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Hairy cell leukemia

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https://www.readbyqxmd.com/read/28918710/trametinib-for-the-treatment-of-ighv4-34-map2k1-mutant-variant-hairy-cell-leukemia
#1
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
https://www.readbyqxmd.com/read/28894563/hairy-b-cell-lymphoproliferative-disorder-and-its-differential-diagnosis-a-case-with-long-term-follow-up
#2
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
https://www.readbyqxmd.com/read/28892161/a-gene-is-known-by-the-company-it-keeps-enrichment-of%C3%A2-tnfaip3%C3%A2-gene-aberrations-in-malt-lymphomas-expressing-ighv4-34-antigen-receptors
#3
Andreas Agathangelidis, Aliki Xochelli, Kostas Stamatopoulos
Associations between immunoglobulin (IG) receptors with distinctive immunogenetic features and particular gene mutations is 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
https://www.readbyqxmd.com/read/28840848/time-to-cure-hairy-cell-leukemia
#4
Ilana Levy, Tamar Tadmor
No abstract text is available yet for this article.
August 25, 2017: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/28830605/quantitative-flow-cytometric-evaluation-of-cd200-cd123-cd43-and-cd52-as-a-tool-for-the-differential-diagnosis-of-mature-b-cell-neoplasms
#5
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
https://www.readbyqxmd.com/read/28801450/genomic-analysis-of-hairy-cell-leukemia-identifies-novel-recurrent-genetic-alterations
#6
Benjamin H Durham, Bartlomiej Getta, Sascha Dietrich, Justin Taylor, Helen Won, James M Bogenberger, Sasinya Scott, Eunhee Kim, Young Rock Chung, Stephen S Chung, Jennifer Hüllein, Tatjana Walther, Lu Wang, Sydney X Lu, Christopher C Oakes, Raoul Tibes, Torsten Haferlach, Barry S Taylor, Martin S Tallman, Michael F Berger, Jae H Park, Thorsten Zenz, Omar Abdel-Wahab
Classical hairy cell leukemia (cHCL) is characterized by a near 100% frequency of the BRAFV600E mutation while ~30% of variant HCL (vHCL) have MAP2K1 mutations. However, recurrent genetic alterations cooperating with BRAFV600E or MAP2K1 mutations in HCL, as well as those in MAP2K1- wildtype vHCL, are not well defined. We therefore performed deep targeted mutational and copy number analysis of cHCL (n=53) and vHCL (n=8). The most common genetic alteration in cHCL outside of BRAFV600E was heterozygous loss of chromosome 7q, the minimally deleted region of which targeted wildtype BRAF, subdividing cHCL into those hemizygous versus heterozygous for the BRAFV600E mutation...
August 11, 2017: Blood
https://www.readbyqxmd.com/read/28766548/-hairy-cell-leukemia-and-pregnancy
#7
L S Al-Radi, T N Moiseeva, S Yu Smirnova, R G Shmakov
The paper presents experience in following up and treating hairy cell leukemia (HCL) during pregnancy. The combination of HCL and pregnancy was observed in 5 patients. The patients' median age was 35 years (range, 28-42 years). The diagnosis of HCL was based on a conventional examination protocol: clinical blood analysis with the morphological assessment of lymphocytes, a myelogram and trepanobiopsy, immunophenotypic analysis of lymphocytes or bone marrow (in all the patients), cytochemical determination of tartrate-resistant acid phosphatase in 3 patients, and identification of BRAFV600E mutation in 3 patients...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28747289/retrospective-evaluation-of-hairy-cell-leukemia-patients-treated-with-3-different-first-line-treatment-modalities-in-the-last-two-decades-a-single-center-experience
#8
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 analysed the clinical outcome, treatment responses, infectious complications and survival rates of seventy-one hairy cell leukemia (HCL) cases. MATERIALS AND METHODS: Sixty-six patients received a first-line treatment, and 2-chlorodeoxyadenosine (cladribine-2-CdA) was administered in thirty-one, nineteen 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 upfront 2-CdA, ORRs were comparable in 2-CdA, INF-α, and splenectomy subgroups...
July 27, 2017: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/28698853/hairy-cell-leukemia-a-case-report-of-atypical-presentation-without-splenomegaly
#9
Mona Alfaraj, Hussain Alsaeed
No abstract text is available yet for this article.
June 2017: Blood Research
https://www.readbyqxmd.com/read/28664939/clinical-utility-of-recently-identified-diagnostic-prognostic-and-predictive-molecular-biomarkers-in-mature-b-cell-neoplasms
#10
REVIEW
Arantza Onaindia, L Jeffrey Medeiros, Keyur P Patel
Genomic profiling studies have provided new insights into the pathogenesis of mature B-cell neoplasms and have identified markers with prognostic impact. Recurrent mutations in tumor-suppressor genes (TP53, BIRC3, ATM), and common signaling pathways, such as the B-cell receptor (CD79A, CD79B, CARD11, TCF3, ID3), Toll-like receptor (MYD88), NOTCH (NOTCH1/2), nuclear factor-κB, and mitogen activated kinase signaling, have been identified in B-cell neoplasms. Chronic lymphocytic leukemia/small lymphocytic lymphoma, diffuse large B-cell lymphoma, follicular lymphoma, mantle cell lymphoma, Burkitt lymphoma, Waldenström macroglobulinemia, hairy cell leukemia, and marginal zone lymphomas of splenic, nodal, and extranodal types represent examples of B-cell neoplasms in which novel molecular biomarkers have been discovered in recent years...
June 30, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28608720/diagnostic-algorithm-of-common-mature-b-cell-lymphomas-by-immunohistochemistry
#11
Huan-You Wang, Youli Zu
CONTEXT: - Different types of mature B-cell lymphomas, including plasma cell neoplasms, exhibit distinct immunohistochemical profiles, which enable them to be correctly diagnosed. However, except for rare examples of lymphoma-specific immunohistochemistry, such as cyclin D1 in mantle cell lymphoma and annexin A1 in hairy cell leukemia, immunohistochemical profiles of mature B-cell lymphomas overlap and lack specificity. OBJECTIVES: - To systemically review immunohistochemical features associated with commonly encountered mature B-cell lymphomas based on the presence or absence of CD5 and CD10; to review the immunophenotypic profile of plasma cells derived from plasma cell myelomas and B-cell lymphomas; and to review a group of rare, aggressive B-cell lymphomas with antigen expression features of plasma cells...
September 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28603911/cd11c-expression-in-chronic-lymphocytic-leukemia-revisited-related-with-complications-and-survival
#12
E G Umit, M Baysal, Y Durmus, A M Demir
INTRODUCTION: Chronic lymphocytic leukemia (CLL) is a disorder of mature but dysfunctional monoclonal B cells. Microenvironment, antigenic stimulation and genetical mutations are demonstrated in etiopathogenesis. We aimed to evaluate the expression of CD11c in patients with CLL and its possible clinical significance. METHODS: Data of 259 patients with CLL between 2010 and 2016 in Trakya University Faculty of Medicine, including age at diagnosis, sex, whole blood count, stage, percentage of CLL cells in bone marrow, line of treatments, development of Richter's transformation and secondary tumors, autoimmune complications, IgG level, prognostic cytogenetic analysis, and length of survival were recorded from files...
October 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28600336/diagnosis-and-classification-of-hematologic-malignancies-on-the-basis-of-genetics
#13
REVIEW
Justin Taylor, Wenbin Xiao, Omar Abdel-Wahab
Genomic analysis has greatly influenced the diagnosis and clinical management of patients affected by diverse forms of hematologic malignancies. Here, we review how genetic alterations define subclasses of patients with acute leukemias, myelodysplastic syndromes (MDS), myeloproliferative neoplasms (MPNs), non-Hodgkin lymphomas, and classical Hodgkin lymphoma. These include new subtypes of acute myeloid leukemia defined by mutations in RUNX1 or BCR-ABL1 translocations as well as a constellation of somatic structural DNA alterations in acute lymphoblastic leukemia...
July 27, 2017: Blood
https://www.readbyqxmd.com/read/28596664/bullous-pyoderma-gangrenosum-associated-with-hairy-cell-leukemia-and-its-complete-response-to-cladribine-therapy
#14
Ankur Jain, Deepesh Lad, Gaurav Prakash, Alka Khadwal, Pankaj Malhotra, Amanjit Bal, Nabhajit Mallik, Narendar Kumar, Subhash Varma
Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis seen in association with systemic disorders including hematologic malignancies. Hairy cell leukemia (HCL) however, is an unusual association of PG. We describe a 49-year old lady who presented to our hematology clinic with easy fatiguability and ulcerative skin lesions of 6 months duration. Examination revealed pallor and massive splenomegaly. Indurated, ulcerated lesion with undermined edges and necrotic base was observed on left thigh. Investigations revealed pancytopenia and bone marrow examination identified typical hairy cells...
June 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28592763/malignant-lymphoma-pathophysiology-and-current-therapy
#15
Koji Izutsu
Recent advances in the understanding of molecular pathogenesis of lymphoma have enabled us to clearly define disease entity by means of a disease-specific gene mutation and to select candidates for novel targeted therapy. In this review three different clinically relevant topics related to the molecular pathogenesis of lymphoma were covered. In the 2016 revision of the World Health Organization classification of lymphoid malignancies, firstly, disease-specific mutations such as MYD88 L265P in Waldenström macroglobulinemia and BRAF V600E in hairy cell leukemia were incorporated into diagnostic tests, and secondly, the determination of cell-of-origin in diffuse large B-cell lymphoma (DLBCL) was strongly recommended in diagnosis...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28575142/flow-cytometric-patterns-of-cd200-and-cd1d-expression-distinguish-cd10-negative-cd5-negative-mature-b-cell-lymphoproliferative-disorders
#16
Emily F Mason, Olga Pozdnyakova, Betty Li, Graham Dudley, David M Dorfman
Objectives: The importance of distinguishing mature B-cell lymphoproliferative disorders (B-LPDs) is highlighted by the distinct treatments used for and varying prognoses seen in association with these different diseases. Immunophenotyping allows for accurate and efficient differentiation of many B-LPDs. Recently, we showed that CD200 is highly expressed in hairy cell leukemia (HCL) but not in marginal zone lymphoma (MZL), lymphoplasmacytic lymphoma (LPL), or hairy cell leukemia-variant (HCL-v)...
July 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28512562/morphologic-confounders-and-cd19-negativity-in-a-case-of-hairy-cell-leukemia
#17
Pulkit Rastogi, Sreejesh Sreedharanunni, Uday Yanamandra, Man Updesh Singh Sachdeva, Neelam Varma
OBJECTIVES: We report a case of hairy cell leukemia (HCL) initially misdiagnosed as plasma cell dyscrasia due to various clinical, morphological and immunophenotypic confounders. METHODS AND RESULTS: In a patient diagnosed of marrow plasmacytosis and serum monoclonal protein elsewhere and referred to our hospital, morphological evaluation of bone marrow aspirate smears and trephine biopsy, immunophenotyping, and molecular testing (BRAFV600E mutation) were done. Clinically, the patient was asymptomatic; bone marrow revealed plasmacytosis, mastocytosis, and lymphocytosis with a few "hairy" cells...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28512405/concomitant-b-hairy-cell-leukemia-and-mycosis-fungoides-in-an-elderly-man
#18
Eric Wong, Muhammad N Mahmood, Thomas G Salopek
The development of both a T- and B-cell lymphoproliferative disorder in one patient is an unlikely coincidence due to the low prevalence of each malignancy. We report a 65-year-old man with a previously documented history of B hairy cell leukemia, who presented with a new-onset acneiform eruption of his scalp, face, trunk, back, and extremities. Routine pathology of the skin lesions with immunohistochemical stains and molecular studies were consistent with a folliculotropic mycosis fungoides. B hairy cell leukemia and mycosis fungoides occurring in the same patient seems to be a rare phenomenon with only 5 cases reported in the literature...
January 2017: Case Reports in Dermatology
https://www.readbyqxmd.com/read/28512190/hematopoietic-origin-of-langerhans-cell-histiocytosis-and-erdheim-chester-disease-in-adults
#19
Paul Milne, Venetia Bigley, Chris M Bacon, Antoine Néel, Naomi McGovern, Simon Bomken, Muzlifah Haniffa, Eli L Diamond, Benjamin H Durham, Johannes Visser, David Hunt, Harsha Gunawardena, Mac Macheta, Kenneth L McClain, Carl Allen, Omar Abdel-Wahab, Matthew Collin
Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) are rare histiocytic disorders induced by somatic mutation of MAPK pathway genes. BRAF(V600E) mutation is the most common mutation in both conditions and also occurs in the hematopoietic neoplasm hairy cell leukemia (HCL). It is not known if adult LCH or ECD arises from hematopoietic stem cells (HSCs), nor which potential blood borne precursors lead to the formation of histiocytic lesions. In this study, BRAF(V600E) allele-specific polymerase chain reaction was used to map the neoplastic clone in 20 adults with LCH, ECD, and HCL...
July 13, 2017: Blood
https://www.readbyqxmd.com/read/28485619/characteristics-and-treatment-patterns-among-us-patients-with-hairy-cell-leukemia-a-retrospective-claims-analysis
#20
Victoria Divino, Sudeep Karve, Andrew Gaughan, Mitch DeKoven, Guozhi Gao, Kevin B Knopf, Mark C Lanasa
AIM: Describe hairy cell leukemia (HCL) treatment patterns using a large, nationally representative US database. PATIENTS & METHODS: Adults newly diagnosed with HCL (1 January 2006 to 30 June 2014) with continuous health plan enrollment ≥180 days pre- and 90 days post-diagnosis were identified from the QuintilesIMS PharMetrics Plus Health Plan Claims Database. Treatment patterns by line of therapy were assessed over the variable follow-up. RESULTS: Among 749 HCL patients (77...
May 9, 2017: Journal of Comparative Effectiveness Research
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