How we treat Richter syndrome.
نویسندگان
چکیده
Richter syndrome (RS) is defined as the transformation of chronic lymphocytic leukemia (CLL) into an aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL). RS occurs in approximately 2% to 10% of CLL patients during the course of their disease, with a transformation rate of 0.5% to 1% per year. A combination of germline genetic characteristics, clinical features (eg, advanced Rai stage), biologic (ζ-associated protein-70(+), CD38(+), CD49d(+)) and somatic genetic (del17p13.1 or del11q23.1) characteristics of CLL B cells, and certain CLL therapies are associated with higher risk of RS. Recent studies have also identified the crucial role of CDKN2A loss, TP53 disruption, C-MYC activation, and NOTCH1 mutations in the transformation from CLL to RS. An excisional lymph node biopsy is considered the gold standard for diagnosis of RS; a (18)F-fluorodeoxyglucose positron emission tomography scan can help inform the optimal site for biopsy. Approximately 80% of DLBCL cases in patients with CLL are clonally related to the underlying CLL, and the median survival for these patients is approximately 1 year. In contrast, the remaining 20% of patients have a clonally unrelated DLBCL and have a prognosis similar to that of de novo DLBCL. For patients with clonally related DLBCL, induction therapy with either an anthracycline- or platinum-based regimen is the standard approach. Postremission stem cell transplantation should be considered for appropriate patients. This article summarizes our approach to the clinical management of CLL patients who develop RS.
منابع مشابه
A systematic review evaluating the efficacy of autologous hematopoietic transplantation for diffuse large B cell lymphoma-type Richter syndrome
Background: Chronic lymphocytic leukemia (CLL) can transform into fast growing lymphoma for diffuse large B-cell lymphoma (DLBCL) called Richter's syndrome (RS), which is commonly related to an existence of large B-cells with equal or larger size than macrophage nuclei or more than twice those of normal lymphocyte. We conducted a systematic review of the existing literature to assess the clinic...
متن کاملThe Dialectic of Truth and Appearance in Lacan’s Reading of Gerhard Richter’s Overpainted-from-photographs
One of the most important contemporary theoretical approaches that can be used to analyze works of art is the theories of Jacques Lacan (1901-1980), a French post-structuralist psychoanalyst. By combining psychoanalysis, philosophy, linguistics, and anthropology, he analyzes the world of the human subject’s mind in a series of intertwined and extensive cultural and social relationships.This art...
متن کاملP61: Pandard Syndrome, Disorders and Treatments Available: An Overview Article
Pendard syndrome is a genetic disorder that is usually associated with hearing loss in children and thyroid status called goiter, and sometimes affects the balance of the person. Researchers estimate that 7 to 8 percent of the total congenital hearing loss is Pandard's syndrome. A sign that a person may have mutated the SLC26A4 gene is a family history of hearing loss in the early days. Another...
متن کاملModified Simple Decompression of Ulnar Nerve in Treat-ment of Cubital Tunnel Syndrom
BACKGROUND There are two main surgical approaches, simple ulnar nerve decompression at the elbow, and anterior transposition of the nerve in treatment of cubital tunnel syndrome. Both techniques were reported in literature in details with similar reported success rates. Here, we present a modified simple de-compression surgical technique in treatment of cubital tunnel syndrome. METHODS Fifty...
متن کاملNon-Leukemic Granulocytic Sarcoma Presenting with Multiple Lymphadenopathies
3. Parikh SA, Kay NE, Shanafelt TD. How we treat Richter syndrome. Blood 2014;123:1647-1657. 4. Tsimberidou AM, O’Brien S, Khouri I, Giles FJ, Kantarjian HM, Champlin R, Wen S, Do KA, Smith SC, Lerner S, Freireich EJ, Keating MJ. Clinical outcomes and prognostic factors in patients with Richter’s syndrome treated with chemotherapy or chemoimmunotherapy with or without stem-cell transplantation....
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Blood
دوره 123 11 شماره
صفحات -
تاریخ انتشار 2014