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https://www.readbyqxmd.com/read/27913534/myelodysplastic-and-myeloproliferative-disorders-of-childhood
#1
Henrik Hasle
Myelodysplastic syndrome (MDS) and myeloproliferative disorders are rare in children; they are divided into low-grade MDS (refractory cytopenia of childhood [RCC]), advanced MDS (refractory anemia with excess blasts in transformation), and juvenile myelomonocytic leukemia (JMML), each with different characteristics and management strategies. Underlying genetic predisposition is recognized in an increasing number of patients. Germ line GATA2 mutation is found in 70% of adolescents with MDS and monosomy 7. It is challenging to distinguish RCC from aplastic anemia, inherited bone marrow failure, and reactive conditions...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913517/treatment-of-low-risk-myelodysplastic-syndromes
#2
Valeria Santini
The majority of myelodysplastic syndrome (MDS) patients belong to the International Prognostic Scoring System (IPSS) and IPSS-revised (IPSS-R) lower-risk categories. Their precise diagnostics and prognostic stratification is often a challenge, but may ensure the optimization of therapy. The availability of diverse treatment options has significantly improved the quality of life and survival of this group of patients. Anemia is the most relevant cytopenia in terms of frequency and symptoms in lower-risk MDS, and may be treated successfully with erythropoietic stimulating agents, provided a careful selection is performed on the basis of IPSS-R, endogenous erythropoietin levels, and transfusion independence...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913467/transplantation-for-bone-marrow-failure-current-issues
#3
Régis Peffault de Latour
The preferred treatment of idiopathic aplastic anemia (AA) is allogeneic hematopoietic stem cell transplantation (HSCT) from a human leukocyte antigen (HLA)-identical sibling donor. Transplantation from a well-matched unrelated donor (MUD) may be considered for patients without a sibling donor after failure of immunosuppressive therapy, as may alternative transplantation (mismatched, cord blood or haplo-identical HSCT) for patients without a MUD. HSCT may also be contemplated for congenital disorders in cases of pancytopenia or severe isolated cytopenia...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913466/nontransplant-therapy-for-bone-marrow-failure
#4
Danielle M Townsley, Thomas Winkler
Nontransplant therapeutic options for acquired and constitutional aplastic anemia have significantly expanded during the last 5 years. In the future, transplant may be required less frequently. That trilineage hematologic responses could be achieved with the single agent eltrombopag in refractory aplastic anemia promotes new interest in growth factors after years of failed trials using other growth factor agents. Preliminary results adding eltrombopag to immunosuppressive therapy are promising, but long-term follow-up data evaluating clonal evolution rates are required before promoting its standard use in treatment-naive disease...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913464/new-challenges-in-evaluating-anemia-in-older-persons-in-the-era-of-molecular-testing
#5
David P Steensma
Anemia is common in older persons, and often remains unexplained despite a thorough clinical history, physical examination, and focused laboratory testing, including marrow aspiration, biopsy, and karyotyping. The advent of molecular genetic testing panels in hematology clinical practice has complicated the evaluation of older patients with unexplained anemia. While the presence of a somatic mutation provides evidence of clonal hematopoiesis and may support a diagnosis of a hematologic neoplasm such as one of the myelodysplastic syndromes (MDS), with rare exceptions, individual mutations are not strongly associated with one specific diagnosis, nor are they by themselves diagnostic of neoplasia...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27902998/autoimmune-cytopenias-diagnosis-management
#6
Christian P Nixon, Joseph D Sweeney
The autoimmune cytopenias are a related group of disorders in which differentiated hematopoietic cells are destroyed by the immune system. Single lineage disease is characterized by the production of autoantibodies against red cells (autoimmune hemolytic anemia [AIHA]), platelets (autoimmune thrombocytopenia [ITP]) and neutrophils (autoimmune neutropenia [AIN]) whereas multilineage disease may include various combinations of these conditions. Central to the genesis of this disease is the breakdown of central and/or peripheral tolerance, and the subsequent production of autoantibodies by both tissue and circulating self-reactive B lymphocytes with support from T helper lymphocytes...
December 1, 2016: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/27902982/limitation-of-simultaneous-analysis-of-t-cell-receptor-and-%C3%AE%C2%BA-deleting-recombination-excision-circles-based-on-multiplex-real-time-polymerase-chain-reaction-in-common-variable-immunodeficiency-patients
#7
Faranaz Atschekzei, Fareed Ahmad, Torsten Witte, Roland Jacobs, Reinhold E Schmidt
AIM OF STUDY: We used a triplex real-time polymerase chain reaction (PCR) to classify our common variable immunodeficiency (CVID) patients into distinct groups according to the amount of their T-cell receptor excision circles (TRECs) and κ-deleting recombination excision circles (KRECs). MATERIALS AND METHODS: TREC and KREC analysis was performed using a multiplex real-time PCR assay. The T- and B-lymphocyte subsets were measured by flow cytometry. RESULTS: The copy number of TRECs and KRECs was significantly reduced in CVID patients compared to healthy controls...
December 1, 2016: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/27902785/apoptosis-related-gene-expression-profiling-in-hematopoietic-cell-fractions-of-mds-patients
#8
Saskia Mc Langemeijer, Niccolo Mariani, Ruth Knops, Christian Gilissen, Rob Woestenenk, Theo de Witte, Gerwin Huls, Bert A van der Reijden, Joop H Jansen
Although the vast majority of patients with a myelodysplastic syndrome (MDS) suffer from cytopenias, the bone marrow is usually normocellular or hypercellular. Apoptosis of hematopoietic cells in the bone marrow has been implicated in this phenomenon. However, in MDS it remains only partially elucidated which genes are involved in this process and which hematopoietic cells are mainly affected. We employed sensitive real-time PCR technology to study 93 apoptosis-related genes and gene families in sorted immature CD34+ and the differentiating erythroid (CD71+) and monomyeloid (CD13/33+) bone marrow cells...
2016: PloS One
https://www.readbyqxmd.com/read/27894982/multiple-opportunistic-infections-in-a-woman-with-gata2-mutation
#9
Andrea Vila, Juan I Dapás, Cynthia V Rivero, Florencia Bocanegra, Roberto F Furnari, Amy P Hsu, Steven M Holland
GATA2 deficiency is a genetic disorder caused by inherited or sporadic haploinsufficient mutations in the GATA2 gene. Patients have abnormalities in hematopoiesis, lymphangiogenesis and immunity; encompassing a broad range of clinical syndromes, mainly characterized by monocytopenia, B and NK cell cytopenia, severe or recurrent infections, and a high risk of developing myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML).We report a case of an Argentinean woman, who presented with multiple opportunistic infections as her first manifestation of GATA2 deficiency...
November 25, 2016: International Journal of Infectious Diseases: IJID
https://www.readbyqxmd.com/read/27891349/unusual-presentation-of-acute-leukaemia-a-tripod-of-cases
#10
Manjari Kishore, Vijay Kumar, Sadhna Marwah, Abhay S Nigam
Acute Leukemia is one of the common haematological malignancies encountered with varied clinical and haematological presentation. In acute leukaemia, complications like bleeding and infection cause significant morbidity and mortality, thus overshadowing the thromboembolic events. Among the various malignant haematological disorders, the association of thromboembolic events is often noted with acute promyelocytic leukemia, though the overall frequency of such events remains very low. Acute Lymphoblastic Leukemia (ALL) is, however, more common than Acute non-lymphoblastic Leukaemia...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27888688/an-oral-hemokine-tm-%C3%AE-methylhydrocinnamate-enhances-myeloid-and-neutrophil-recovery-following-irradiation-in-vivo
#11
Douglas V Faller, Serguei A Castaneda, Daohong Zhou, Merriline Vedamony, Peter E Newburger, Gary L White, Stanley Kosanke, P Artur Plett, Christie M Orschell, Michael S Boosalis, Susan P Perrine
An oral therapeutic which reduces duration of cytopenias and is active following accidental radiation exposures is an unmet need in radiation countermeasures. Alpha methylhydrocinnamate (ST7) prolongs STAT-5 phosphorylation, reduces growth-factor dependency of multi-lineage cell lines, and stimulates erythropoiesis. Here, ST7 and its isomers were studied for their effects on myeloid progenitors and hematopoietic stem cells (HSCs) following radiation, in nonhuman primates, and murine irradiation models. Addition of ST7 or ST7-S increased CFU-GM production by 1...
October 31, 2016: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/27882812/should-we-be-treating-lower-risk-myelofibrosis-patients-with-a-jak2-inhibitor
#12
Guido Lancman, John Mascarenhas
Myelofibrosis (MF) is a Philadelphia chromosome-negative myeloproliferative neoplasm that is associated with debilitating constitutional symptoms, progressive splenomegaly, and cytopenias. Ruxolitinib, a JAK1/2 inhibitor, is currently the only drug approved for the treatment of patients with intermediate or high risk MF. There is rationale and even limited clinical data supporting the use of ruxolitinib in lower risk patients, although this has not yet been validated in a randomized controlled trial. Areas Covered: We examine rationale for using ruxolitinib in lower risk MF patients, including survival data from COMFORT-I and COMFORT-II, specific patient populations that may derive clinical benefit, and the future impact of molecular analysis on risk stratification and treatment...
November 24, 2016: Expert Review of Hematology
https://www.readbyqxmd.com/read/27880061/the-safety-profile-of-monoclonal-antibodies-for-chronic-lymphocytic-leukemia
#13
Anna Korycka-Wołowiec, Dariusz Wołowiec, Tadeusz Robak
Monoclonal antibodies (MoAbs), non-chemotherapeutic agents targeting the antigens present on chronic lymphocytic leukemia (CLL) lymphocytes, are being implemented increasingly more often as treatment options. Areas covered: This article reviews the similarities and differences in the structure, mechanism of action, efficacy and safety profile of commercially-available MoAbs and prevents new agents potentially useful for CLL treatment. Publications in English before June 2016 were surveyed on the MEDLINE database for articles...
November 23, 2016: Expert Opinion on Drug Safety
https://www.readbyqxmd.com/read/27875374/implications-of-molecular-genetic-diversity-in-myelodysplastic-syndromes
#14
Rafael Bejar
PURPOSE OF REVIEW: Myelodysplastic syndromes (MDS) have remarkably diverse somatic mutation patterns that can be challenging to interpret clinically. Yet, genetic information is increasingly available to physicians. This review will examine several implications of genetic diversity in MDS. RECENT FINDINGS: Somatic mutations can serve as clinically relevant biomarkers in MDS. Molecular subtypes may exist that share clinical features including risk of progression to acute myeloid leukemia, response to treatment, and overall survival...
November 21, 2016: Current Opinion in Hematology
https://www.readbyqxmd.com/read/27870251/getting-to-the-core-of-inherited-bone-marrow-failures
#15
Soheir Adam, Dario Melguizo Sanchis, Ghada El-Kamah, Sujith Samarasinghe, Sameer Alharthi, Lyle Armstrong, Majlinda Lako
Bone marrow failure syndromes (BMFS) are a group of disorders with complex pathophysiology characterized by a common phenotype of peripheral cytopenia and/or a hypoplastic bone marrow. Understanding genetic factors contributing to the pathophysiology of BMFS has enabled the identification of causative genes and development of diagnostic tests. To date more than 40 mutations in genes involved in maintenance of genomic stability, DNA repair, ribosome and telomere biology have been identified. In addition, pathophysiological studies have provided insights into several biological pathways leading to the characterization of genotype/phenotype correlations as well as the development of diagnostic approaches and management strategies...
November 21, 2016: Stem Cells
https://www.readbyqxmd.com/read/27866577/novel-developments-in-leukopenia-and-pancytopenia
#16
REVIEW
Chisom Onuoha, Junaid Arshad, John Astle, Mina Xu, Stephanie Halene
Cytopenias are not disease entities in and of themselves; rather, they are the expression of various underlying disease processes. Careful attention to details in patients' presentation, careful history and examination, as well as attention to the ancillary parameters of the complete blood count with a peripheral blood smear can point the clinician toward the appropriate workup. Causes of cytopenias can be inherited or acquired; the latter include medication related, autoimmune, or neoplastic causes. Emergencies need to be recognized in a timely fashion and expert consultation obtained...
December 2016: Primary Care
https://www.readbyqxmd.com/read/27862375/concomitant-mds-with-isolated-5q-deletion-and-mgus-case-report-and-review-of-molecular-aspects
#17
Florian Nolte, Maximilian Mossner, Johann-Christoph Jann, Daniel Nowak, Tobias Boch, Nadine Zoe Müller, Wolf-Karsten Hofmann, Georgia Metzgeroth
Myelodysplastic syndromes (MDS) are considered oligoclonal hematopoietic stem cell disorders resulting in peripheral cytopenias and an increased risk of developing acute myeloid leukemia (AML). Anemia is present in the majority of cases requiring regular blood transfusion in most patients. The WHO subtype of MDS with isolated deletion (5q) is the first cytogenetically determined entity introduced in the classification of the MDS. This article is protected by copyright. All rights reserved.
November 15, 2016: European Journal of Haematology
https://www.readbyqxmd.com/read/27848183/an-atypical-case-of-late-onset-systemic-lupus-erythematosus-with-systemic-lymphadenopathy-and-severe-autoimmune-thrombocytopenia-neutropenia-mimicking-malignant-lymphoma
#18
Keita Tamaki, Satoko Morishima, Sawako Nakachi, Sakiko Kitamura, Sachie Uchibori, Shouhei Tomori, Taeko Hanashiro, Natsuki Shimabukuro, Iori Tedokon, Kazuho Morichika, Yukiko Nishi, Takeaki Tomoyose, Kennosuke Karube, Takuya Fukushima, Hiroaki Masuzaki
Here, we report a rare case of systemic lupus erythematosus (SLE) with conspicuous manifestation of hematological abnormalities. At onset, the 52-year-old male patient showed systemic lymphadenopathy and splenomegaly, severe autoimmune thrombocytopenia, and autoimmune neutropenia. Bone marrow examination and lymph node biopsy excluded the possibility of malignant lymphoma. Based on laboratory findings, he was finally diagnosed with combined autoimmune cytopenia coupled with SLE. Atypical clinical manifestations of SLE prompted us to explore the possibility of autoimmune lymphoproliferative syndrome (ALPS)...
November 15, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27846610/bone-marrow-findings-in-autoimmune-lymphoproliferative-syndrome-with-germline-fas-mutation
#19
Yi Xie, Stefania Pittaluga, Susan Price, Mark Raffeld, Jamie Hahn, Elaine S Jaffe, V Koneti Rao, Irina Maric
Autoimmune lymphoproliferative syndrome is rare genetic disorder characterized by defective FAS-mediated apoptosis, autoimmune disease, accumulation of mature T-cell receptor alpha/beta positive, CD4 and CD8 double-negative T cells and increased risk for lymphoma. Despite frequent hematological abnormalities, literature is scarce regarding the bone marrow pathology in autoimmune lymphoproliferative syndrome. We retrospectively reviewed 3l bone marrow biopsies from a cohort of 240 patients with germline FAS mutation...
October 20, 2016: Haematologica
https://www.readbyqxmd.com/read/27843864/bilateral-painful-parotid-lumps-and-a-lump-in-the-groin-an-uncommon-presentation-of-common-kikuchi-s-disease
#20
Sumeet Prakash Mirgh, Jinendra Satiya, Jehangir Soli Sorabjee
Kikuchi-Fujimoto disease (KFD) is an under-recognized disease most commonly presenting with cervical lymphadenopathy, fever, and cytopenias in young females. Bilateral parotid enlargement is usually caused by infections (e.g., mumps) and autoimmune conditions (e.g., Sjogren syndrome). Parotid enlargement, inguinal lymphadenopathy, and pyrexia of unknown origin are uncommon presenting features of KFD and should be suspected in the appropriate setting.
April 2016: Journal of Family Medicine and Primary Care
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