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      Diffuse Large B-Cell Lymphoma Relapsing in Leukaemic Phase Presenting as Acute Leukaemia

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          Abstract

          Diffuse large B-cell lymphoma (DLBCL) accounts for 30% to 40% of the newly diagnosed adult non-Hodgkin lymphomas, but rarely presents in leukaemic phase. Here in, we report a case of DLBCL presenting in leukaemic phase and masquerading as acute leukaemia. A 28-year-old woman presented to our outpatient department with complaints of fever for 1 week. Her peripheral blood smear showed 5% to 8% blasts. Bone marrow aspirate showed an infiltration by ~30% blasts. Flow cytometry and immunohistochemistry confirmed relapse of DLBCL. Also, patient’s poor response to therapeutic regimen for DLBCL prompted to consider second differential diagnosis of acute leukaemia. This case is a learning case, as it emphasizes the combined role of diagnostic ancillary techniques along with clinical judgments for management. The case also makes us more vigilant towards the pathobiology of DLBCL and dynamics of personalized individual treatment response.

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          Characteristics and outcomes of diffuse large B-cell lymphoma presenting in leukaemic phase.

          Diffuse large B-cell lymphoma (DLBCL) occasionally presents with circulating malignant cells. The clinical characteristics and long-term outcomes of these patients have not been described. Twenty-nine newly diagnosed DLBCL presenting in leukaemic phase were identified between 1996 and 2010, at two institutions. Median age was 48 years, and patients presented with leucocytosis, high lactate dehydrogenase levels, B symptoms, and high International Prognostic Index score. Extra nodal site involvement was observed in all patients and affected the bone marrow (100%), spleen (62%), pleura/lung (41%), liver (21%), bone (17%), bowels (7%) and cerebrospinal fluid (14%). Blood lymphomatous cells co-expressed CD19, CD20, CD22, CD38, CD45, HLA-DR and FMC7 in >90%, and kappa or lambda light chain restriction in >50%. Ninety per cent received rituximab and anthracycline-based chemotherapy. Overall, remission was complete in 54% and partial in 31%; 15% had resistant disease. Median follow-up was 47 months; 13 (45%) patients remain alive in complete remission. Median progression-free and overall survivals were 11·5 and 46·7 months, respectively. In summary, patients with DLBCL in leukaemic phase present with high tumour burden and frequent involvement of extra nodal sites. In this uncommon DLBCL subgroup, anthracycline-based regimens with rituximab are associated with early morbidity and mortality, but yield approximately 50% 4-year survival.
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            Adhesion molecule profiles of B-cell non-Hodgkin's lymphomas in the leukemic phase.

            We evaluated the expression of 10 adhesion molecules on peripheral blood tumor cells of 17 patients with chronic lymphocytic leukemia, 17 with mantle-cell lymphoma, and 13 with nodal or splenic marginal B-cell lymphoma, all in the leukemic phase and before the beginning of any therapy. The diagnosis of B-cell non-Hodgkin's lymphomas was based on cytological, histological, immunophenotypic, and molecular biology methods. The mean fluorescence intensity of the adhesion molecules in tumor cells was measured by flow cytometry of CD19-positive cells and differed amongst the types of lymphomas. Comparison of chronic lymphocytic leukemia and mantle-cell lymphoma showed that the former presented a higher expression of CD11c and CD49c, and a lower expression of CD11b and CD49d adhesion molecules. Comparison of chronic lymphocytic leukemia and marginal B-cell lymphoma showed that the former presented a higher expression of CD49c and a lower expression of CD11a, CD11b, CD18, CD49d, CD29, and CD54. Finally, comparison of mantle-cell lymphoma and marginal B-cell lymphoma showed that marginal B-cell lymphoma had a higher expression of CD11a, CD11c, CD18, CD29, and CD54. Thus, the CD49c/CD49d pair consistently demonstrated a distinct pattern of expression in chronic lymphocytic leukemia compared with mantle-cell lymphoma and marginal B-cell lymphoma, which could be helpful for the differential diagnosis. Moreover, the distinct profiles of adhesion molecules in these diseases may be responsible for their different capacities to invade the blood stream.
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              Diffuse large B-cell lymphoma in leukemic phase.

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                Author and article information

                Journal
                Clin Med Insights Blood Disord
                Clin Med Insights Blood Disord
                BDX
                spbdx
                Clinical Medicine Insights: Blood Disorders
                SAGE Publications (Sage UK: London, England )
                1179-545X
                15 January 2019
                2019
                : 12
                : 1179545X18821160
                Affiliations
                [1 ]Laboratory Oncology Unit, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, New Delhi, India
                [2 ]Department of Pathology, All India Institute of Medical Sciences, New Delhi, New Delhi, India
                [3 ]Department of Medical Oncology, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, New Delhi, India
                Author notes
                [*]Pranay Tanwar, Laboratory Oncology Unit, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, Room No 425, 4th Floor, New Delhi 110029, India. Email: pranaytanwar@ 123456gmail.com
                Author information
                https://orcid.org/0000-0002-2357-976X
                Article
                10.1177_1179545X18821160
                10.1177/1179545X18821160
                6343450
                d3568c96-6073-4de1-972b-67bebb92af57
                © The Author(s) 2019

                This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License ( http://www.creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages ( https://us.sagepub.com/en-us/nam/open-access-at-sage).

                History
                : 10 October 2018
                : 30 November 2018
                Categories
                Case Report
                Custom metadata
                January-December 2019

                dlbcl relapse,high grade nhl,acute leukemia
                dlbcl relapse, high grade nhl, acute leukemia

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