Diagnosis, biology and treatment of hairy-cell leukaemia
Hairy-cell leukaemia (HCL) is usually readily diagnosed by seeing typical hairy cells (HCs) in the blood film. The diagnosis is then confirmed by tartrate-resistant acid phosphatase staining, marker analysis, and bone marrow examination. HCs are clonal mature memory B cells with specific features of...
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Veröffentlicht in: | Clinical and experimental medicine 2004-12, Vol.4 (3), p.132-138 |
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description | Hairy-cell leukaemia (HCL) is usually readily diagnosed by seeing typical hairy cells (HCs) in the blood film. The diagnosis is then confirmed by tartrate-resistant acid phosphatase staining, marker analysis, and bone marrow examination. HCs are clonal mature memory B cells with specific features of activation. This HC activation is responsible for many of the pathological features of the disease, including its distinctive bone marrow fibrosis, splenic red pulp invasion, and pseudo-sinus formation. Chlorodeoxyadenasine is the treatment of first choice. Deoxycoformycin and rituximab are useful for the treatment of relapsed/refractory disease. The nature of the primary oncogenic event(s) remains unknown and is the major unresolved issue in HCL. |
doi_str_mv | 10.1007/s10238-004-0046-z |
format | Article |
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The diagnosis is then confirmed by tartrate-resistant acid phosphatase staining, marker analysis, and bone marrow examination. HCs are clonal mature memory B cells with specific features of activation. This HC activation is responsible for many of the pathological features of the disease, including its distinctive bone marrow fibrosis, splenic red pulp invasion, and pseudo-sinus formation. Chlorodeoxyadenasine is the treatment of first choice. Deoxycoformycin and rituximab are useful for the treatment of relapsed/refractory disease. The nature of the primary oncogenic event(s) remains unknown and is the major unresolved issue in HCL.</description><identifier>ISSN: 1591-8890</identifier><identifier>EISSN: 1591-9528</identifier><identifier>DOI: 10.1007/s10238-004-0046-z</identifier><identifier>PMID: 15599661</identifier><identifier>CODEN: CEMLBA</identifier><language>eng</language><publisher>Italy: Springer Nature B.V</publisher><subject>Chemotherapy ; Diagnosis, Differential ; Humans ; Leukemia ; Leukemia, Hairy Cell - diagnosis ; Leukemia, Hairy Cell - drug therapy ; Leukemia, Hairy Cell - pathology ; Leukemia, Hairy Cell - physiopathology ; Medical diagnosis ; Medical treatment</subject><ispartof>Clinical and experimental medicine, 2004-12, Vol.4 (3), p.132-138</ispartof><rights>Copyright Springer-Verlag 2004</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c326t-47ac47386f3755b792aabc3d13bb29a83d051fa1e5c77330f94927fda4d42dfd3</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27903,27904</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/15599661$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Allsup, D J</creatorcontrib><creatorcontrib>Cawley, J C</creatorcontrib><title>Diagnosis, biology and treatment of hairy-cell leukaemia</title><title>Clinical and experimental medicine</title><addtitle>Clin Exp Med</addtitle><description>Hairy-cell leukaemia (HCL) is usually readily diagnosed by seeing typical hairy cells (HCs) in the blood film. 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The diagnosis is then confirmed by tartrate-resistant acid phosphatase staining, marker analysis, and bone marrow examination. HCs are clonal mature memory B cells with specific features of activation. This HC activation is responsible for many of the pathological features of the disease, including its distinctive bone marrow fibrosis, splenic red pulp invasion, and pseudo-sinus formation. Chlorodeoxyadenasine is the treatment of first choice. Deoxycoformycin and rituximab are useful for the treatment of relapsed/refractory disease. The nature of the primary oncogenic event(s) remains unknown and is the major unresolved issue in HCL.</abstract><cop>Italy</cop><pub>Springer Nature B.V</pub><pmid>15599661</pmid><doi>10.1007/s10238-004-0046-z</doi><tpages>7</tpages></addata></record> |
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subjects | Chemotherapy Diagnosis, Differential Humans Leukemia Leukemia, Hairy Cell - diagnosis Leukemia, Hairy Cell - drug therapy Leukemia, Hairy Cell - pathology Leukemia, Hairy Cell - physiopathology Medical diagnosis Medical treatment |
title | Diagnosis, biology and treatment of hairy-cell leukaemia |
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