Richter syndrome

Richter syndrome (RS), also known as Richter's transformation, is a rare and aggressive transformation of chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL) into a fast-growing type of non-Hodgkin lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL). This transformation is named after the German pathologist Maurice Richter, who first described the condition in 1928.

Pathophysiology

Richter syndrome occurs when the slow-growing CLL/SLL cells transform into a more aggressive form of lymphoma. This transformation is characterized by a rapid increase in the size of lymph nodes, spleen, or other organs, and a sudden worsening of symptoms. The exact mechanisms behind this transformation are not fully understood, but genetic mutations and changes in the tumor microenvironment are believed to play significant roles.

Symptoms

The symptoms of Richter syndrome can vary but often include:

• Rapidly enlarging lymph nodes
• Fever
• Night sweats
• Weight loss
• Fatigue
• Abdominal pain or discomfort

These symptoms are more severe and progress more quickly than those typically seen in CLL/SLL.

Diagnosis

Diagnosis of Richter syndrome involves a combination of clinical evaluation, imaging studies, and biopsy. Key diagnostic tools include:

• Computed tomography (CT) scans
• Positron emission tomography (PET) scans
• Lymph node biopsy
• Bone marrow biopsy

Histological examination of the biopsy samples is crucial to distinguish Richter syndrome from other types of lymphoma and to confirm the transformation from CLL/SLL to DLBCL or another aggressive lymphoma.

Treatment

Treatment options for Richter syndrome are more limited and less effective than those for CLL/SLL. Common treatment approaches include:

• Chemotherapy
• Immunotherapy
• Targeted therapy
• Stem cell transplantation

The choice of treatment depends on various factors, including the patient's overall health, the extent of the disease, and previous treatments for CLL/SLL.

Prognosis

The prognosis for patients with Richter syndrome is generally poor, with a median survival of less than one year after diagnosis. However, outcomes can vary based on individual patient factors and response to treatment.

Related Pages

• Chronic lymphocytic leukemia
• Small lymphocytic lymphoma
• Diffuse large B-cell lymphoma
• Non-Hodgkin lymphoma
• Lymph node biopsy
• Bone marrow biopsy
• Chemotherapy
• Immunotherapy
• Targeted therapy
• Stem cell transplantation