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Hairy cell leukemia

Robert J Kreitman, Martin S Tallman, Tadeusz Robak, Steven Coutre, Wyndham H Wilson, Maryalice Stetler-Stevenson, David J FitzGerald, Linda Santiago, Guozhi Gao, Mark C Lanasa, Ira Pastan
Anti-CD22 moxetumomab pasudotox achieved 46% complete remissions (CRs) in previously reported phase I testing in relapsed/refractory hairy cell leukemia (HCL; n=28). The importance of minimal residual disease (MRD) after CR in HCL is unknown. A 21-patient extension cohort received 50 µg/kg every other day for 3 doses in 4-week cycles. These patients plus 12 previously reported at this upper dose level received 143 cycles without dose-limiting toxicity. The combined 33-patient cohort achieved 64% CR and 88% overall response rates, with median CR duration of 42...
February 27, 2018: Blood
Avinash G Dinmohamed, Eduardus F M Posthuma, Otto Visser, Arnon P Kater, Reinier A P Raymakers, Jeanette K Doorduijn
No abstract text is available yet for this article.
January 30, 2018: Blood
Peter H Wiernik
The purpose of this paper is to review the preclinical and clinical rationale for androgen therapy of acute myeloid (AML) and hairy cell leukemia (HCL). A major recent positive study should renew interest in this therapy, which has been reported to be effective in these leukemias for more than 50 years. Hopefully, renewed interest in this approach, which seems promising, will lead to well-designed modern studies that will precisely define a role for androgens in these leukemias. A recent large prospective, randomized study has demonstrated enhanced survival for elderly AML patients who present with WBC counts < 30,000/μL have improved overall survival when androgens are given post-remission, and numerous case reports suggest that androgens frequently restore normal peripheral blood counts in severely pancytopenic patients with HCL...
January 23, 2018: Current Treatment Options in Oncology
Andrea Visentin, Silvia Imbergamo, Federica Frezzato, Marco Pizzi, Roberta Bertorelle, Edoardo Scomazzon, Tamara Berno, Marcello Riva, Elisa Piva, Monica Facco, Francesco Piazza, Gianpietro Semenzato, Livio Trentin
Hairy cell leukemia variant (HCLv) is a chronic lymphoproliferative disorder classified as a provisional entity in the 2016 WHO Classification of Lymphoid Tumors. HCLv is characterized by unfavorable prognosis, low complete remission rates and limited disease control following classical hairy cell leukemia-based regimens. In this study, we report 3 cases of elderly patients with treatment-naive, TP53 un-mutated HCLv, who were effectively treated with four cycles of bendamustine plus rituximab. The regimen was completed in all the patients with acceptable toxicity...
December 15, 2017: Oncotarget
Anamarija M Perry, Kant Matsuda, Vikram Wadhwa, Donna Hewitt, Muhamad Almiski, James B Johnston, Versha Banerji
Brain involvement, although rare, can occur in HCL.The combination of cladribine and rituximab is a highly effective treatment of HCL with brain involvement.
June 13, 2017: Blood Advances
Marc Sorigue, Edurne Sarrate, Mireia Franch-Sarto, Evarist Feliu, Jordi Junca
BACKGROUND: The Moreau score is essential for the diagnosis of B-cell lymphoproliferative disorders (B-LPD). METHODS: We assessed the consistency of the Moreau score in a series of 138 patients with at least two samples involved by a B-LPD (316 samples) other than germinal center-derived malignancies, hairy cell leukemia, and mantle cell lymphomas. Patients with evidence of two distinct B-LPDs were also excluded. RESULTS: We found 53 inconsistencies in 44 of 138 (32%) patients...
December 28, 2017: Journal of Clinical Laboratory Analysis
Davide Vacca, Valeria Cancila, Alessandro Gulino, Giosuè Lo Bosco, Beatrice Belmonte, Arianna Di Napoli, Ada Maria Florena, Claudio Tripodo, Walter Arancio
The MinION is a miniaturized high-throughput next generation sequencing platform of novel conception. The use of nucleic acids derived from formalin-fixed paraffin-embedded samples is highly desirable, but their adoption for molecular assays is hurdled by the high degree of fragmentation and by the chemical-induced mutations stemming from the fixation protocols. In order to investigate the suitability of MinION sequencing on formalin-fixed paraffin-embedded samples, the presence and frequency of BRAF c.1799T > A mutation was investigated in two archival tissue specimens of Hairy cell leukemia and Hairy cell leukemia Variant...
December 13, 2017: Molecular Biology Reports
Jacqueline N Poston, Pamela S Becker
This review focuses on the data supporting the use of myeloid growth factors (MGFs) in patients being treated for acute myeloid leukemia, acute lymphoblastic leukemia, chronic myeloid leukemia, and hairy cell leukemia, for which neutropenia is a common complication of treatment. However, due to the lack of randomized trial data or conflicting results of clinical studies, comprehensive guidelines have been difficult to formulate. Moreover, to date, these diagnoses have not been addressed in the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for MGFs...
December 2017: Journal of the National Comprehensive Cancer Network: JNCCN
Şeniz Öngören, Ahmet Emre Eşkazan, Selin Berk, Tuğrul Elverdi, Ayşe Salihoğlu, Muhlis Cem Ar, Zafer Başlar, Yıldız Aydın, Nükhet Tüzüner, Teoman Soysal
OBJECTIVE: In this study, we retrospectively analyzed the clinical outcome, treatment responses, infectious complications, and survival rates of 71 hairy cell leukemia (HCL) cases. MATERIALS AND METHODS: Sixty-seven patients received a first-line treatment and 2-chlorodeoxyadenosine (cladribine-2-CdA) was administered in 31 cases, 19 patients received interferon-alpha (INF-α), splenectomy was performed in 16 cases, and rituximab was used in one. RESULTS: Although the highest overall response rate (ORR) was observed in patients receiving 2-CdA upfront, ORRs were comparable in the 2-CdA, INF-α, and splenectomy subgroups...
December 1, 2017: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
Tamim Alsuliman, Kaiss Lassoued, Maifa Belghoul, Karima Debbache, Bachra Choufi
INTRODUCTION: Pentostatin (2'-deoxycoformycin) and cladribine (2-chlorodeoxyadenosine) are adenosine analogues widely used to treat lymphoid malignancies, mainly hairy cell leukemia (HCL). Oral or parenteral adenosine analogues have been also used as immunomodulatory agents in multiple sclerosis and in acute graft-versus-host disease. CASE REPORT: Here, we report the case of a 43-year-old patient with a history of extensive psoriasis who later developed HCL. RESULTS: The patient had achieved complete remission of both psoriasis and HCL after receiving intravenous infusions of pentostatin...
March 2018: Dermatology and Therapy
William G Wierda, John C Byrd, Jeremy S Abramson, Seema Bhat, Greg Bociek, Danielle Brander, Jennifer Brown, Asher Chanan-Khan, Steve E Coutre, Randall S Davis, Christopher D Fletcher, Brian Hill, Brad S Kahl, Manali Kamdar, Lawrence D Kaplan, Nadia Khan, Thomas J Kipps, Jeffrey Lancet, Shuo Ma, Sami Malek, Claudio Mosse, Mazyar Shadman, Tanya Siddiqi, Deborah Stephens, Nina Wagner, Andrew D Zelenetz, Mary A Dwyer, Hema Sundar
Hairy cell leukemia (HCL) is a rare type of indolent B-cell leukemia, characterized by symptoms of fatigue and weakness, organomegaly, pancytopenia, and recurrent opportunistic infections. Classic HCL should be considered a distinct clinical entity separate from HCLvariant (HCLv), which is associated with a more aggressive disease course and may not respond to standard HCL therapies. Somatic hypermutation in the IGHV gene is present in most patients with HCL. The BRAF V600E mutation has been reported in most patients with classic HCL but not in those with other B-cell leukemias or lymphomas...
November 2017: Journal of the National Comprehensive Cancer Network: JNCCN
Xavier Troussard, Edouard Cornet
DISEASE OVERVIEW: Hairy cell leukemia (HCL) and HCL-like disorders, including HCL variant (HCL-V) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogeneous group of mature lymphoid B-cell disorders, characterized by the identification of hairy cells, a specific genetic profile, a different clinical course and the need for appropriate treatment. DIAGNOSIS: Diagnosis of HCL is based on morphological evidence of hairy cells, an HCL immunologic score of 3 or 4 based on the CD11C, CD103, CD123, and CD25 expression, the trephine biopsy which makes it possible to specify the degree of tumoral medullary infiltration and the presence of BRAF V600E somatic mutation...
December 2017: American Journal of Hematology
Thomas A Waldmann
Cytokines that control the immune response were shown to have efficacy in preclinical murine cancer models. Interferon (IFN)-α is approved for treatment of hairy cell leukemia, and interleukin (IL)-2 for the treatment of advanced melanoma and metastatic renal cancer. In addition, IL-12, IL-15, IL-21, and granulocyte macrophage colony-stimulating factor (GM-CSF) have been evaluated in clinical trials. However, the cytokines as monotherapy have not fulfilled their early promise because cytokines administered parenterally do not achieve sufficient concentrations in the tumor, are often associated with severe toxicities, and induce humoral or cellular checkpoints...
November 3, 2017: Cold Spring Harbor Perspectives in Biology
Jong Gwon Choi, Hwan Hwi Cho, Sang Rok Kang, Se Min Jang, Eun Hyung Yoo, Hyun Jung Cho, Sun Moon Kim, Do Yeun Cho
Myelofibrosis (MF) is often accompanied by chronic myeloid leukemia, hairy cell leukemia, or certain primary myeloproliferative neoplasms, but is rarely associated with lymphoid neoplasms. We herein describe a case of intravascular large B-cell lymphoma (IVLBCL) with MF. IVLBCL is a rare, aggressive type of extranodal B-cell lymphoma, defined by proliferation of lymphomatous cells within small-to medium-sized vessels. A 60-year-old woman was admitted to the hospital with anemia, thrombocytopenia and fever. Bone marrow biopsy findings included trilineage hematopoiesis, increased numbers of immature cells, markedly abnormal and enlarged megakaryocytes, and diffuse fibrosis in multiple focal areas throughout the entire bone marrow space...
November 2017: Molecular and Clinical Oncology
Amir Ghorbani-Aghbolaghi, Mirna Lechpammer, Saba F Ali, Nam K Ku, Denis M Dwyre, Hooman H Rashidi
BRAF protein is a serine/threonine kinase with 766 amino acids. Approximately 15% of human cancers harbor BRAF mutations as well as other BRAF anomalies (amplifications, fusions). Somatic mutations mainly occur in the catalytic kinase domain (CR3), and the predominant mutation is p.V600E which is the substitution of glutamic acid (E) for valine (V) as result of a mutation at codon 600 of the kinase domain. To our knowledge, the vast majority of the cancers have non-germline BRAF mutations. Here we describe a case of a 60-year-old female with a history of hairy cell leukemia (HCL) who presented with aphasia and forgetfulness...
July 2017: Autopsy & Case Reports
Leslie A Andritsos, Nicole R Grieselhuber, Mirela Anghelina, Kerry A Rogers, Sameek Roychowdhury, Julie W Reeser, Cynthia D Timmers, Aharon G Freud, James S Blachly, David M Lucas, Gerard Lozanski, Jeffrey A Jones, Katie Williams, Christopher Oakes, Dan Jones, Michael R Grever
No abstract text is available yet for this article.
September 18, 2017: Leukemia & Lymphoma
Kensuke Matsuda, Yosuke Matsumoto, Mihoko Yoshida, Kazuho Shimura, Hiroto Kaneko, Tohru Inaba, Shigeo Horiike, Junya Kuroda, Masafumi Taniwaki
Hairy B-cell lymphoproliferative disorder (HBLD) is one of chronic polyclonal B-cell lymphocytosis. We report a 47-year-old female Japanese patient diagnosed as having HBLD based on lymphocytosis with hairy cell appearance and characteristic phenotypes including CD11c+ and without B-cell monoclonality. She was a non-smoker and possessed HLA-DR4. She has been closely followed up without treatment and lymphoma development for over five years. Although this disease is quite rare and has been reported, to our knowledge, in only 13 Japanese cases, an accurate diagnosis, particularly differential diagnosis from persistent polyclonal B-cell lymphocytosis or hairy cell leukemia-Japanese variant is essential for the prevention of unnecessary treatments...
2017: Mediterranean Journal of Hematology and Infectious Diseases
Andreas Agathangelidis, Aliki Xochelli, Kostas Stamatopoulos
Associations between immunoglobulin (IG) receptors with distinctive immunogenetic features and particular gene mutations are a recurring theme in mature B-cell lymphomas. Relevant observations have been made in chronic lymphocytic leukemia (CLL), where gene mutations are distributed asymmetrically in cases bearing or not somatic hypermutations within the clonotypic immunoglobulin heavy chain variable region (IGHV) genes (e.g. TP53 mutations predominate in IG-unmutated CLL, whereas the opposite is seen for MYD88 mutations, enriched in IG-mutated CLL) and in subsets of cases with stereotyped IG (enrichment for SF3B1 mutations in CLL subset #2)...
September 11, 2017: Journal of Pathology
Ilana Levy, Tamar Tadmor
No abstract text is available yet for this article.
December 1, 2017: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
Elissandra Machado Arlindo, Natália Aydos Marcondes, Flavo Beno Fernandes, Gustavo Adolpho Moreira Faulhaber
BACKGROUND: Distinction between mature B-cell neoplasms can be difficult due to overlapping of immunologic features and clinical manifestations. This study investigated whether quantifying mean fluorescence intensity of four monoclonal antibodies in a flow cytometry panel is useful for the differential diagnosis and characterization of these disorders. METHODS: The expressions of CD52, CD200, CD123 and CD43 were analyzed in samples from 124 patients with mature B-cell neoplasms...
July 2017: Revista Brasileira de Hematologia e Hemoterapia
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