Diagnosis, biology and treatment of hairy-cell leukaemia
Abstract
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.
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RhoH expression levels are a crucial factor in protection from the development of haematopoietic malignancies.
“Nevertheless, the recent finding that RHOH is underexpressed in certain types of leukaemia implies that RhoH expression levels are a crucial factor in protection from the development of haematopoietic malignancies [56,57].”
From The small GTPase RhoH is an atypical regulator of haematopoietic cells (2008) · cites this paper as [57]
Most data gathered so far indicate that in human cancers the absence of a functional RhoH protein or reduced RhoH expression levels rather than its overexpression may play a role in pathogenesis.
“Most data gathered so far however indicate that in human cancers the absence of a functional RhoH protein or reduced RhoH expression levels rather than its overexpression may play a role in pathogenesis [32,56,57].”
From The small GTPase RhoH is an atypical regulator of haematopoietic cells (2008) · cites this paper as [57]
RHOH is underexpressed in certain types of leukaemia.
“Nevertheless, the recent finding that RHOH is underexpressed in certain types of leukaemia implies that RhoH expression levels are a crucial factor in protection from the development of haematopoietic malignancies [56,57].”
From The small GTPase RhoH is an atypical regulator of haematopoietic cells (2008) · cites this paper as [57]
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The small GTPase RhoH is an atypical regulator of haematopoietic cells
Fueller, F., & Kubatzky, K. F. (2008) · Cell Communication and Signaling · doi:10.1186/1478-811x-6-6 · Cited by 40 · see citing works
- Authors
- Allsup, D. J., & Cawley, J. C.
- Affiliations
- Hull Royal Infirmary (GB)
- Royal Liverpool University Hospital (GB)
- Corresponding author
- Allsup, D. J.
- ORCID
- Allsup, D. J.
- Venue
- Clinical and Experimental Medicine
- Published
- 2004-12
- Volume
- 4
- Issue
- 3
- Pages
- 132-138
- ISSN
- 1591-8890 (ISSN-L)
- 1591-9528
- OpenAlex
- W2007590926
- Cited by
- 11
- References
- 0
- Citations by year
- 2014: 1 · 2012: 1
- FWCI
- 0.92
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Clinical and Experimental Medicine (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Genetics › Chronic Lymphocytic Leukemia Research
- Health Sciences › Medicine › Pathology and Forensic Medicine › Lymphoma Diagnosis and Treatment
- Health Sciences › Medicine › Emergency Medicine › Hematological disorders and diagnostics
- Keywords
- hairy cell leukemia, hairy cells, B cell activation, bone marrow fibrosis, deoxycoformycin, rituximab, relapsed/refractory disease, pathogenesis
- MeSH terms
- Diagnosis, Differential, Humans, Leukemia, Hairy Cell
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