Richter transformation

Richter transformation is defined as development of high-grade non-Hodgkin lymphoma (NHL) in patients with chronic lymphocytic leukaemia (CLL) or small lymphocytic lymphoma.

It has been expanded to include other lymphoid malignancies that develop in CLL patients, including Hodgkin diseaseprolymphocytic leukaemia (PLL), the Hodgkin variant of Richter transformation, small non-cleaved cell lymphoma, lymphoblastic lymphoma, and hairy cell leukaemia 8. Lymph node biopsy is required for the diagnosis of Richter transformation.

Richter transformation is observed in about 5-10% of patients with chronic lymphocytic leukaemia 5

Patients present with an aggressive disease course, with rapidly enlarging lymph nodes, hepatosplenomegaly, and elevated serum lactate dehydrogenase (LDH) levels. Systemic symptoms include weight loss, fever, and drenching night sweats. Abdominal pain may be a consequence of further hepatic and/or splenic enlargement.

The nodal disease can potentially occur anywhere in the body. The disease can also present as distinct mass-like lesions 1.

Extranodal involvement is rare and can involve the gastrointestinal tract, central nervous system, skin, eye, lung, kidney, or testis.

Aetiology

The molecular mechanisms involved are poorly understood. Richter transformation may be triggered by viral infections, common in immunosuppressed patients, such as Epstein-Barr infection. Multiple genetic defects have been described that may cause CLL cell proliferation and eventual transformation 8.

CT

Development of lymphadenopathy and splenomegaly in a context of known CLL.

Nuclear medicine

PET-CT may play a role and nodes show high FDG uptake  3,9

Treatment strategies include chemotherapy regimens given for aggressive lymphoma or acute lymphoblastic leukaemia (ALL), with or without monoclonal antibodies, allogeneic stem cell transplantation, and, rarely, radiotherapy.

The disease responds poorly to therapy. Prognosis is poor, with a mean survival duration of 5-8 months for patients on therapy 8.

It was first described by M N Richter in 1928 4.

Share article

Article information

rID: 39619
Systems: Chest, Haematology
Synonyms or Alternate Spellings:
  • Richter transformation of chronic lymphocytic leukaemia
  • Richter tranformation of CLL
  • Richter's syndrome
  • Richter syndrome
  • Richter's tranformation
  • Richter's tranformation of CLL
  • Richter's transformation of chronic lymphocytic leukaemia
  • Richter syndrome (RS)

Support Radiopaedia and see fewer ads

Cases and figures

  • Drag
    Case 1
    Drag here to reorder.
  • Updating… Please wait.
    Loadinganimation

    Alert accept

    Error Unable to process the form. Check for errors and try again.

    Alert accept Thank you for updating your details.