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Hairy cell leukaemia

D B Chen, D H Shen, H Zhang, Y Wang, Q J Song, S M Yang, X Z Fang
Objective: To study the clinicopathologic features, diagnosis and differential diagnosis of the tumors of lymphoidand hematopoietic tissue of the spleen(TLTS). Methods: Fifty-three cases of TLTS were selected from the pathologic files from Peking University People's Hospital from April 2002 to April 2017. According to WHO classification of tumors of hematopoietic and lymphoid tissues (2008) and its updated classification (2016), the cases were studied by microscopy, immunohistochemistry and in situ hybridization, combined with the bone marrow biopsy and clinical examination...
November 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Dianne Pulte, Janick Weberpals, Lina Jansen, Sabine Luttmann, Bernd Holleczek, Alice Nennecke, Meike Ressing, Alexander Katalinic, Hermann Brenner
INTRODUCTION: Population-level survival has improved for common haematologic malignancies in the early 21st century. However, relatively few population-level data are available for rare haematologic malignancies. METHODS: Data were extracted from 12 cancer registries in Germany and the Surveillance, Epidemiology and End Results database in the United States (US). Cases of haematologic malignancies with an incidence of less than 1 per 100,000 were selected for analysis...
October 2017: European Journal of Cancer
Yi Miao, Lei Cao, Qian Sun, Xiao-Tong Li, Yan Wang, Chun Qiao, Li Wang, Rong Wang, Hai-Rong Qiu, Wei Xu, Jian-Yong Li, Yu-Jie Wu, Lei Fan
The incidence of B-cell chronic lymphoproliferative disorders (B-CLPDs) is significantly lower in China than that in western countries. There have been studies involving small cohorts with conflicting results regarding the spectrum of B-CLPDs in China, and the types and immunophenotyping of B-CLPDs in China remain largely unexplored. We conducted a retrospective analysis of 653 cases of B-CLPDs seen in our centre from 2011 to 2015. Four-colour flow cytometry was used to determine the expression of each immunological marker, and the diagnostic values of the immunological markers were also investigated...
February 2018: Hematological Oncology
Laurent Jallades, Lucile Baseggio, Pierre Sujobert, Sarah Huet, Kaddour Chabane, Evelyne Callet-Bauchu, Aurélie Verney, Sandrine Hayette, Jean-Pierre Desvignes, David Salgado, Nicolas Levy, Christophe Béroud, Pascale Felman, Françoise Berger, Jean-Pierre Magaud, Laurent Genestier, Gilles Salles, Alexandra Traverse-Glehen
Splenic diffuse red pulp lymphoma is an indolent small B-cell lymphoma recognized as a provisional entity in the World Health Organization 2008 classification. Its precise relationship to other related splenic B-cell lymphomas with frequent leukemic involvement or other lymphoproliferative disorders remains undetermined. We performed whole-exome sequencing to explore the genetic landscape of ten cases of splenic diffuse red pulp lymphoma using paired tumor and normal samples. A selection of 109 somatic mutations was then evaluated in a cohort including 42 samples of splenic diffuse red pulp lymphoma and compared to those identified in 46 samples of splenic marginal zone lymphoma and eight samples of hairy-cell leukemia...
October 2017: Haematologica
Laura Puigví, Anna Merino, Santiago Alférez, Andrea Acevedo, José Rodellar
AIMS: This work aims to propose a set of quantitative features through digital image analysis for significant morphological qualitative features of different cells for an objective discrimination among reactive, abnormal and blast lymphoid cells. METHODS: Abnormal lymphoid cells circulating in peripheral blood in chronic lymphocytic leukaemia, B-prolymphocytic leukaemia, hairy cell leukaemia, splenic marginal zone lymphoma, mantle cell lymphoma, follicular lymphoma, T-prolymphocytic leukaemia, T large granular lymphocytic leukaemia and Sézary syndrome, normal, reactive and blast lymphoid cells were included...
December 2017: Journal of Clinical Pathology
Michael J McKay, Kirsty L Rady, Thomas A McKay
Hairy cell leukaemia (HCL) is rare, accounting for only 2% of leukaemias. An even more infrequent variant has been described, HCL-V. The clinicopathologic features of these two entities overlap significantly, although they differ in a number of aspects, including demographics and immunophenotype. In this report, we present the case of a man with HCL-V diagnosed 12 years previously, who is currently haematologically stable with an unusual complication of joint pain due to extensive bony expansion secondary to leukaemic infiltration, and atypical skeletal imaging...
April 2017: Annals of Translational Medicine
Gemma Davis, Alexandra Perks, Pemith Liyanage, Konrad Staines
Oral hairy leukoplakia (OHL) is an oral mucosal lesion that is associated with Epstein-Barr virus infection. It commonly presents as an asymptomatic, non-removable white patch on the lateral borders of the tongue in individuals who are immunocompromised. Historically, OHL was thought to be pathognomonic of HIV infection; however, it is now an established phenomenon in a range of conditions affecting immune competence. Hairy cell leukaemia (HCL) is a rare chronic B cell lymphoproliferative disease named after the distinctive cytology of the atypical cells...
April 6, 2017: BMJ Case Reports
Philip A Thompson, Farhad Ravandi
Patients with hairy cell leukaemia (HCL) have highly favourable outcomes after purine analogue therapy. However, most patients subsequently relapse and require re-treatment. A minority of patients develop purine analogue-refractory disease. Targeted therapies have improved outcomes for such patients. Recently, the BRAF V600E mutation was identified in most patients with classical HCL, resulting in constitutive mitogen-activated protein kinase pathway activation; impressive responses are achieved in heavily pre-treated patients with BRAF inhibition...
May 2017: British Journal of Haematology
Samuel Barbosa Fonseca, Júlio Rodrigues de Oliveira, Cristina Gonçalves, Virgínia Lopes
A 67-year-old man was treated with cladribine for hairy cell leukaemia. A few weeks later, he presented with persistent headaches, intermittent hypoesthesia of the right upper limb and language impairment. Brain CT scan showed 3 contrast-enhancing lesions. MRI revealed infracentimetric nodular lesions with restricted diffusion. One of the lesions was surgically removed and tested positive for acid-fast bacilli. Moreover, Mycobacterium tuberculosis was confirmed by PCR. Antituberculous drugs were prescribed for 12 months, with complete resolution of neurological deficits...
December 7, 2016: BMJ Case Reports
Paul D Madge, Andrea Maggioni, Mauro Pascolutti, Moein Amin, Mario Waespy, Bernadette Bellette, Robin J Thomson, Sørge Kelm, Mark von Itzstein, Thomas Haselhorst
Siglec-2 undergoes constitutive endocytosis and is a drug target for autoimmune diseases and B cell-derived malignancies, including hairy cell leukaemia, marginal zone lymphoma, chronic lymphocytic leukaemia and non-Hodgkin's lymphoma (NHL). An alternative to current antibody-based therapies is the use of liposomal nanoparticles loaded with cytotoxic drugs and decorated with Siglec-2 ligands. We have recently designed the first Siglec-2 ligands (9-biphenylcarboxamido-4-meta-nitrophenyl-carboxamido-Neu5Acα2Me, 9-BPC-4-mNPC-Neu5Acα2Me) with simultaneous modifications at C-4 and C-9 position...
November 3, 2016: Scientific Reports
Luis Miguel Juárez Salcedo, Jose Sandoval-Sus, Ling Zhang, Donald Klippenstein, Julio C Chavez
No abstract text is available yet for this article.
September 2016: British Journal of Haematology
Bartlomiej M Getta, Kaitlin M Woo, Sean Devlin, Jae H Park, Omar Abdel-Wahab, Alan Saven, Kanti Rai, Martin S Tallman
Repeated therapy of hairy cell leukaemia (HCL) with treatments that have potential long-term toxicities has raised concerns regarding increased risk for younger patients. We compared clinical outcomes and disease complications in 63 patients with HCL aged ≤40 years at diagnosis with 268 patients >40 years treated at Memorial Sloan Kettering Cancer Center. The rate of complete remission following initial therapy was 87% and 83% (P = 0·71) and estimated 10-year overall survival was 100% and 82% (P = 0·25) in younger and older patients, respectively...
November 2016: British Journal of Haematology
Dai Chihara, Hagop Kantarjian, Susan O'Brien, Jeffrey Jorgensen, Sherry Pierce, Stefan Faderl, Alessandra Ferrajoli, Rebecca Poku, Preetesh Jain, Phillip Thompson, Mark Brandt, Rajyalakshmi Luthra, Jan Burger, Michael Keating, Farhad Ravandi
Nucleoside analogues are highly active in patients with hairy cell leukaemia (HCL); however, patients continue to relapse. This phase II study evaluated the efficacy and safety of cladribine followed by rituximab in patients with untreated HCL (N = 59), relapsed HCL (N = 14) and HCL variant (HCLv, N = 7). Cladribine 5·6 mg/m(2) was given intravenously (IV) daily for 5 d and was followed approximately 1 month later with rituximab 375 mg/m(2) IV weekly for 8 weeks. Complete response rate in patients with untreated HCL, relapsed HCL and HCLv was 100%, 100% and 86%, respectively...
September 2016: British Journal of Haematology
Aradhana Rani, Roseanna Greenlaw, Richard A Smith, Christine Galustian
Hairy and enhancer of split homolog-1 (HES1) is a part of an extensive family of basic helix-loop-helix (bHLH) proteins and plays a crucial role in the control and regulation of cell cycle, proliferation, cell differentiation, survival and apoptosis in neuronal, endocrine, T-lymphocyte progenitors as well as various cancers. HES1 is a transcription factor which is regulated by the NOTCH, Hedgehog and Wnt signalling pathways. Aberrant expression of these pathways is a common feature of cancerous cells. There appears to be a fine and complicated crosstalk at the molecular level between the various signalling pathways and HES1, which contributes to its effects on the immune response and cancers such as leukaemia...
August 2016: Cytokine & Growth Factor Reviews
Eleftheria Lakiotaki, Georgia Levidou, Maria K Angelopoulou, Christos Adamopoulos, Gerassimos Pangalis, George Rassidakis, Theodoros Vassilakopoulos, Gabriella Gainaru, Pagona Flevari, Sotirios Sachanas, Angelica A Saetta, Athanasia Sepsa, Maria Moschogiannis, Christina Kalpadakis, Nikolaos Tsesmetzis, Vassilios Milionis, Ilenia Chatziandreou, Irene Thymara, Panayiotis Panayiotidis, Maria Dimopoulou, Eleni Plata, Konstantinos Konstantopoulos, Efstratios Patsouris, Christina Piperi, Penelope Korkolopoulou
The potential role of AKT/mTOR signalling proteins and its association with the Raf-MEK-ERK pathway was investigated in hairy cell leukaemia (HCL). BRAFV600E expression and activated forms of AKT, mTOR, ERK1/2, p70S6k and 4E-BP1 were immunohistochemically assessed in 77 BM biopsies of HCL patients and correlated with clinicopathological and BM microvascular characteristics, as well as with c-Caspase-3 levels in hairy cells. Additionally, we tested rapamycin treatment response of BONNA-12 wild-type cells or transfected with BRAFV600E...
February 19, 2016: Scientific Reports
Nicola J Weston-Bell, Will Tapper, Jane Gibson, Dean Bryant, Yurany Moreno, Melford John, Sarah Ennis, Hanneke C Kluin-Nelemans, Andrew R Collins, Surinder S Sahota
In classic Hairy cell leukaemia (HCLc), a single case has thus far been interrogated by whole exome sequencing (WES) in a treatment naive patient, in which BRAF V(600)E was identified as an acquired somatic mutation and confirmed as occurring near-universally in this form of disease by conventional PCR-based cohort screens. It left open however the question whether other genome-wide mutations may also commonly occur at high frequency in presentation HCLc disease. To address this, we have carried out WES of 5 such typical HCLc cases, using highly purified splenic tumour cells paired with autologous T cells for germline...
2016: PloS One
Estella Matutes, Alejandra Martínez-Trillos, Elias Campo
Hairy cell leukaemia-variant (HCL-V) is a rare B-cell malignancy that affects elderly males and manifests with splenomegaly, lymphocytosis and cytopenias without monocytopenia. The neoplastic cells have morphological features of prolymphocytes and hairy cells. The immunophenotype is that of a clonal B-cell CD11c and CD103 positive but, unlike classical HCL, CD25, CD123 and CD200 negative. The spleen histology is similar to classical HCL and the pattern of bone marrow infiltration is interstitial and/or intrasinusoidal...
December 2015: Best Practice & Research. Clinical Haematology
Monica Else, Claire E Dearden, Daniel Catovsky
Since 2006 when we last reviewed the literature concerning the use of purine analogues in hairy cell leukaemia (HCL), results from several new and updated series have been published. Here we examine these reports and consider their implications for patient management. The two purine analogues pentostatin and cladribine remain the first-line treatments of choice for all patients with HCL. Although they have not been compared in randomised trials, they appear to be equally effective. A complete response is important for the long-term outcome and we look at how best this can be achieved...
December 2015: Best Practice & Research. Clinical Haematology
Andrew Wotherspoon, Ayoma Attygalle, Larissa Sena Teixeira Mendes
Hairy cell leukaemia is a rare chronic neoplastic B-cell lymphoproliferation that characteristically involves blood, bone marrow and spleen with liver, lymph node and skin less commonly involved. Histologically, the cells have a characteristic appearance with pale/clear cytoplasm and round or reniform nuclei. In the spleen, the infiltrate involves the red pulp and is frequently associated with areas of haemorrhage (blood lakes). The cells stain for B-cell related antigens as well as with antibodies against tartrate-resistant acid phosphatase, DBA44 (CD72), CD11c, CD25, CD103, CD123, cyclin D1 and annexin A1...
December 2015: Best Practice & Research. Clinical Haematology
Tamar Tadmor, Aaron Polliack
Hairy cell leukaemia (HCL) is an orphan subtype of leukaemia which constitutes less than 2% of all leukaemia's, with an incidence of less than 1 per 100,000 persons per annum. Median age at presentation is 55 years and it is 3-4 times more frequent in males. It is also more frequently encountered in whites and less in Asians, Africans and Arabs. The epidemiologic data are multi-factorial and influenced by ethnicity and geographical factors. Other reported associations relate to some environmental exposures and possible occupational factors...
December 2015: Best Practice & Research. Clinical Haematology
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