Richter transformation
Updates to Article Attributes
Richter transformation is defined as a diffuse largedevelopment 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 disease, prolymphocytic leukaemia (PLL), the Hodgkin variant of Richter transformation, small non-cleaved cell lymphoma, occurring by transformation of chronic lymphocytic leukemia (CLL)
Epidemiology
It may beRichter transformation is observed is approximatelyin about 5-10% of patient'spatients with chronic lymphocytic leukaemia 5.
Clinical presentation
Patient's mayPatients present with an aggressive disease course, with rapidly enlarging lymph nodes 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.
Pathology
The nodal disease can potentially occur anywhere in the body. The disease can also present as distinct mass like-like lesions 1.
Extranodal involvement is rare and can involve the gastrointestinal tract, central nervous system, skin, eye, lung, kidney, or testis.
AssociationsAetiology
The molecular mechanisms involved are poorly understood. Richter transformation may be triggered by viral infections, common in immunosuppressed patients, such as Epstein Barr virus-Barr infection:
-
has. Multiple genetic defects have beenshowndescribed that may cause CLL cell proliferation and eventual transformation 8.Diagnosis
Lymph node biopsy is required for the diagnosis of Richter transformation.
Treatment and prognosis
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
toassociated in smal proportiontherapy. Prognosis is poor, with a mean survival duration of 5-8 months for patients on therapy 8.
History and etymology
It was first described by M N Richter in 1928 4.
Differential diagnosis
On pathology the biopsy results can mimic small lymphocytic lymphoma
-<p><strong>Richter transformation</strong> is defined as a diffuse large cell lymphoma, occurring by transformation of <a href="/articles/chronic-lymphocytic-leukaemia">chronic lymphocytic leukemia</a> (CLL).</p><h4>Epidemiology</h4><p>It may be observed is approximately 5-10% of patient's with chronic lymphocytic leukaemia <sup>5</sup>.</p><h4>Clinical presentation</h4><p>Patient's may present with an aggressive disease course with rapidly enlarging lymph nodes, hepatosplenomegaly, and elevated serum lactate dehydrogenase (LDH) levels.</p><h4>Pathology</h4><p>The nodal disease can potentially occur anywhere in the body. The disease can also present as distinct mass like lesions <sup>1</sup>.</p><h5>Associations</h5><p>Epstein Barr virus infection:</p><ul><li>has been shown to associated in smal proportion of patients.</li></ul><h4>History and etymology</h4><p>It was first described by <strong>M N Richter</strong> in 1928 <sup>4</sup>.</p><h4>Differential diagnosis</h4><p>On pathology the biopsy results can mimic <a href="/articles/small-lymphocytic-lymphoma">small lymphocytic lymphoma</a></p>- +<p><strong>Richter transformation</strong> is defined as development of high-grade <a href="/articles/non-hodgkin-lymphoma">non-Hodgkin lymphoma</a> (NHL) in patients with <a href="/articles/chronic-lymphocytic-leukaemia">chronic lymphocytic leukaemia</a> (CLL) or <a href="/articles/small-lymphocytic-lymphoma">small lymphocytic lymphoma</a>.</p><p>It has been expanded to include other lymphoid malignancies that develop in CLL patients, including <a href="/articles/hodgkin-lymphoma">Hodgkin disease</a>, <a href="/articles/t-cell-prolymphocytic-leukaemia">prolymphocytic leukaemia</a> (PLL), the Hodgkin variant of Richter transformation, small non-cleaved cell lymphoma, lymphoblastic lymphoma, and hairy cell leukaemia. <sup>8</sup></p><h4>Epidemiology</h4><p>Richter transformation is observed in about 5-10% of patients with chronic lymphocytic leukaemia <sup>5</sup>. </p><h4>Clinical presentation</h4><p>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.</p><h4>Pathology</h4><p>The nodal disease can potentially occur anywhere in the body. The disease can also present as distinct mass-like lesions <sup>1</sup>.</p><p>Extranodal involvement is rare and can involve the gastrointestinal tract, central nervous system, skin, eye, lung, kidney, or testis.</p><h5>Aetiology</h5><p>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 <sup>8</sup>.</p><h4>Diagnosis</h4><p>Lymph node biopsy is required for the diagnosis of Richter transformation.</p><h4>Treatment and prognosis</h4><p>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.</p><p>The disease responds poorly to therapy. Prognosis is poor, with a mean survival duration of 5-8 months for patients on therapy <sup>8</sup>.</p><h4>History and etymology</h4><p>It was first described by <strong>M N Richter</strong> in 1928 <sup>4</sup>.</p>
References changed:
- 4. Richter MN. Generalized Reticular Cell Sarcoma of Lymph Nodes Associated with Lymphatic Leukemia. Am. J. Pathol. 1928;4 (4): 285-292.7. <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2006994">Free text at pubmed</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/19969796">Pubmed citation</a><span class="auto"></span>
- 8. Tsimberidou AM, Keating MJ. Richter syndrome: biology, incidence, and therapeutic strategies. Cancer. 103 (2): 216-28. <a href="https://doi.org/10.1002/cncr.20773">doi:10.1002/cncr.20773</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/15578683">Pubmed</a> <span class="ref_v4"></span>
- 4. Richter MN. Generalized Reticular Cell Sarcoma of Lymph Nodes Associated with Lymphatic Leukemia. Am. J. Pathol. 2010;4 (4): 285-292.7. <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2006994">Free text at pubmed</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/19969796">Pubmed citation</a><span class="auto"></span>
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