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Atypical Leukemia

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https://www.readbyqxmd.com/read/27913552/dao-k-ht-tyner-jw-what-s-different-about-atypical-cml-and-chronic-neutrophilic-leukemia-hematology-am-soc-hematol-educ-program-2015-2015-264-271
#1
(no author information available yet)
No abstract text is available yet for this article.
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913526/update-from-the-latest-who-classification-of-mpns-a-user-s-manual
#2
Francesco Passamonti, Margherita Maffioli
The 2016 multiparameter World Health Organization (WHO) classification for Philadelphia-negative myeloproliferative neoplasms (MPNs) integrates clinical features, morphology, and genetic data to diagnose polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). The main novelties are: (1) the reduction of the hemoglobin (Hb) level threshold to diagnose PV, now established at 16.5 g/dL for men and 16 g/dL for women (based on the identification of MPN patients with PV-consistent bone marrow [BM] features and a Hb level lower than that established in the 2008 WHO classification for PV); (2) the recognition of prefibrotic/early PMF, distinguishable from ET on the basis of BM morphology, an entity having a higher tendency to develop overt myelofibrosis or acute leukemia, and characterized by inferior survival; (3) the central role of BM morphology in the diagnosis of ET, prefibrotic/early PMF, PMF, and PV with borderline Hb values; megakaryocyte number and morphology (typical in ET, atypical in both PMF forms) accompanied by a new distinction of reticulin fibrosis grade in PMF (grade 1 in prefibrotic/early PMF and grade 2-3 in PMF) constitute diagnostic criteria; and (4) the inclusion of all mutually exclusive MPN driver mutations (JAK2, CALR, and MPL) as major diagnostic criteria in ET and PMF; 10% to 15% of these patients are triple negative, and in these cases the search for an additional clonal marker (eg, mutations in ASXL1, EZH2, TET2, IDH1/IDH2, SRSF2, and SF3B1) is warranted...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27904177/a-clinicopathological-analysis-of-primary-cutaneous-lymphomas-a-6-year-observational-study-at-a-tertiary-care-center-of-south-india
#3
Anza Khader, Shiny Padinjarayil Manakkad, Mohammed Shaan, Sarita Sasidharan Pillai, Najeeba Riyaz, P Binitha Manikoth, Muhammed Kunnummel, Sunitha Balakrishnan
BACKGROUND: Little data are available concerning clinical and pathological patterns of cutaneous lymphomas in India. AIM: To analyze the clinical and histopathological characteristics of cutaneous lymphomas in Indian patients. MATERIALS AND METHODS: This is a single-center, prospective, observational study carried out from January 1, 2010, to December 31, 2015. The patients underwent clinical examination, human T-cell lymphotropic virus-1 (HTLV-1) screening, skin biopsy with hematoxylin and eosin and immunohistochemistry staining...
November 2016: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/27899359/how-i-treat-atypical-chronic-myeloid-leukemia
#4
Jason Gotlib
Atypical chronic myeloid leukemia, BCR-ABL1-negative (aCML) is a rare myelodysplastic syndrome/myeloproliferative neoplasm for which no current standard of care exists. The challenges of atypical CML relate to its heterogeneous clinical and genetic features, high rate of transformation to acute myeloid leukemia, and historically poor survival. Therefore, allogeneic hematopoietic stem cell transplantation should always be an initial consideration for eligible patients with a suitable donor. Non-transplant approaches for treating aCML have otherwise largely relied on adopting treatment strategies used for MDS and MPN...
November 29, 2016: Blood
https://www.readbyqxmd.com/read/27863543/reduced-humoral-immunity-and-atypical-cell-mediated-immunity-in-response-to-vaccination-in-cows-naturally-infected-with-bovine-leukemia-virus
#5
Meredith C Frie, Kelly R Sporer, Joseph C Wallace, Roger K Maes, Lorraine M Sordillo, Paul C Bartlett, Paul M Coussens
Bovine leukemia virus (BLV) is a retrovirus that is widely distributed across US dairy herds: over 83% of herds are BLV-infected and within-herd infection rates can approach 50%. BLV infection reduces both animal longevity and milk production and can interfere with normal immune health. With such a high prevalence of BLV infection in dairy herds, it is essential to understand the circumstances by which BLV negatively affects the immune system of infected cattle. To address this question, BLV- and BLV+ adult, lactating Holstein dairy cows were vaccinated with Bovi-Shield GOLD(®) FP(®) 5 L5 HB and their immune response to vaccination was measured over the course of 28days...
December 2016: Veterinary Immunology and Immunopathology
https://www.readbyqxmd.com/read/27858857/primary-amenorrhea-after-bone-marrow-transplantation-and-adjuvant-chemotherapy-misdiagnosed-as-disorder-of-sex-development-a-case-report
#6
He Huang, Qinjie Tian
INTRODUCTION: Disorders of sex development (DSD) is a congenital condition in which the development of chromosomal, gonadal or genital sex is atypical. Majority of patients present clinical characteristics of primary amenorrhea, absent secondary sex characters, and abnormal hormone level. A female appearance patient with primary amenorrhea and 46 XY karyotype seems to be solid evidences to diagnose Y-chromosome-related DSD diseases, while it is not necessarily the accurate diagnosis. We report the case of an 18-year-old girl with primary amenorrhea and 46 XY karyotype misdiagnosed as Y-chromosome-related DSD...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27830541/achievement-of-disease-control-with-donor-derived-eb-virus-specific-cytotoxic-t-cells-after-allogeneic-peripheral-blood-stem-cell-transplantation-for-aggressive-nk-cell-leukemia
#7
Shojiro Haji, Motoaki Shiratsuchi, Takamitsu Matsushima, Akiko Takamatsu, Mariko Tsuda, Yasuhiro Tsukamoto, Emi Tanaka, Hirofumi Ohno, Eriko Fujioka, Yuriko Ishikawa, Ken-Ichi Imadome, Yoshihiro Ogawa
Aggressive NK-cell leukemia (ANKL) is characterized by systemic infiltration of Epstein-Barr virus (EBV)-associated natural killer cells and poor prognosis. We report a case of ANKL in which EBV-specific cytotoxic T lymphocytes (CTLs) were induced. A 41-year-old male suffered from fever, pancytopenia, and hepatosplenomegaly. The number of abnormal large granular lymphocytes in the bone marrow was increased and the cells were positive for CD56 and EBV-encoded small nuclear RNAs. The patient was diagnosed with ANKL and achieved a complete response following intensive chemotherapy...
November 9, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27824644/rapid-fatal-acute-peripheral-t-cell-lymphoma-associated-with-igg-plasma-cell-leukemia-and-iga-hypergammaglobulinemia
#8
Nives Jonjić, Irena Seili Bekafigo, Dora Fučkar Čupić, Ksenija Lučin, Antica Duletić Načinović, Toni Valković
Simultaneous occurrence of T-cell and B-cell neoplasms is rare, and etiologic relationships between these 2 malignancies are poorly understood. We describe the case of a 66-year-old woman who was admitted to the hospital because of fever, hemoptysis, lymphadenopathy, and skin rash. Enlarged lymph nodes in axillary, pectoral, paratracheal, and periportal regions as well as slight hepatomegaly and splenomegaly were confirmed. A peripheral blood smear revealed rouleaux formation and numerous circulating plasma cells, with plasmacytoid lymphocytes...
November 2016: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/27807503/clinical-management-of-myelodysplastic-syndrome-myeloproliferative-neoplasm-overlap-syndromes
#9
Joseph A Clara, David A Sallman, Eric Padron
The myelodysplastic/myeloproliferative neoplasms (MDS/MPNs) are a unique group of hematologic malignancies characterized by concomitant myelodysplastic and myeloproliferative features. According to the 2008 WHO classification, the category includes atypical chronic myeloid leukemia (aCML), chronic myelomonocytic leukemia (CMML), juvenile myelomonocytic leukemia (JMML), MDS/MPN-unclassifiable (MDS/MPN-U), and the provisional entity refractory anemia with ring sideroblasts and thrombocytosis (RARS-T). Although diagnosis currently remains based on clinicopathologic features, the incorporation of next-generation platforms has allowed for the recent molecular characterization of these diseases which has revealed unique and complex mutational profiles that support their distinct biology and is anticipated to soon play an integral role in diagnosis, prognostication, and treatment...
September 2016: Cancer Biology & Medicine
https://www.readbyqxmd.com/read/27789332/granulocyte-colony-stimulating-factor-receptor-signaling-in-severe-congenital-neutropenia-chronic-neutrophilic-leukemia-and-related-malignancies
#10
REVIEW
Pankaj Dwivedi, Kenneth D Greis
Granulocyte colony-stimulating factor is a hematopoietic cytokine that stimulates neutrophil production and hematopoietic stem cell mobilization by initiating the dimerization of homodimeric granulocyte colony-stimulating factor receptor. Different mutations of CSF3R have been linked to a unique spectrum of myeloid disorders and related malignancies. Myeloid disorders caused by the CSF3R mutations include severe congenital neutropenia, chronic neutrophilic leukemia, and atypical chronic myeloid leukemia. In this review, we provide an analysis of granulocyte colony-stimulating factor receptor, various mutations, and their roles in the severe congenital neutropenia, chronic neutrophilic leukemia, and malignant transformation, as well as the clinical implications and some perspective on approaches that could expand our knowledge with respect to the normal signaling mechanisms and those associated with mutations in the receptor...
October 24, 2016: Experimental Hematology
https://www.readbyqxmd.com/read/27759688/an-index-case-of-concomitant-tumoral-and-ichthyosiform-mycosis-fungoides-like-presentation-of-chronic-adult-t-cell-leukemia-lymphoma-associated-with-upregulation-of-tox
#11
Giang Huong Nguyen, James Y Wang, Kenneth B Hymes, Cynthia M Magro
Adult T-cell leukemia/lymphoma (ATLL) is a rare and often aggressive lymphoid malignancy known to be associated with human T-cell lymphotropic virus type 1. There are 2 broad categories: acute and chronic. In the acute category, there is a leukemic and a lymphomatous variant, whereas in the designated "chronic" form, there is mild peripheral blood lymphocytosis. The intermediate "smoldering" category is without peripheral blood lymphocytosis with only discernible skin involvement. We present a 68-year-old human T-cell lymphotropic virus type 1 seropositive female with a mild peripheral blood atypical lymphocytosis who had indurated nodules on her hands of 2 years duration and a new scaly ichthyosiform eruption on her lower extremities...
October 12, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27752371/myeloproliferative-neoplasm-or-reactive-process-a-rare-case-of-acute-myeloid-leukemia-and-transient-posttreatment-megakaryocytic-hyperplasia-with-jak-2-mutation
#12
Steven Wang, Jie Yan, Guangde Zhou, Rebecca Heintzelman, J Steve Hou
Myeloproliferative neoplasms (MPNs) are hematopoietic malignancies characterized by unchecked proliferation of differentiated myeloid cells. The most common BCR-ABL1-negative MPNs are polycythemia vera, essential thrombocythemia, and primary myelofibrosis. The discovery of JAK2 V617F mutation has improved our understanding of the molecular basis of MPN. The high frequency of JAK2 mutation in MPN makes JAK2 mutation testing an essential diagnostic tool and potential therapeutic target for MPN. Here, we present a rare case of a 34-year-old patient who was initially diagnosed with acute myeloid leukemia (AML) with mutated NPM1...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27721761/an-atypical-initial-presentation-of-chronic-myeloid-leukemia-with-central-nervous-system-and-lymph-node-blast-crises
#13
Khadega A Abuelgasim, Saeed Alshieban, Nada A Almubayi, Ayman Alhejazi, Abdulrahman R Jazieh
We describe the case of a young man with therapy-naive chronic myeloid leukemia who did not initially have any peripheral blood or bone marrow excess blasts but presented with extramedullary myeloid blast crises involving the central nervous system and multiple lymph nodes. Conventional cytogenetic tests were positive for t(9;22)(q34:q11) as well as for trisomy 8, 14 and 21 and del(16q). The patient's peripheral blood and bone marrow were positive for the BCR-ABL oncogene when analyzed by fluorescence in situ hybridization and polymerase chain reaction...
May 2016: Case Reports in Oncology
https://www.readbyqxmd.com/read/27708445/acute-myeloid-leukemia-with-basophilic-differentiation-in-a-3-year-old-standardbred-gelding
#14
Mary Catherine Furness, Emile Setlakwe, John Sallaway, Darren Wood, Jordan Fromstein, Luis G Arroyo
A 3-year-old Standardbred gelding with a history of pyrexia, persistent hemorrhage from the oral cavity, and a large, soft swelling at the junction of the caudal aspect of the mandibular rami and proximal neck was evaluated. The horse had neutropenia and anemia, with atypical granulated cells in a blood smear. Additional tests confirmed acute myeloid leukemia with basophilic differentiation, which has been reported in humans, cats, dogs, and cattle but not horses.
October 2016: Canadian Veterinary Journal. la Revue Vétérinaire Canadienne
https://www.readbyqxmd.com/read/27701759/spurious-reticulocyte-profiles-in-dogs-with-large-form-babesiosis-a-retrospective-study
#15
Laetitia Piane, Karen M Young, Lena Giraud, Nathalie Bourges-Abella, Catherine Trumel
BACKGROUND: Erroneously high reticulocyte counts (pseudoreticulocytosis) have been reported in dogs with leukemia. Pseudoreticulocytosis and an abnormal reticulocyte profile were observed in a dog with large form babesiosis presented at our institution. OBJECTIVES: The aims of this retrospective study were to determine if dogs with babesiosis and other dogs had abnormal reticulocyte profiles, and to correlate these profiles with the primary diagnosis. METHODS: All canine CBCs obtained with the Sysmex XT-2000iV or Procyte DX were reviewed...
October 4, 2016: Veterinary Clinical Pathology
https://www.readbyqxmd.com/read/27664113/an-exercise-in-extrapolation-clinical-management-of-atypical-cml-mds-mpn-unclassifiable-and-mds-mpn-rs-t
#16
Chetasi Talati, Eric Padron
According to the recently published 2016 World Health Organization (WHO) classification of myeloid malignancies, myelodysplastic/myeloproliferative neoplasms (MDS/MPN) include atypical chronic myeloid leukemia (aCML), MDS/MPN-unclassifiable (MDS/MPN-U), chronic myelomonocytic leukemia (CMML), juvenile myelomonocytic leukemia (JMML), and MDS/MPN ring sideroblasts with thrombocytosis (MDS/MPN-RS-T). MDS/MPN-RS-T was previously a provisional category known as refractory anemia with ring sideroblasts with thrombocytosis (RARS-T) which has now attained a distinct designation in the 2016 WHO classification...
September 24, 2016: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/27626202/the-nedd8-activating-enzyme-inhibitor-pevonedistat-activates-the-eif2%C3%AE-and-mtor-pathways-inducing-upr-mediated-cell-death-in-acute-lymphoblastic-leukemia
#17
Gilles M Leclerc, Shuhua Zheng, Guy J Leclerc, Joanna DeSalvo, Ronan T Swords, Julio C Barredo
Acute lymphoblastic leukemia (ALL) is the leading cause of cancer-related death in children, and cure rates for adults remain dismal. Further, effective treatment strategies for relapsed/refractory ALL remain elusive. We previously uncovered that ALL cells are prone to apoptosis via endoplasmic reticulum (ER) stress/unfolded protein response (UPR)-mediated mechanisms. We investigated the antineoplastic activity of pevonedistat(®), a novel NEDD8-activating enzyme inhibitor that targets E3 cullin-RING ligases (CRLs) dependent proteasomal protein degradation, in ALL...
September 5, 2016: Leukemia Research
https://www.readbyqxmd.com/read/27620005/viral-associated-diarrhea-in-immunocompromised-and-cancer-patients-at-a-large-comprehensive-cancer-center-a-10-year-retrospective-study
#18
Natasha Ghosh, Farida A Malik, Roshni G Daver, Jakapat Vanichanan, Pablo C Okhuysen
BACKGROUND: Viral associated diarrhea (VAD) due to Norovirus (NV), Rotavirus (RV) and Adenovirus (AV) is common in immunocompromised and cancer patients. We sought to determine if the clinical characteristics, morbidity and seasonality of infection differed according to the type of enteric virus identified. METHODS: Cases of NV, RV and AV were identified in stool specimens submitted to the clinical microbiology laboratory between November 2005 and February 2015...
September 13, 2016: Infectious Diseases
https://www.readbyqxmd.com/read/27602777/mast-cell-sarcoma-new-cases-and-literature-review
#19
Jilliana Monnier, Sophie Georgin-Lavialle, Danielle Canioni, Ludovic Lhermitte, Michael Soussan, Michel Arock, Julie Bruneau, Patrice Dubreuil, Christine Bodemer, Marie-Olivia Chandesris, Olivier Lortholary, Olivier Hermine, Gandhi Damaj
Mast cell sarcoma (MCS) is a rare form of mastocytosis characterized by the presence of solid tumor(s) comprising malignant mast cells that harbor destructive infiltration capability and metastatic potential. Here, we present an extensive literature review and report on 23 cases of MCS, including 3 new cases from the French National Reference Center for Mastocytosis. From our analysis, it appears that MCS can occur at any age. It can manifest de novo or, to a lesser extent, may evolve from a previously established mastocytosis...
September 1, 2016: Oncotarget
https://www.readbyqxmd.com/read/27566498/leukemia-cutis-an-atypical-case
#20
Matteo Coen, Jacques Serratrice
No abstract text is available yet for this article.
December 2016: American Journal of Medicine
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