (the majority presenting in "sanctuary sites") increased, while that in the bone marrow decreased. Interestingly, in patients in CR presenting with EMIL, the first sign of relapse was unilateral peripheral facial paralysis in 60%. Finally, it should be stressed that the course of disease in patients presenting with simultaneous EML- and EMIL involvement was like that seen for EMIL patients. We conclude that while involvement of leukemia in EMIL represents a bad prognostic sign, the affection of leukemia in EML does not seem to confer a poorer prognosis, for adult ALL patients.
authors.affiliation: Medicinsk-haematologisk afdeling 2, Arhus Amtssygehus.
authors.forename: I M
authors.initials: IM
authors.lastname: Jensen
authors.forename: P D
authors.initials: PD
authors.lastname: Jensen
authors.forename: J
authors.initials: J
authors.lastname: Ellegård
authors.forename: P
authors.initials: P
authors.lastname: Bastrup-Madsen
authors.forename: P
authors.initials: P
authors.lastname: Hokland
mesh_terms: D000328:Adult; D002490:Central Nervous System / Q000473:pathology; D005260:Female; D006801:Humans; D008221:Lymphoid Tissue / Q000473:pathology; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D011379:Prognosis; D012867:Skin / Q000473:pathology