Edit concept Question Editor Create issue ticket

Richter Syndrome


Presentation

  • In the majority of cases, RS presents an activated B cell (ABC) phenotype of diffuse large B-cell lymphoma (DLBCL).[dovepress.com]
  • In the present study, RS karyotypes demonstrated complexity of chromosomal aberrations.[academic.oup.com]
  • RS presents with the same histological aspect as primitive Diffuse Large B-Cell Lymphoma (DLBCL), but is associated with a poor prognosis, due to chemorefractoriness.[clinicaltrials.gov]
  • It is important to note that whilst some of these signs and symptoms may already be present to a lesser degree in people with previously diagnosed CLL/SLL, the possibility of Richter’s Syndrome needs to be considered if there is a sudden and dramatic[leukaemia.org.au]
  • 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.[radiopaedia.org]
Fishing
  • In case 8, FISH confirmed IGH/MYC fusion. In case 6, simultaneous rearrangements of MYC and IGH suggested IGH/MYC fusion, but confirmation by FISH for IGH/MYC was impossible due to lack of a specimen.[academic.oup.com]
  • Unmutated IGHV, high-risk FISH category [del(11q22) or del(17p13)], and positive expression of ZAP-70, CD49d and CD38 were all associated with an increased risk of RS.[ncbi.nlm.nih.gov]
Congestive Heart Failure
  • Clinically significant cardiac disease including unstable angina, uncontrolled congestive heart failure, and unstable arrhythmias requiring therapy, with the exception of extra systoles or minor conduction abnormalities 11.[isrctn.com]
Aspiration
  • Image 1 Fine-needle aspiration biopsy/flow cytometry analysis of case 4.[academic.oup.com]
Palpitations
  • Other signs and symptoms may include low haemoglobin (anaemia) characterised by: increasing fatigue shortness of breath dizziness and possible palpitations low platelets (thrombocytopaenia) characterised by excessive bruising or bleeding an increase in[leukaemia.org.au]
Long Arm
  • In case 1, with IGH rearrangement and two normal chromosomes 14, analysis of FISH metaphases with inverted DAPI banding revealed the insertion of 5′ IGH in the long arm of add(17p) ( Table 3 ) Image 3.[academic.oup.com]
Suggestibility
  • In the second case, we can suggest the presence of IGH/MYC. In the third case, the insertion of IGH occurred at 17q.[academic.oup.com]
  • Still, other studies have suggested that younger CLL patients—that is, younger than 55 years of age—may have an increased risk as well.[verywellhealth.com]
  • The median OS of all RS patients in our study was 2.1 years, but the range of 0.1 – 12.0 years suggests considerable variability.[ncbi.nlm.nih.gov]
Withdrawn
  • This response was maintained during the idelalisib monotherapy, but the patient relapsed rapidly after treatment was withdrawn, because of a grade three immune colitis that developed at 10 months of treatment.[dovepress.com]

Workup

Enlargement of the Spleen
  • Characteristics People with RS develop the aggressive disease with rapidly enlarging lymph nodes, enlargement of the spleen and liver, and elevated levels of a marker in the blood known as serum lactate dehydrogenase, or LDH.[verywellhealth.com]
Colitis
  • This response was maintained during the idelalisib monotherapy, but the patient relapsed rapidly after treatment was withdrawn, because of a grade three immune colitis that developed at 10 months of treatment.[dovepress.com]

Treatment

  • […] service, product or treatment in your circumstances.[leukaemia.org.au]
  • Patients who have received treatment with any non-marketed drug substance or experimental therapy within 4 weeks prior to proposed start of treatment 15. Current participation in any other interventional clinical study 16.[isrctn.com]
  • This response was maintained during the idelalisib monotherapy, but the patient relapsed rapidly after treatment was withdrawn, because of a grade three immune colitis that developed at 10 months of treatment.[dovepress.com]
  • RS occurred prior to CLL treatment in 17 (46%) patients and after treatment of CLL in 20 (54%) patients.[ncbi.nlm.nih.gov]
  • Treatment and Prognosis Treatment of RS usually involves chemotherapy protocols that are commonly used for NHL. These regimens have typically produced overall response rates of about 30 percent.[verywellhealth.com]

Prognosis

  • Treatment and Prognosis Treatment of RS usually involves chemotherapy protocols that are commonly used for NHL. These regimens have typically produced overall response rates of about 30 percent.[verywellhealth.com]
  • [ edit ] The prognosis is generally poor.[en.wikipedia.org]
  • Prognosis is poor, with a mean survival duration of 5-8 months for patients on therapy 8.[radiopaedia.org]
  • RS presents with the same histological aspect as primitive Diffuse Large B-Cell Lymphoma (DLBCL), but is associated with a poor prognosis, due to chemorefractoriness.[clinicaltrials.gov]
  • To determine if the prognosis of newly diagnosed RS could be better predicted, we tested the validity of the MDACC scoring method ( Tsimberidou, et al 2006 ).[ncbi.nlm.nih.gov]

Etiology

  • Etiology The molecular mechanisms involved are poorly understood. Richter transformation may be triggered by viral infections, common in immunosuppressed patients, such as Epstein-Barr infection.[radiopaedia.org]

Epidemiology

  • Epidemiology [ edit ] In the United States, about 500 patients are diagnosed with Richter's transformation each year. [5] References [ edit ] Jain, P; O'Brien, S (Dec 2012). "Richter's transformation in chronic lymphocytic leukemia".[en.wikipedia.org]
Sex distribution
Age distribution

Prevention

  • Whatever the case may be, it does not appear that there is anything you can do to cause or prevent your CLL from transforming. Treatment and Prognosis Treatment of RS usually involves chemotherapy protocols that are commonly used for NHL.[verywellhealth.com]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!