Presentation
The usual presentation is sudden clinical deterioration, assymetric adenomegaly, esplenomegaly, fever, weight loss, increased lactate dehydrogenase, and monoclonal gammopathy. [scielo.br]
In the majority of cases, RS presents an activated B cell (ABC) phenotype of diffuse large B-cell lymphoma (DLBCL). [dovepress.com]
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]
Progression was unfavorable and in the following weeks he presented with acute abdomen secondary to extensive mesenteric ischemia. He was operated on again but died in the postoperative period. [revistagastroenterologiamexico.org]
In the present study, RS karyotypes demonstrated complexity of chromosomal aberrations. [academic.oup.com]
Liver, Gall & Pancreas
- Hepatomegaly
Patients present with an aggressive disease course, with rapidly enlarging lymph nodes, hepatomegaly, splenomegaly, and elevated serum lactate dehydrogenase (LDH) levels. Systemic symptoms include weight loss, fever, and drenching night sweats. [radiopaedia.org]
Neurologic
- Dizziness
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]
Workup
Serum
- Neutropenia
Trial of the German CLL Study Group (GCLLSG) Comparing Fludarabine and Cyclophosphamide (FC) to FC Plus Rituximab (FCR) for Previously Untreated Patients with Chronic Lymphocytic Leukemia (CLL): Results On Survival, Progression-Free Survival, Delayed Neutropenias [ncbi.nlm.nih.gov]
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This response was maintained during the idelalisib monotherapy, but the patient relapsed rapidly after treatment was withdrawn, because of a grade three immune colitis that developed at 10 months of treatment.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]
You have to be fairly fit and well to have this treatment as it is very intensive. [cancerresearchuk.org]
Prognosis
[ 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]
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]
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]
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]
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]