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Richter's Syndrome manifested as diffuse large B-cell lymphoma of the mandible with lytic lesions and hypercalcemic crisis
Affiliations- Students’ Research Group, Department of Hematology and Bone Marrow Transplantation, Silesian Medical University, Katowice, Poland
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Abstract
Richter's Syndrome (RS) is a transformation from chronic lymphocytic leukemia (CLL) into more aggressive lymphoma. RS occurs in about 5% of patients with CLL and its clinical outcome is poor. Extranodal involvements of RS may be present in up to 40% of patients and central nervous system manifestation was found to be the most common. Mandibular localization of RS has not been reported so far. There is no established standard treatment for RS. The improvement of survival in RS patients is achievable with intensive anti-lymphoma chemotherapy and subsequent allogeneic stem cell transplantation (alloHSCT) performed in complete remission. Herein we report a female with RS of the right mandible and hypercalcemia was its first clinical manifestation.
Abstract
Richter's Syndrome (RS) is a transformation from chronic lymphocytic leukemia (CLL) into more aggressive lymphoma. RS occurs in about 5% of patients with CLL and its clinical outcome is poor. Extranodal involvements of RS may be present in up to 40% of patients and central nervous system manifestation was found to be the most common. Mandibular localization of RS has not been reported so far. There is no established standard treatment for RS. The improvement of survival in RS patients is achievable with intensive anti-lymphoma chemotherapy and subsequent allogeneic stem cell transplantation (alloHSCT) performed in complete remission. Herein we report a female with RS of the right mandible and hypercalcemia was its first clinical manifestation.
Keywords
Chronic lymphocytic leukemia; Richter' s Syndrome; Diffuse large B-cell lymphoma; Hypercalcemia; Outcome
About this articleTitle
Richter's Syndrome manifested as diffuse large B-cell lymphoma of the mandible with lytic lesions and hypercalcemic crisis
Journal
Issue
Pages
409-412
Published online
2013-10-01
Page views
116
Article views/downloads
109
DOI
10.1016/j.achaem.2013.01.001
Bibliographic record
Acta Haematol Pol 2013;44(4):409-412.
Keywords
Chronic lymphocytic leukemia
Richter's Syndrome
Diffuse large B-cell lymphoma
Hypercalcemia
Outcome
Authors
Katarzyna Wiśniewska-Piąty
Grzegorz Helbig
Krzysztof Woźniczka
Andrzej Frankiewicz
Joanna Dziaczkowska-Suszek
Sławomira Kyrcz-Krzemień