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Hairy Cell Leukaemia (hairy + cell_leukaemia)

Distribution by Scientific Domains

Medical Sciences	100%

Selected Abstracts

Acquired Glanzmann's thrombasthenia associated with Hairy cell leukaemia

EUROPEAN JOURNAL OF CLINICAL INVESTIGATION, Issue 12 2009
M. Kannan
No abstract is available for this article. [source]

High density genome-wide DNA profiling reveals a remarkably stable profile in hairy cell leukaemia

BRITISH JOURNAL OF HAEMATOLOGY, Issue 5 2008
Francesco Forconi
Summary Hairy cell leukaemia (HCL) is a rare B-cell neoplasm for which the molecular mechanisms are largely unknown. High-density genome-wide DNA profiling was performed with Affymetrix 250K arrays to analyse copy number (CN) changes and loss of heterozygosity (LOH) in 16 cases of HCL. Four of 16 cases (25%) demonstrated gross non-recurrent CN deletions. Within the affected regions, we identified genes involved in bone marrow fibrosis (FGF12) and response to treatment (TP53) in individual cases. Large regions (>5 Mb) of LOH without any concomitant DNA CN changes were identified in 5/16 (31%) HCL and were indicative of uniparental disomy UD. The germline origin of UD was demonstrated in one case for which a matched normal sample was available. Overall analysis of LOH showed that identical loci were recurrently targeted in chromosomes 1, 2 and 6. As a whole, however, HCL showed a remarkably stable genome. This finding adds to several other features that are unique to HCL among mature B-cell tumours. [source]

A narrow deletion of 7q is common to HCL, and SMZL, but not CLL

EUROPEAN JOURNAL OF HAEMATOLOGY, Issue 6 2004
Claus Lindbjerg Andersen
Abstract: To further characterise the genetic background of the two closely related B-lymphocytic malignancies hairy cell leukaemia (HCL), and splenic marginal zone lymphoma (SMZL) we have identified characteristic copy number imbalances by comparative genomic hybridisation (CGH). Based on these findings, areas of special interest were fine mapped, and relevant probes constructed for use in interphase-fluorescence in situ hybridisation (FISH) investigations. Thus, using the CGH data from 52 HCL and 61 SMZL patients, we identified the characteristic profiles of copy number imbalances for both diseases. These were a gain of 5q13-31 (19%) and loss of 7q22-q35 (6%) for HCL, and gain of 3q25 (28%), loss of 7q31 (16%), and gain of 12q15 (16%) for SMZL. A partial loss of 7q unsual for low-malignant B-cell diseases was found to be common to the two diseases. This loss was therefore fine mapped with BAC/PAC clones. Fine mapping revealed that in SMZL the minimal lost region covers 11.4 Mb spanning from 7q31.33 to 7q33 located between sequence tagged site (STS)-markers SHGC-3275 and D7S725. This area was distinct from the commonly deleted 7q region of myelodysplastic syndrome/acute myeloid leukaemia (MDS/AML). A FISH probe specific for the 7q region was constructed. Using this probe in an interphase-FISH investigation we showed the minimal lost 7q-region of HCL and SMZL to be one and the same. In one HCL case, this investigation furthermore showed the extent of the deleted region to be below the detection limit of CGH, whereas interphase-FISH screening of 36 chronic lymphocytic leukaemia (CLL) cases showed no deletion of the 7q area. In conclusion, we have identified characteristic profiles of copy number imbalances in HCL and SMZL and fine mapped the minimal extent of a commonly lost 7q area of special interest. We hypothesise that this region may contain (a) gene(s) important for the pathology of HCL and SMZL. [source]

Systemic vasculitis complicating hairy cell leukaemia treatment with cladribine

INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 4 2002
B. Tousi
Summary A single course of cladribine has been used commonly over the past decade to treat hairy cell leukaemia, with an impressive rate of complete remission and few serious adverse effects. Although vasculitis has been reported in the course of hairy cell leukaemia, it has only rarely been reported as the consequence of cladribine treatment. We describe a 73-year-old woman who developed serious systemic vasculitis with associated thrombocytopenia in the course of treatment with cladribine. [source]

Sustained long-term remissions with weekly interferon maintenance therapy in hairy cell leukemia

ASIA-PACIFIC JOURNAL OF CLINICAL ONCOLOGY, Issue 3 2010
Raj RAMAKRISHNA
Abstract Aim: This study evaluates the efficacy of weekly ,-interferon (IFN) maintenance therapy in hairy cell leukaemia (HCL), a disease that remains incurable. Method: Nine patients (six male, three female, aged 41,69 yrs) with hairy cell leukaemia (HCL) received IFN 3mU s.c. once weekly as long-term maintenance therapy after achieving optimal clinical and hematological response to initial therapy with thrice weekly IFN. Results: Eight of the nine patients are in a state of sustained response at 3,17 years (median 12 years). Conclusion: Our results are similar to those from three previous studies using long-term IFN maintenance therapy, bringing the total number of patients in sustained remission to 118. We hope these reports will lead to a multi-centre, phase III study of IFN maintenance therapy (including pegylated IFN, given less frequently) in HCL patients achieving optimal response to initial therapy, be it IFN or a purine analogue. [source]