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Intravascular large B cell lymphoma: proposed of the strategy for early diagnosis and treatment of patients with rapid deteriorating condition

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Abstract

We summarize our experience and propose methods for early diagnosis and treatment of intravascular large B cell lymphoma (IVL). A total of 16 patients with IVL between 1994 and 2007 were included and analyzed in this study. Predicted survival durations were short until September 2003. However, there have been marked improvement since the introduction of rituximab, and all patients responded to treatment and survived for more than 1 year following diagnosis of IVL. We propose an early clinical diagnostic strategy for starting treatment for IVL patients with quite poor performance status (PS) and in whom time is a limiting factor: (1) age >40 years, (2) fever above 38°C with poor PS (ECOG 2-4), (3) lactate dehydrogenase (LDH) more than twice the upper limit of the normal level and/or sIL2R >5,000 IU/ml in serum, (4) worsening PS and/or elevation of serum LDH on a daily basis, and (5) confirmation of pathological lymphoid cells in peripheral blood or bone marrow smear and/or flow cytometry. Although accurate pathological diagnosis is quite important, time is a limiting factor for most of IVL patients. In such cases, we can start chemotherapy based on early clinical diagnostic strategy with high sensitivity and obtain good clinical outcome.

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Acknowledgments

We are grateful to the members of the IVL study group of Japan and the members of Hokuriku Hematological Tumor Research Group for critical discussion. This work was supported by grants from the Japanese Ministry of Education, Culture, Sports, Science and Technology (13557160, 15024236, 15390313, 13877075, to H. Umehara and 17591060 to Y. Masaki), Uehara Memorial Foundation (to H. Umehara) and Kanazawa Medical University Research Foundation (C2006-1 to H. Umehara and S2004-16, S2007-5 to Y. Masaki).

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Correspondence to Yasufumi Masaki.

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Masaki, Y., Dong, L., Nakajima, A. et al. Intravascular large B cell lymphoma: proposed of the strategy for early diagnosis and treatment of patients with rapid deteriorating condition. Int J Hematol 89, 600–610 (2009). https://doi.org/10.1007/s12185-009-0304-7

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