Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the 'cutaneous variant'.
Despite its recognition as a distinct, extremely rare entity, no large studies of intravascular lymphoma (IVL) have been reported. The clinico-pathological characteristics of 38 human immunodeficiency virus-negative patients with IVL diagnosed in Western countries were reviewed to better delineate clinical presentation, clinical variants, natural history and optimal therapy. The IVL is an aggressive and usually disseminated disease (Ann Arbor stage IV in 68% of cases) that predominantly affects elderly patients (median age 70 years, range: 34-90; male:female ratio 0.9), resulting in poor Eastern Cooperative Oncology Group Performance Status (ECOG-PS >1 in 61%), B symptoms (55%), anaemia (63%) and high serum lactate dehydrogenase level (86%). The brain and skin are the most common sites of disease. In contrast to previous reports, hepatosplenic involvement (26%) and bone marrow infiltration (32%) were found to be common features in IVL, while nodal disease was confirmed as rare (11% of cases). Patients with disease limited to the skin ('cutaneous variant'; 26% of cases) were invariably females with a normal platelet count, and exhibited a significantly better outcome than the remaining patients, which deserves further investigation. Overall survival was usually poor; however, the early use of intensive therapies could improve outcome in young patients with unfavourable features. ECOG-PS >1, 'cutaneous variant', stage I and chemotherapy use were independently associated with improved survival.
Ferreri
Andrés J M
AJ
Department of Radiochemotherapy, San Raffaele H Scientific Institute, Milan, Italy.
Campo
Elías
E
Seymour
John F
JF
Willemze
Rein
R
Ilariucci
Fiorella
F
Ambrosetti
Achille
A
Zucca
Emanuele
E
Rossi
Giuseppe
G
López-Guillermo
Armando
A
Pavlovsky
Miguel A
MA
Geerts
Marie-Louise
ML
Candoni
Anna
A
Lestani
Maurizio
M
Asioli
Silvia
S
Milani
Mario
M
Piris
Miguel A
MA
Pileri
Stefano
S
Facchetti
Fabio
F
Cavalli
Franco
F
Ponzoni
Maurilio
M
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D005260:Female; D018023:HIV Seronegativity; D006801:Humans; D008223:Lymphoma / Q000188:drug therapy / Q000401:mortality / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D009378:Neoplasms, Multiple Primary / Q000188:drug therapy / Q000401:mortality / Q000473:pathology; D011379:Prognosis; D012878:Skin Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000473:pathology; D016019:Survival Analysis; D019043:Vascular Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000473:pathology