Sézary Syndrome Coexisting with B-Cell Chronic Lymphocytic Leukemia: Case Report and Review of the Literature
Introduction: The simultaneous presentation of chronic B-cell lymphocytic leukemia (B-CLL) and cutaneous T-cell lymphoma (CTCL) is extremely rare. Case Report: We describe a patient with B-CLL and Sézary syndrome (SS), an erythrodermic and leukemic variant of CTCL. Despite treatment, the SS progress...
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| Veröffentlicht in: | Dermatology (Basel) 2008-01, Vol.216 (1), p.68-75 |
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| creator | Karsai, Syrus Hou, J. Steve Telang, Gladys Kantor, Gary R. Nowell, Peter C. Vonderheid, Eric C. |
| description | Introduction: The simultaneous presentation of chronic B-cell lymphocytic leukemia (B-CLL) and cutaneous T-cell lymphoma (CTCL) is extremely rare. Case Report: We describe a patient with B-CLL and Sézary syndrome (SS), an erythrodermic and leukemic variant of CTCL. Despite treatment, the SS progressed to involve internal organs and eventual death of the patient from sepsis. This is the first reported case of SS coexisting with chronic lymphocytic leukemia in which an anti-Vβ13.6 antibody was used to serially track changes in circulating neoplastic T cells vis-à-vis neoplastic B cells and to detect neoplastic T cells in ascitic fluid near the end of the patient’s life. Discussion: We speculate that the coexistence of B-CLL and CTCL is the result of an initiating genetic or epigenetic defect at the level of the common lymphoid stem cell that predisposes both B-cell and T-cell lineages to additional oncogenic changes at a more advanced stage of differentiation. |
| doi_str_mv | 10.1159/000109362 |
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Steve ; Telang, Gladys ; Kantor, Gary R. ; Nowell, Peter C. ; Vonderheid, Eric C.</creator><creatorcontrib>Karsai, Syrus ; Hou, J. Steve ; Telang, Gladys ; Kantor, Gary R. ; Nowell, Peter C. ; Vonderheid, Eric C.</creatorcontrib><description>Introduction: The simultaneous presentation of chronic B-cell lymphocytic leukemia (B-CLL) and cutaneous T-cell lymphoma (CTCL) is extremely rare. Case Report: We describe a patient with B-CLL and Sézary syndrome (SS), an erythrodermic and leukemic variant of CTCL. Despite treatment, the SS progressed to involve internal organs and eventual death of the patient from sepsis. This is the first reported case of SS coexisting with chronic lymphocytic leukemia in which an anti-Vβ13.6 antibody was used to serially track changes in circulating neoplastic T cells vis-à-vis neoplastic B cells and to detect neoplastic T cells in ascitic fluid near the end of the patient’s life. Discussion: We speculate that the coexistence of B-CLL and CTCL is the result of an initiating genetic or epigenetic defect at the level of the common lymphoid stem cell that predisposes both B-cell and T-cell lineages to additional oncogenic changes at a more advanced stage of differentiation.</description><identifier>ISSN: 1018-8665</identifier><identifier>EISSN: 1421-9832</identifier><identifier>DOI: 10.1159/000109362</identifier><identifier>PMID: 18032903</identifier><language>eng</language><publisher>Basel, Switzerland: Karger</publisher><subject>Aged ; Aged, 80 and over ; Antigens, CD - analysis ; B-Lymphocytes - metabolism ; Biological and medical sciences ; Case Report ; Dermatology ; Fatal Outcome ; Female ; Hematologic and hematopoietic diseases ; Humans ; Leukemia ; Leukemia, Lymphocytic, Chronic, B-Cell - complications ; Leukemia, Lymphocytic, Chronic, B-Cell - immunology ; Leukemia, Lymphocytic, Chronic, B-Cell - pathology ; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis ; Lymphocytes ; Male ; Medical diagnosis ; Medical sciences ; Middle Aged ; Pathology ; Receptors, Antigen, T-Cell, alpha-beta - analysis ; Sezary Syndrome - complications ; Sezary Syndrome - immunology ; Sezary Syndrome - pathology ; Skin - pathology ; Skin Neoplasms - complications ; Skin Neoplasms - immunology ; Skin Neoplasms - pathology ; T-Lymphocytes - metabolism</subject><ispartof>Dermatology (Basel), 2008-01, Vol.216 (1), p.68-75</ispartof><rights>2008 S. Karger AG, Basel</rights><rights>2008 INIST-CNRS</rights><rights>(c) 2008 S. Karger AG, Basel.</rights><rights>Copyright (c) 2007 S. 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Steve</creatorcontrib><creatorcontrib>Telang, Gladys</creatorcontrib><creatorcontrib>Kantor, Gary R.</creatorcontrib><creatorcontrib>Nowell, Peter C.</creatorcontrib><creatorcontrib>Vonderheid, Eric C.</creatorcontrib><title>Sézary Syndrome Coexisting with B-Cell Chronic Lymphocytic Leukemia: Case Report and Review of the Literature</title><title>Dermatology (Basel)</title><addtitle>Dermatology</addtitle><description>Introduction: The simultaneous presentation of chronic B-cell lymphocytic leukemia (B-CLL) and cutaneous T-cell lymphoma (CTCL) is extremely rare. Case Report: We describe a patient with B-CLL and Sézary syndrome (SS), an erythrodermic and leukemic variant of CTCL. Despite treatment, the SS progressed to involve internal organs and eventual death of the patient from sepsis. This is the first reported case of SS coexisting with chronic lymphocytic leukemia in which an anti-Vβ13.6 antibody was used to serially track changes in circulating neoplastic T cells vis-à-vis neoplastic B cells and to detect neoplastic T cells in ascitic fluid near the end of the patient’s life. 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Myelofibrosis</topic><topic>Lymphocytes</topic><topic>Male</topic><topic>Medical diagnosis</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>Pathology</topic><topic>Receptors, Antigen, T-Cell, alpha-beta - analysis</topic><topic>Sezary Syndrome - complications</topic><topic>Sezary Syndrome - immunology</topic><topic>Sezary Syndrome - pathology</topic><topic>Skin - pathology</topic><topic>Skin Neoplasms - complications</topic><topic>Skin Neoplasms - immunology</topic><topic>Skin Neoplasms - pathology</topic><topic>T-Lymphocytes - metabolism</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Karsai, Syrus</creatorcontrib><creatorcontrib>Hou, J. 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This is the first reported case of SS coexisting with chronic lymphocytic leukemia in which an anti-Vβ13.6 antibody was used to serially track changes in circulating neoplastic T cells vis-à-vis neoplastic B cells and to detect neoplastic T cells in ascitic fluid near the end of the patient’s life. Discussion: We speculate that the coexistence of B-CLL and CTCL is the result of an initiating genetic or epigenetic defect at the level of the common lymphoid stem cell that predisposes both B-cell and T-cell lineages to additional oncogenic changes at a more advanced stage of differentiation.</abstract><cop>Basel, Switzerland</cop><pub>Karger</pub><pmid>18032903</pmid><doi>10.1159/000109362</doi><tpages>8</tpages></addata></record> |
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| subjects | Aged Aged, 80 and over Antigens, CD - analysis B-Lymphocytes - metabolism Biological and medical sciences Case Report Dermatology Fatal Outcome Female Hematologic and hematopoietic diseases Humans Leukemia Leukemia, Lymphocytic, Chronic, B-Cell - complications Leukemia, Lymphocytic, Chronic, B-Cell - immunology Leukemia, Lymphocytic, Chronic, B-Cell - pathology Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis Lymphocytes Male Medical diagnosis Medical sciences Middle Aged Pathology Receptors, Antigen, T-Cell, alpha-beta - analysis Sezary Syndrome - complications Sezary Syndrome - immunology Sezary Syndrome - pathology Skin - pathology Skin Neoplasms - complications Skin Neoplasms - immunology Skin Neoplasms - pathology T-Lymphocytes - metabolism |
| title | Sézary Syndrome Coexisting with B-Cell Chronic Lymphocytic Leukemia: Case Report and Review of the Literature |
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