Autor: |
Karan Seegobin, Zhuo Li, Muhamad Alhaj Moustafa, Umair Majeed, Jing Wang, Liuyan Jiang, Justin Kuhlman, David Menke, Ke Li, Mohamed A. Kharfan‐Dabaja, Ernesto Ayala, Madiha Iqbal, Grzegorz S. Nowakowski, Thomas M. Habermann, Thomas E. Witzig, Patrick Johnston, Carrie Thompson, Stephen Ansell, Han W. Tun |
Rok vydání: |
2022 |
Předmět: |
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Zdroj: |
American journal of hematology. 97(9) |
ISSN: |
1096-8652 |
Popis: |
Intravascular lymphoma (IVL) is a rare extranodal non-Hodgkin lymphoma. We performed a retrospective analysis of 55 IVL patients who were treated at our institution 2003-2018. Median age at diagnosis was 68 years, and 64% were males. The most frequent presenting symptoms were skin rash 43% and weight loss 30%. MRI brain on IVL patients with CNS involvement (CNS-IVL) showed multifocal involvement in 76% (13/17). 89% (17/19) of non-CNS-IVL patients with abnormal FDG-PET had biopsy of an avid lesion resulting in definitive diagnosis. The top diagnostic biopsy site was the bone marrow (45%). 56% had multiorgan involvement. Based on CNS involvement, 36.5% (20/55) had CNS-IVL and 63.5% (35/55) had non-CNS-IVL. CNS-IVL group consists of clinically isolated CNS involvement (CNS-only IVL) (22%;12/55) and mixed clinical CNS and peripheral site involvement (M-IVL) (14.5%; 8/55). Non-CNS-IVL group consists of clinically isolated skin involvement (skin-only IVL) (9%; 5/55) and peripheral IVL with or without skin involvement (P-IVL); (54.5%; 30/55). Skin involvement was predominantly in the lower extremities. Pathologically, 89% (48/54) were B-cell IVL. Rituximab + high-dose methotrexate-based regimen were used in 75% (12/16) of CNS-IVL patients and RCHOP in 60% (17/28) of non-CNS-IVL patients. Estimated 5-year progression free survival (PFS) and overall survival (OS) for the entire cohort were 38.6% and 52%, respectively. Skin-only IVL was associated with excellent survival. Platelet count150x10 |
Databáze: |
OpenAIRE |
Externí odkaz: |
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