Transformation of CLI/ SLL into DLBCL is called-
Ans. is 'a' i.e., Richter syndrome o Richter's transformation (RT, Richter's syndrome) was first described in 1928 by Maurice Richter as the development of an aggressive large-cell lymphoma in the setting of underlying chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). Although diffuse large B cell lymphoma is the most common histology seen in patients with RT, Hodgkin lymphoma and T cell lymphomas have also been reported less commonly. The clinical features, pathogenesis, and treatment of RT will be discussed here.Clinical presentationo Richter's transformation (RT) should be suspected in patients with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) who develop rapidly progressive lymphadenopathy or extranodal sites of disease, systemic symptoms, or elevated levels of serum lactate dehydrogenase.Diagnosiso Biopsy is required to confirm the diagnosis, and usually shows a histologic pattern consistent with diffuse large B cell lymphoma (DLBCL).o Occasional patients may have a histologic picture consistent with Hodgkin lymphoma.Treatmento The prognosis and outcome are historically poor for RT and the disease is invariably fatal if left untreated.o For patients with the DLBCL histologic pattern of RT, the use of combination chemotherapy as employed for aggressive lymphoma, combined with rituximab is recommended (eg, CHOP-R).o Since complete remissions after chemotherapy are short-lived, and long-term survivors have been reported following hematopoietic stem cell transplantation (HCT), the use of either nonmyeloablative allogeneic or autologous HCT when first remission has been achieved in patients who are transplant candidates.o Autologous HCT may be preferred in older patients or those with chemotherapy sensitive disease.o For patients with the Hodgkin lymphoma (HL) the use of combination chemotherapy regimens employed in patients with advanced stage HL is suggested (eg, ABVD)o Given the limited data on treatment outcomes for RT, all patients with RT are best advised to enroll in an appropriately designed clinical trial.
Richter syndrome
Evans syndrome
Li Fraumeni syndrome
Kostmann syndrome
Leukemia