Main Article Content
Chronic lymphocytic leukemia is the most commonly recognized type of leukemia in adults. The appearance of systemic symptoms such as weight loss, fever, or local symptoms in the form of rapidly growing organomegaly, lymphadenopathy in a patient with CLL raises the suspicion of transformation into a high-grade lymphoma – defined as Richter syndrome which is usually associated with very poor prognosis. The described case concerns a 71-year-old patient with this diagnosis, in whom due to the confirmed resistance to subsequent lines of immuno- and chemotherapy, an „emergency” treatment with a modern chemotherapy drug from the aza-anthracendion group – pixantrone was used. Treatment with pixantrone was associated with a relatively good response, translating into partial remission (also in the area of infiltrative changes in the head and neck structures), stabilization of the course of the disease and, consequently, allowed to extend the patient’s life.
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
Copyright: © Medical Education sp. z o.o. This is an Open Access article distributed under the terms of the Attribution-NonCommercial 4.0 International (CC BY-NC 4.0). License (https://creativecommons.org/licenses/by-nc/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
Address reprint requests to: Medical Education, Marcin Kuźma (email@example.com)
2. Brown JR. How I treat CLL patients with ibrutinib. Blood. 2018; 131(4): 379-86.
3. Adamowicz K, Knopińska-Posłuszny W, Mital A et al. Transformacja przewlekłej białaczki limfocytowej w chłoniaka Hodgkina u chorego ze współistniejącym rakiem płuca. Onkol Prak Klin. 2008; 4(3): 110-3.
4. Deptala A, Asendrych A, Staszewska-Skurczynska M. Chemioimmunoterapia na bazie alemtuzumabu jest skuteczna w zespole Richtera i pozwala na uzyskanie długotrwałej regresji choroby. Opis przypadku. Acta Haematologica Polonica. 2009; 40(4): 917-22.
5. Richter MN. Generalized reticular cell sarcoma of lymph nodes associated with lymphocytic leukemia. Am J Pathol. 1928; 4(4): 285-92.
6. Lortholary P, Boiron M, Ripault P et al. Chronic lymphoid leukemia secondary associated with a malignant reticulopathy: Richter’s syndrome. Nouv Rev Fr Hematol. 1964; 78: 621-44.
7. Pocock C, Matutes E, Wotherspoon A et al. Fludarabine therapy may precipitate large cell transformation in chronic lymphocytic leukemia but not in follicular lymphoma. Blood. 1998; 92: 429a (abstract 1774).
8. Tabateau S, Garidi R, Fernandes J et al. Fludarabine (FAMP) and risk of occurrence of Richter’s syndrome among B-CLL. Blood. 2000; 96: 295b (abstract 5019).
9. Tsimberidou AM, O’Brien S, Khouri I et al. Clinical outcomes and prognostic factors in patients with Richter’s syndrome treated with chemotherapy or chemoimmunotherapy with or without stem cell transplantation. J Clin Oncol. 2006; 24: 2343-51.
10. Pettengell R, Coiffier B, Egorov A et al. Long-Term Response and Remission with Pixantrone in Patients with Relapsed or Refractory Aggressive Non-Hodgkin Lymphoma: Post-Hoc Analysis of the Multicenter, Open-Label, Randomized PIX301 Trial. Clin Drug Investig. 2018; 38(6): 527‐33. http://doi.org/10.1007/s40261-018-0635-3.
11. Péan E, Flores B, Hudson I et al. The European Medicines Agency review of pixantrone for the treatment of adult patients with multiply relapsed or refractory aggressive non-Hodgkin’s B-cell lymphomas: summary of the scientific assessment of the committee for medicinal products for human use. Oncologist. 2013; 18(5): 625-33.