Richter syndrome: chronic lymphocytic leukemia transformation into Hodgkin's disease.
نویسندگان
چکیده
r ichter syndrome is a clinical condition in which indolent chronic lymphocytic leukemia evolves into a more aggressive diffuse large B cell lymphoma. Maurice Nethaniel Richter first described this phenomenon in 1928, which was named the “Richter syndrome” in 1964 [1]. Due to the aggressive nature of the disorder, the clinical manifestations, prognosis and treatment differ from CLL1 [2]. Symptoms will usually include lymphadenopathy, the appearance of “B symptoms” and elevated levels of serum lactate dehydrogenase [2]. The incidence of Richter syndrome originating from CLL to diffuse large B cell lymphoma ranges between 2% and 8% of CLL patients [3]. Although uncommon, approximately 0.4% of CLL patients will experience transformation into Hodgkin’s disease [3]. In the current communication we report on such an occurrence and elaborate on the possible effect of fludarabine therapy on this association.
منابع مشابه
Detection of Hodgkin Transformation in a Case of Chronic Lymphocytic Leukemia by PET/CT
Richter's transformation (RT) represents the development of high grade lymphoma, most commonly diffuse large B-cell lymphoma, in patients with chronic lymphocytic leukemia or small lymphocytic lymphoma (CLL/SLL). CLL/SLL may convert also to Hodgkin's lymphoma, the so-called Hodgkin's variant of Richter transformation. Histopathological proof is needed to confirm a definitive diagnosis. Patients...
متن کاملSecondary CD5+ diffuse large B-cell lymphoma not associated with transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma (Richter syndrome).
Few cases of secondary CD5+ diffuse large B-cell lymphoma (DLBCL) that are not Richter syndrome have been reported previously. We report 9 cases of non-Richter syndrome secondary CD5+ DLBCL. Among 529 cases of DLBCL, 38 (7.2%) were CD5+ DLBCL, including 9 of secondary CD5+ DLBCL. Five cases gained CD5 expression during the clinical course of DLBCL (group 1). Three cases showed transformation fr...
متن کاملRichter transformation of chronic lymphocytic leukemia presenting as a dural-based non-hodgkin lymphoma mass.
Richter transformation is defined as a diffuse large cell lymphoma, occurring by transformation of chronic lymphocytic leukemia (CLL). We present a 64-year-old man with a history of CLL and a left parieto-occipital transtentorial extra-axial mass. The patient underwent CT and MR imaging, demonstrating a large dural-based mass with extracranial extension, which occluded the left transverse sinus...
متن کاملHodgkin's lymphoma variant of Richter's syndrome.
Chronic lymphocytic leukemia/small lymphocitic lymphoma (CLL/SLL) is low-grade malignant lymphoprolipheration, that has tendency to convert to a higher-grade neoplasm over time. More common is the development of a diffuse large cell lymphoma or transformation into prolymphocytic cell population. In rare cases, 0.1-0.5% of patients develop multiple myeloma or Hodgkin's disease. We present 65-yea...
متن کاملA new look at an old syndrome.
In 1928, a pathologist at Bellevue Hospital in New York City named Maurice Richter performed an autopsy on a 46-year-old man. The patient had a 2-month history of abdominal pain and weight loss and was admitted to the hospital, where he died. He was found to have lymphatic leukemia and reticulum cell sarcoma.[1] In 1964, the term “Richter's syndrome” (or “Richter's transformation”) was introduc...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- The Israel Medical Association journal : IMAJ
دوره 15 10 شماره
صفحات -
تاریخ انتشار 2013