Keywords
low grade lymphoma, autoimmune hemolytic anemia, splenic marginal zone lymphoma, BRAF V600E, hairy cell leukemia
How to Cite
Goyanarte, B., Parra, S., Cazap, N., Gotta, D., Altuve, J., Huber, M., García, A., Alsina, M., Avagnina, A., Cuello, M., Denninghoff, V., & Dupont, J. (2019). Autoinmune hemolytic anemia as initial manifestation of low‐grade‐B‐cell lymphoma: a diagnostic and therapeutic challenge. Journal of Hematology, 21(3), 317–323. Retrieved from https://revistahematologia.com.ar/index.php/Revista/article/view/171
Abstract
Hairy cell leukemia (LCV) and splenic marginal zone lymphoma (LME) are two lymphoproliferative disorders that usually have in common a similar clinical presentation with splenomegaly, cytopenias and an indolent clinical course. It is essential to make a proper differential diagnosis, since the therapeutic approach and prognosis differ between both entities. LCV responds to treatment with nucleoside analogs, while LME treatment options include splenectomy or rituximab. The diagnostic approach includes imaging studies, flow cytometry and pathology of bone marrow, although the results of these methods are not always conclusive. We report a clinical case that had an acute and severe presentation, with autoimmune hemolytic anemia and it also implied a diagnostic challenge in which the use of a novel method was helpful to arrive to a definitive diagnosis and an accurate treatment.
References
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13. Iannitto Emilio and Tripodo Claudio. How I diagnose and treat splenic lymphomas. Blood. 2011;117(9):2585-2595.
14. Audouin J, Le Tourneau A, Molina T et al. Patterns of bone marrow involvement in 58 patients presenting primary splenic marginal zone lymphoma with or without circulating villous lymphocytes. Br J Haematol. 2003; 122:404.
15. Salido M, Bar´o C, Oscier D et al. Cytogenetic aberrations and their prognostic value in a series of 330 splenic marginal zone B-cell lymphomas: a multicenter study of the Splenic B-Cell Lymphoma Group. Blood. 2010;116(9): 1479-1488.
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17. Tiacci E, Trifonov V, Schiavoni G et al. BRAF mutations in hairy-cell leukemia. N Engl J Med. 2011; 364 (24): 2305-2315.
18. Turakhia A, Lanigan C et al. Immunohistochemistry for BRAF V600E in the Differential Diagnosis of Hairy Cell Leukemia vs Other Splenic B-Cell Lymphomas. Am J Clin Pathol. July
2015;144:87-93.
19. Dreyling M, Thieblemont C, Gallamini A, et al. ESMO Consensus conferences: guidelines on malignant lymphoma. part 2: marginal zone lymphoma, mantle cell lymphoma, peripheral T-cell
lymphoma. Ann Oncol. 2013;24(4):857-877.
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21. Catovsky D, Matutes E. Splenic lymphoma with circulating villous lymphocytes/splenic marginal zone lymphoma. Semin Hematol. 1999; 36:148. 22. Franco V, Florena AM, Iannitto E. Splenic marginal zone lymphoma. Blood. 2003; 101:2464.
23. Parry Jones N, Matutes E, Gruszka Westwood
AM et al. Prognostic features of splenic lymphoma with villous lymphocytes: a report on 129 patients. Br J Haematol. 2003; 120:759. 24. Lenglet J, Traullé C, Mounier N et al. Long term
follow up analysis of 100 patients with splenic marginal zone lymphoma treated with splenectomy as first line treatment. Leuk Lymphoma. 2014; 55:1854.
25. Xing KH, Kahlon A, Skinnider BF et al. Outcomes in splenic marginal zone lymphoma: analysis of 107 patients treated in British Columbia. Br J Haematol. 2015; 169:520.
26. Kalpadakis C, Pangalis G et al. Treatment of Splenic Marginal Zone Lymphoma With Rituximab Monotherapy: Progress Report and Comparison With Splenectomy. The Oncologist.
2013;18:190-197.
27. Else M, Marine Niebla. Rituximab, used alone or in combination, is superior to other treatment modalities in splenic marginal zone lymphoma. Br J Haematol. 2012 Nov;159(3):322-8.
28. Bennett M, Schechter GP. Treatment of splenic marginal zone lymphoma: splenectomy versus Rituximab. Semin Hematol. 2010 Apr;47(2):143-7.
29. Iannitto E, Luminari S, Tripodo C et al. Rituximab with cyclophosphamide, vincristine, non pegylated liposomal doxorubicin and prednisone as first-line treatment for splenic marginal zone lymphoma: a Fondazione Italiana Linfomi phase II study. Leuk Lymphoma. 2015; 56(12):3281-3287.
30. Arcaini L, Rossi D, Paulli M. Splenic marginal zone lymphoma: from genetics to management. Blood. 2016 Apr 28;127(17):2072-81.
2. Morton LM, Wang SS, Devesa SS, Hartge P, Weisenburger DD, Linet MS. Lymphoma incidence patterns by WHO subtype in the United States, 1992-2001. Blood. 2006;107(1):265.
3. Hoffman R, Benz Jr. E, Silberstein L, Heslop H, Weitz J, Anastasi J. Hematology: principles and practice 6th Ed. Elsevier 2013.
4. Matutes E, Oscier D et al. Splenic marginal zone lymphoma proposals for a revision of diagnostic, staging and therapeutic criteria. Leukemia. 2008; 22, 487-495.
5. Kraut E. Clinical manifestations and infectious complications of hairy-cell leukemia. Best Pract Res Clin Haematol. 2003;16:33-40.
6. Franco V, Florena AM, Iannitto E. Splenic marginal zone lymphoma. Blood. 2003; 101:2464. Declaración de conflictos de interés: Los autores declaran que no poseen conflictos de interés.
7. Mauro FR, Foa R, Cerretti R et al. Autoimmune hemolytic anemia in chronic lymphocytic leukemia: clinical, therapeutic, and prognostic features. Blood. 2000;95:2786-92.
8. Gronbaek K, D’Amore F, Schmidt K. Autoimmune phenomena in non-Hodgkin’s lymphoma. Leuk Lymphoma. 1995;18:311-316.
9. Bennett M, Schechter GP. Treatment of splenic marginal zone lymphoma: splenectomy versus Rituximab. Semin Hematol. 2010 Apr;47(2):143-7.
10. Goodman GR, Burian C, Koziol JA, Saven A. Extended follow-up of patients with hairy-cell leukemia after treatment with cladribine. J Clin Oncol. 2003;21:891-896.
11. Falini B., Martelli, MP., Tiacci E. BRAF V600E mutation in hairy cell leukemia: from bench to bedside. Blood. 2016; 128: 1918-1927
12. Varoczy L, Gergely L, Zeher M, Szegedi G, Illes A. Malignant lymphoma-associated autoimmune diseases-a descriptive epidemiological study. Rheumatol Int. 2002; 22:233-237.
13. Iannitto Emilio and Tripodo Claudio. How I diagnose and treat splenic lymphomas. Blood. 2011;117(9):2585-2595.
14. Audouin J, Le Tourneau A, Molina T et al. Patterns of bone marrow involvement in 58 patients presenting primary splenic marginal zone lymphoma with or without circulating villous lymphocytes. Br J Haematol. 2003; 122:404.
15. Salido M, Bar´o C, Oscier D et al. Cytogenetic aberrations and their prognostic value in a series of 330 splenic marginal zone B-cell lymphomas: a multicenter study of the Splenic B-Cell Lymphoma Group. Blood. 2010;116(9): 1479-1488.
16. Parker et al. Best Practice in Lymphoma Diagnosis and Reporting. Haemotology Guidelines. British Committee for Standards in Haematology Royal College of Pathologists Tiacci E, Trifonov V, Schiavoni G et al. BRAF mutations in hairy-cell leukemia. N Engl J Med. 2011; 364 (24): 2305-2315.
17. Tiacci E, Trifonov V, Schiavoni G et al. BRAF mutations in hairy-cell leukemia. N Engl J Med. 2011; 364 (24): 2305-2315.
18. Turakhia A, Lanigan C et al. Immunohistochemistry for BRAF V600E in the Differential Diagnosis of Hairy Cell Leukemia vs Other Splenic B-Cell Lymphomas. Am J Clin Pathol. July
2015;144:87-93.
19. Dreyling M, Thieblemont C, Gallamini A, et al. ESMO Consensus conferences: guidelines on malignant lymphoma. part 2: marginal zone lymphoma, mantle cell lymphoma, peripheral T-cell
lymphoma. Ann Oncol. 2013;24(4):857-877.
20. Tarella C, Arcaini L, Baldini L, et al. Italian Society of Hematology, Italian Society of Experimental Hematology and Italian Group for Bone Marrow Transplantation guidelines for the management of indolent, nonfollicular B-cell lymphoma (marginal zone, lymphoplasmacytic, and small lymphocytic lymphoma). Clin Lymphoma Myeloma Leuk. 2015;15(2):75-85.
21. Catovsky D, Matutes E. Splenic lymphoma with circulating villous lymphocytes/splenic marginal zone lymphoma. Semin Hematol. 1999; 36:148. 22. Franco V, Florena AM, Iannitto E. Splenic marginal zone lymphoma. Blood. 2003; 101:2464.
23. Parry Jones N, Matutes E, Gruszka Westwood
AM et al. Prognostic features of splenic lymphoma with villous lymphocytes: a report on 129 patients. Br J Haematol. 2003; 120:759. 24. Lenglet J, Traullé C, Mounier N et al. Long term
follow up analysis of 100 patients with splenic marginal zone lymphoma treated with splenectomy as first line treatment. Leuk Lymphoma. 2014; 55:1854.
25. Xing KH, Kahlon A, Skinnider BF et al. Outcomes in splenic marginal zone lymphoma: analysis of 107 patients treated in British Columbia. Br J Haematol. 2015; 169:520.
26. Kalpadakis C, Pangalis G et al. Treatment of Splenic Marginal Zone Lymphoma With Rituximab Monotherapy: Progress Report and Comparison With Splenectomy. The Oncologist.
2013;18:190-197.
27. Else M, Marine Niebla. Rituximab, used alone or in combination, is superior to other treatment modalities in splenic marginal zone lymphoma. Br J Haematol. 2012 Nov;159(3):322-8.
28. Bennett M, Schechter GP. Treatment of splenic marginal zone lymphoma: splenectomy versus Rituximab. Semin Hematol. 2010 Apr;47(2):143-7.
29. Iannitto E, Luminari S, Tripodo C et al. Rituximab with cyclophosphamide, vincristine, non pegylated liposomal doxorubicin and prednisone as first-line treatment for splenic marginal zone lymphoma: a Fondazione Italiana Linfomi phase II study. Leuk Lymphoma. 2015; 56(12):3281-3287.
30. Arcaini L, Rossi D, Paulli M. Splenic marginal zone lymphoma: from genetics to management. Blood. 2016 Apr 28;127(17):2072-81.
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