PURPOSE OF REVIEW: The treatment paradigm of chronic lymphocytic leukemia (CLL) has dramatically changed with the advent of novel targeted agents over the past decade. Richter transformation (RT), or the development of an aggressive lymphoma from a background of CLL, is a well-recognized complication of CLL and carries significantly poor clinical outcomes. Here, we provide an update on current diagnostics, prognostication, and contemporary treatment of RT. RECENT FINDINGS: Several genetic, biologic, and laboratory markers have been proposed as candidate risk factors for the development of RT. Although a diagnosis of RT is typically suspected based on clinical and laboratory findings, tissue biopsy is essential for histopathologic confirmation of diagnosis. The standard of care for RT treatment at this time remains chemoimmunotherapy with the goal of proceeding to allogeneic stem cell transplantation in eligible patients. Several newer treatment modalities are being studied for use in the management of RT, including small molecules, immunotherapy, bispecific antibodies, and chimeric antigen receptor T-cell (CAR-T) therapy. The management of patients with RT remains a challenge. Ongoing trials show enormous promise for newer classes of therapy in RT, with the hope being that these agents can synergize, and perhaps supersede, the current standard of care in the near future.
Leukemia, Lymphocytic, Chronic, B-Cell , Lymphoma, Large B-Cell, Diffuse , Humans , Leukemia, Lymphocytic, Chronic, B-Cell/diagnosis , Leukemia, Lymphocytic, Chronic, B-Cell/etiology , Leukemia, Lymphocytic, Chronic, B-Cell/therapy , Lymphoma, Large B-Cell, Diffuse/pathology , Immunotherapy , Biopsy , Cell Transformation, Neoplastic/genetics
We describe a case of a 38-year-old male with a remote history of motor vehicle trauma who presented to the emergency department with 1-week history of progressively worsening abdominal pain localized to the epigastric region. Patient history included splenectomy. Computerized tomography demonstrated multiple masses in the left pleural space as well as masses continuous with the diaphragm and abdominal wall in the left upper quadrant. In addition, a lobulated mass was identified in the right upper quadrant along the anterior right hepatic lobe. A diaphragmatic defect was noted containing splenic tissue. A diagnosis of splenosis was made. Disseminated splenosis presenting in both the thorax and abdomen is rare and poorly documented. This case serves to further illuminate this condition.
