Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the ‘cutaneous variant’1
Tóm tắt
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.
Từ khóa
Tài liệu tham khảo
Bhawan J., 1987, Angioendotheliomatosis proliferans systemisata: an angiotropic neoplasm of lymphoid origin, Seminars of Diagnostic Pathology, 4, 18
Carbone P.P., 1971, Report of the Committee on Hodgkin's Disease Staging Classification, Cancer Research, 31, 1860
Chen M., 1998, Angiotropic T cell lymphoma, Chinese Medical Journal (England), 111, 762
Ferreri A.J.M., 2002, Clinical presentation, management and prognosis of intravascular lymphomatosis (IVL): an ongoing clinico‐pathologic study of the I.E.L.S.G, Annals of Oncology, 13
Ferreri A.J.M., 2002, The ‘cutaneous variant’ of intravascular lymphomatosis (IVL) is a favourable clinical form in western countries: a clinico‐pathologic study on 31 cases, Blood, 100
Gatter K.C., 2001, Tumours of Haematopoietic and Lymphoid Tissues, 177
Kuwabara H., 1999, Intravascular lymphomatosis presenting as bilateral adrenal enlargement and insufficiency, Acta Cytologica, 43, 975
Ramus M.W., 1999, Pathologic quiz case. Pathologic diagnosis: intravascular lymphomatosis (angiotropic lymphoma), Archives of Pathology and Laboratory Medicine, 123, 743
Song D.K., 2002, Angiotropic large cell lymphoma with imaging characteristics of CNS vasculitis, AJNR American Journal of Neuroradiology, 23, 239
Takahashi N., 1999, Lymphoma‐associated hemophagocytic syndrome in Japan, Rinsho Ketsueki. Japanese Journal of Clinical Hematology, 40, 542
Takamura K., 1997, Intravascular lymphomatosis diagnosed by transbronchial lung biopsy, European Respiratory Journal, 10, 955, 10.1183/09031936.97.10040955
Wang B.Y., 2001, Renal cell carcinoma with intravascular lymphomatosis: a case report of unusual collision tumors with review of the literature, Archives of Pathology and Laboratory Medicine, 125, 1239, 10.5858/2001-125-1239-RCCWIL