| 词条 | Richter's transformation |
| 释义 |
Richter's transformation affects about 5% of CLL patients at some point during their lives.[4] Signs and symptomsSymptoms of Richter’s transformation in a CLL patient include fever (without infection), elevated serum levels of lactate dehydrogenase, and rapidly enlarging lymph nodes.[5] While about 8% of all CLL patients will have elevated levels of serum lactate dehydrogenase (LDH), more than 50% of CLL patients with Richter's transformation will have elevated LDH levels. Richter's can appear suddenly, even in patients who were in remission. CauseA case of RS may have arisen by one of two different routes: a transformation of the CLL cells into lymphoma, or the appearance of an unrelated lymphoma.[4] It is thought that genetic defects may introduce the additional abnormalities necessary to transform CLL cells into Richter's syndrome cells.[5] TreatmentTreatment with conventional immunochemotherapy is usually indicated; in younger patients, allogeneic bone marrow transplantation may be curative.[4] PrognosisThe prognosis is generally poor. The RS score (Richter syndrome score), which is an estimate of the patient's prognosis, is based on the patient's performance status, LDH, platelet count, the size of the lymphoma tumors, and the number of prior therapies already received.[4] Overall, the median survival is between five and eight months.[6] Untreated, RS is invariably fatal. The Hodgkin's lymphoma variant of Richter's carries a better prognosis than the predominant diffuse large B-cell lymphoma type, but a worse prognosis than a de novo case of Hodgkin's. EpidemiologyIn the United States, about 500 patients are diagnosed with Richter's transformation each year.[5] References1. ^{{cite journal|last1=Jain|first1=P|last2=O'Brien|first2=S|title=Richter's transformation in chronic lymphocytic leukemia.|journal=Oncology (Williston Park, N.Y.)|date=Dec 2012|volume=26|issue=12|pages=1146–52|pmid=23413591}} 2. ^{{cite web|title=NCI Dictionary of Cancer Terms: R|url=http://www.cancer.gov/dictionary?expand=R|work=National Cancer Institute|publisher=NIH|accessdate=4 January 2013}} 3. ^{{cite journal|last1=Hatoum|first1=Hassan A.|last2=Mahfouz|first2=Rami A.R.|last3=Otrock|first3=Zaher K.|last4=Hudaib|first4=Abdel-Rahman|last5=Taher|first5=Ali T.|last6=Shamseddine|first6=Ali I.|title=Acute myeloid leukemia with T-cell receptor gamma gene rearrangement occurring in a patient with chronic lymphocytic leukemia: A case report|journal=American Journal of Hematology|volume=82|issue=1|year=2007|pages=69–72|issn=0361-8609|doi=10.1002/ajh.20762}} 4. ^1 2 3 {{cite journal |vauthors=Rossi D, Gaidano G |title=Richter syndrome: molecular insights and clinical perspectives |journal=Hematol Oncol |volume=27 |issue=1 |pages=1–10 |date=March 2009 |pmid=19206112 |doi=10.1002/hon.880 |url=}} 5. ^1 2 {{cite journal|last=Tsimberidou|first=Apostolia-Maria|author2=Keating, Michael J. |author3=Wierda, William G. |title=Richter’s transformation in chronic lymphocytic leukemia|journal=Current Hematologic Malignancy Reports|date=October 2007|volume=2|issue=4|pages=265–271|doi=10.1007/s11899-007-0036-9|pmid=20425379}} 6. ^{{cite journal|last=Swords|first=R|author2=Bruzzi, J |author3=Giles, F |title=Recent advances in the diagnosis and therapy of Richter's syndrome.|journal=Medical oncology (Northwood, London, England)|year=2007|volume=24|issue=1|pages=17–32|pmid=17673808}} Further reading
2 : Leukemia|Rare cancers |
| 随便看 |
|
开放百科全书收录14589846条英语、德语、日语等多语种百科知识，基本涵盖了大多数领域的百科知识，是一部内容自由、开放的电子版国际百科全书。