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Atypical Leukemia

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https://www.readbyqxmd.com/read/29468060/identification-of-i-x-p10-as-the-sole-molecular-abnormality-in-atypical-chronic-myeloid-leukemia-evolved-into-acute-myeloid-leukemia
#1
Carmelo Gurnari, Paola Panetta, Emiliano Fabiani, Anna Maria Nardone, Diana Postorivo, Giulia Falconi, Luca Franceschini, Manuela Rizzo, Vito Mario Rapisarda, Eleonora De Bellis, Francesco Lo-Coco, Maria Teresa Voso
The World Health Organization classifies atypical chronic myeloid leukemia (aCML) as a myeloproliferative/myelodisplastic hematological disorder. The primary manifestations are leukocytosis with disgranulopoiesis, absence of basophilia and/or monocytosis, splenomegaly and absence of Philadelphia chromosome or BCR/ABL fusion. Overall 50-65% of patients demonstrate karyotypic abnormalities, although no specific cytogenetic alterations have been associated with this disease. X chromosome alterations have been rarely reported in myeloid malignancies...
March 2018: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/29458385/de-novo-mast-cell-leukemia-without-cd25-expression-and-kit-mutations-a-rare-case-report-in-a-13-year-old-child
#2
Yalin Zheng, Lin Nong, Li Liang, Wei Wang, Ting Li
BACKGROUND: Mast cell leukemia (MCL) is a very rare form of systemic mastocytosis (SM) and accounts for less than 0.5% of all mastocytosis. The diagnosis of MCL requires the presence of SM criteria, accompanied by leukemic infiltrating of atypical mast cells (MCs) in bone marrow (BM), peripheral blood as well as extracutaneous organs. MCL is a fatal disease that almost always behaves aggressively, and the median survival time is only about six months. Herein, we present a rare case of de novo MCL without CD25 expression and KIT mutations...
February 20, 2018: Diagnostic Pathology
https://www.readbyqxmd.com/read/29455651/targeting-few-to-help-hundreds-jak-mapk-and-rock-pathways-as-druggable-targets-in-atypical-chronic-myeloid-leukemia
#3
REVIEW
Stefania Rocca, Giovanna Carrà, Pietro Poggio, Alessandro Morotti, Mara Brancaccio
Atypical Chronic Myeloid Leukemia (aCML) is a myeloproliferative neoplasm characterized by neutrophilic leukocytosis and dysgranulopoiesis. From a genetic point of view, aCML shows a heterogeneous mutational landscape with mutations affecting signal transduction proteins but also broad genetic modifiers and chromatin remodelers, making difficult to understand the molecular mechanisms causing the onset of the disease. The JAK-STAT, MAPK and ROCK pathways are known to be responsible for myeloproliferation in physiological conditions and to be aberrantly activated in myeloproliferative diseases...
February 19, 2018: Molecular Cancer
https://www.readbyqxmd.com/read/29404266/necrotizing-fungal-gingivitis-in-a-patient-with-acute-myelogenous-leukemia-visible-yet-obscure
#4
Prajwal Boddu, Pei-Ling Chen, Priyadharsini Nagarajan, Victor G Prieto, Alex Won, Mark Chambers, Steven Kornblau
Oral fungal infections present with atypical and varied manifestations, and distinguishing them from other entities including leukemic infiltration can be diagnostically challenging. In this report, we describe a 62 year old female with acute myeloid leukemia who presented, towards the end of her second treatment cycle of decitabine in a prolonged neutropenic state, with a month of painful, necrotic-appearing marginal gingival lesions. She was duly initiated on empiric broad spectrum antifungal treatment but did not show a clinical response with the appearance of new skin lesions concerning for progressive fungemia...
January 2018: Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology
https://www.readbyqxmd.com/read/29390324/successful-treatment-with-allogeneic-stem-cell-transplantation-followed-by-dli-and-tkis-for-e6a2-bcr-abl-positive-acute-myeloid-leukaemia-a-case-report-and-literature-review
#5
Yasuhiko Harada, Satoshi Nishiwaki, Takumi Sugimoto, Koichi Onodera, Tatsunori Goto, Takahiko Sato, Sonoko Kamoshita, Naomi Kawashima, Aika Seto, Shingo Okuno, Satomi Yamamoto, Toshihiro Iwasaki, Yukiyasu Ozawa, Koichi Miyamura, Yoshiki Akatsuka, Isamu Sugiura
RATIONALE: Patients with the e6a2 BCR-ABL transcript, 1 of the atypical transcripts, have been reported to have a poor prognosis, and allogeneic stem cell transplantation (ASCT) can be considered as additional therapy. However, long-term survival after ASCT for this disease is rare. PATIENT CONCERNS: This report concerns a 55-year-old female patient with e6a2 BCR-ABL-positive acute myeloid leukemia including the outcome of ASCT followed by donor lymphocyte infusion (DLI)...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29367872/photoletter-to-the-editor-atypical-primary-cutaneous-mucormycosis-of-the-scalp
#6
Ricardo García-Sepúlveda, Josefina Navarrete-Solís, Hiram Villanueva-Lozano, Rogelio de J Treviño-Rangel, Gloria M González, Jorge Enríquez-Rojas, Javier Molina-Durazo, Roberto Arenas-Guzmán
Mucormycosis of the scalp is a rare cutaneous presentation of the disease. It is also an unusual infection in children. We present the case of a 4-year-old girl with acute lymphoblastic leukemia, who presented with atypical cutaneous mucormycosis simulating an ecthyma gangrenosum lesion. Risk factors for the infection are diabetes, neoplastic diseases, immunosuppression in organ transplant recipients, and neutropenia. The cutaneos forms have been associated with trauma, burns and surgical wounds. First line treatment is amphotericin B...
December 1, 2017: Journal of Dermatological Case Reports
https://www.readbyqxmd.com/read/29344492/bilateral-hypopyon-uveitis-in-chronic-myeloid-leukemia
#7
Mudit Tyagi, Vishal Govindhari, Rajeev R Pappuru, Vikas Ambiya
Background: A leukemic hypopyon is considered an early sign of central nervous system involvement or systemic relapse. A differential diagnosis of masquerade syndromes should be considered in cases of hypopyon uveitis that are atypical or unresponsive to treatment. We report a case of a 45-year-old man who presented with bilateral hypopyon uveitis and was subsequently diagnosed as having chronic myeloid leukemia. Method: Retrospective case review. Results: A 45-year-old diabetic male presented with diminished vision in both eyes for 10 days...
December 2017: Ocular Oncology and Pathology
https://www.readbyqxmd.com/read/29326799/molecular-heterogeneity-in-acute-promyelocytic-leukemia-a-single-center-experience-from-india
#8
Nikhil Rabade, Goutham Raval, Shruti Chaudhary, P G Subramanian, Rohan Kodgule, Swapnali Joshi, Prashant Tembhare, Syed K Hasan, Hasmukh Jain, Manju Sengar, Gaurav Narula, Shripad Banavali, Pratibha Amare Kadam, Dhanalaxmi Shetty, Sumeet Gujral, Nikhil Patkar
Atypical breakpoints and variant APL cases involving alternative chromosomal aberrations are seen in a small subset of acute promyelocytic leukemia (APL) patients. Over seven different partner genes for RARA have been described. Although rare, these variants prove to be a diagnostic challenge and require a combination of advanced cytogenetic and molecular techniques for accurate characterization. Heterogeneity occurs not only at the molecular level but also at clinico-pathological level influencing treatment response and outcome...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29326798/late-onset-hepatic-veno-occlusive-disease-after-allografting-report-of-two-cases-with-atypical-clinical-features-successfully-treated-with-defibrotide
#9
Alessia Castellino, Stefano Guidi, Chiara Maria Dellacasa, Antonella Gozzini, Irene Donnini, Chiara Nozzoli, Sara Manetta, Semra Aydin, Luisa Giaccone, Moreno Festuccia, Lucia Brunello, Enrico Maffini, Benedetto Bruno, Ezio David, Alessandro Busca
Hepatic Veno-Occlusive Disease (VOD) is a potentially severe complication of hematopoietic stem cell transplantation (HSCT). Here we report two patients receiving an allogeneic HSCT who developed late onset VOD with atypical clinical features. The two patients presented with only few risk factors, namely, advanced acute leukemia, a myeloablative busulphan-containing regimen and received grafts from an unrelated donor. The first patient did not experience painful hepatomegaly and weight gain and both patients showed only a mild elevation in total serum bilirubin level...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29280197/fulminant-cryptococcus-neoformans-infection-with-fatal-pericardial-tamponade-in-a-patient-with-chronic-myelomonocytic-leukemia-who-was-treated-with-ruxolitinib-case-report-and-review-of-fungal-pericarditis
#10
Jing Liu, Elie Mouhayar, Jeffrey J Tarrand, Dimitrios P Kontoyiannis
Cryptococcus neoformans is a saprophytic fungal pathogen that can cause serious illness in immune-compromised hosts and it presents with a wide variety of clinical symptoms. We present a fatal case of fulminant C. neoformans infection presenting as pericardial tamponade in a 71-year-old male with chronic myelomonocytic leukemia undergoing chemotherapy with the JAK-STAT inhibitor ruxolitinib. We also review the published cases of fungal pericarditis/tamponade. In addition to illustrating an atypical presentation of C...
December 27, 2017: Mycoses
https://www.readbyqxmd.com/read/29273895/smear-campaign-misattribution-of-pancytopenia-to-a-tick-borne-illness
#11
Jessica Lee, Soraya Azzawi, Michael J Peluso, Aaron Richterman, Haiyan Ramirez Batlle, Maria A Yialamas
We report the case of a 51-year-old woman presenting with a targetoid rash and pancytopenia after a tick bite. Initial evaluation was notable for severe neutropenia on the complete blood cell count differential, a positive Lyme IgM antibody, and a peripheral blood smear demonstrating atypical lymphocytes. While her pancytopenia was initially attributed to tick-borne illness, peripheral flow cytometry showed 7% myeloblasts, and a bone marrow biopsy confirmed 60% blasts. The patient was ultimately diagnosed with acute myelogenous leukemia, in addition to early, localized Lyme disease...
December 22, 2017: Journal of General Internal Medicine
https://www.readbyqxmd.com/read/29227326/sudden-intracranial-hemorrhage-in-a-patient-with-atypical-chronic-myeloid-leukemia-in-chronic-phase
#12
Kagehiro Kouzuki, Katsutsugu Umeda, Satoshi Saida, Itaru Kato, Hidefumi Hiramatsu, Takeshi Funaki, Kenji Kanda, Hideki Muramatsu, Kenichi Yoshida, Seishi Ogawa, Souichi Adachi
A 16-year-old boy was incidentally found to have hyperleukocytosis during a school physical examination. He was diagnosed with atypical chronic myeloid leukemia in chronic phase. Although treatment with hydoxyurea was started, his white blood cell count increased and he eventually developed lethal intracranial hemorrhage. Although very rare, intracranial hemorrhage should be considered as a possible complication in patients with atypical chronic myeloid leukemia, even in chronic phase, if they have hyperleukocytosis and thrombocytopenia...
December 8, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29226717/atypical-chronic-myeloid-leukemia-a-rare-entity-with-management-challenges
#13
Prajwal Dhakal, Krishna Gundabolu, Catalina Amador, Supratik Rayamajhi, Vijaya Raj Bhatt
The aim of our study was to review the clinicopathologic features and management of atypical chronic myeloid leukemia (aCML). Relevant manuscripts published in English were searched using PubMed. aCML is diagnosed as per WHO 2016 classification in the presence of leukocytosis ≥13 × 109/l with circulating neutrophil precursors ≥10%, monocytes less than 10%, minimal basophils, hypercellular bone marrow with granulocytic proliferation and dysplasia, bone marrow blast less than 20% and absence of BCR/ABL fusion gene...
December 11, 2017: Future Oncology
https://www.readbyqxmd.com/read/29225884/setbp1-mutations-as-a-biomarker-for-myelodysplasia-myeloproliferative-neoplasm-overlap-syndrome
#14
REVIEW
Katherine Linder, Chaitanya Iragavarapu, Delong Liu
Myelodysplasia (MDS) /myeloproliferative neoplasm (MPN) overlap syndrome has been described since the 2001 WHO classification as disorders that have both proliferative and dysplastic changes simultaneously. Specific disorders include chronic myelomonocytic leukemia (CMML), juvenile myelomonocytic leukemia (JMML), BCR-ABL negative atypical chronic myeloid leukemia (aCML) and unclassifiable MDS/MPN (MPN/MDS-U). Recurrent gene mutations in these conditions have been described. Among them, SETBP1 mutations have been identified in up to 32% of aCML, 24% of JMML, 18% of CMML and 10% of MDS/MPN-U patients...
2017: Biomarker Research
https://www.readbyqxmd.com/read/29208261/recurrent-malignancy-associated-atypical-neutrophilic-dermatosis-with-noninfectious-shock
#15
REVIEW
Kathryn Nicole Kinser, Kamaldeep Panach, Arturo Ricardo Dominguez
Sweet syndrome (SS) or acute febrile neutrophilic dermatosis presents with the sudden onset of fever, leukocytosis and tender, erythematous, edematous, well-demarcated papules and plaques that histopathologically demonstrate a dense neutrophilic infiltrate. A total of 20% of patients with SS have malignancy-associated disease that can present with bullous or atypical skin lesions that mimic pyoderma gangrenosum, another neutrophilic dermatosis. Both entities exist on a spectrum, and in the context of underlying malignancy, these neutrophilic diseases become less clinically distinct...
December 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/29203034/atypical-presentation-of-herpes-simplex-virus-type-2-infection-refractory-to-treatment-with-acyclovir-in-2-hematologic-patients
#16
D Nieto Rodríguez, E Sendagorta Cudós, J M Rueda Carnero, P Herranz Pinto
Herpesvirus infections are not uncommon in hematologic patients. Our first patient, diagnosed with chronic lymphatic leukemia, presented extensive genital herpes infection refractory to treatment with acyclovir and with a partial response to foscarnet, which had to be withdrawn due to systemic adverse effects. The second patient, diagnosed with follicular Hodgkin lymphoma, presented hypertrophic herpes infection refractory to treatment with acyclovir but that responded to intralesional cidofovir and topical imiquimod...
December 1, 2017: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/29192651/myeloid-neoplasms-with-features-intermediate-between-primary-myelofibrosis-and-chronic-myelomonocytic-leukemia
#17
Jennifer Chapman, Julia T Geyer, Mahsa Khanlari, Adrienne Moul, Carmen Casas, Scot T Connor, Yao-Shan Fan, Justin M Watts, Ronan T Swords, Francisco Vega, Attilio Orazi
Monocytosis can develop during disease course in primary myelofibrosis simulating that seen in chronic myelomonocytic leukemia, and should not lead to disease reclassification. In contrast, at presentation, rare cases have clinical, morphologic, and molecular genetic features truly intermediate between primary myelofibrosis and chronic myelomonocytic leukemia. The taxonomy and natural history of these diseases are unclear. We identified cases which either: (1) fulfilled the 2008 World Health Organization criteria for primary myelofibrosis but had absolute monocytosis and, when available, chronic myelomonocytic leukemia-related mutations (ASXL1, SRSF2, TET2) or (2) fulfilled criteria of chronic myelomonocytic leukemia but had megakaryocytic proliferation and atypia, marrow fibrosis, and myeloproliferative-type driver mutations (JAK2, MPL, CALR)...
December 1, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29123930/chronic-myeloid-leukemia-with-an-e6a2-bcr-abl1-fusion-transcript-cooperating-mutations-at-blast-crisis-and-molecular-monitoring
#18
Mireille Crampe, Karl Haslam, Emma Groarke, Eileen Kelleher, Derville O'Shea, Eibhlin Conneally, Stephen E Langabeer
A minority of chronic myeloid leukemia patients (CML) express a variety of atypical BCR-ABL1 fusion variants and, of these, the e6a2 BCR-ABL1 fusion is generally associated with an aggressive disease course. Progression of CML to blast crisis is associated with acquisition of additional somatic mutations yet these events have not been elucidated in patients with the e6a2 BCR-ABL1 genotype. Moreover, molecular monitoring is only sporadically performed in CML patients with atypical BCR-ABL1 fusion transcripts due to lack of consensus approaches or standardization...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/29107664/eruption-of-lymphocyte-recovery-with-atypical-lymphocytes-mimicking-a-primary-cutaneous-t-cell-lymphoma-a-series-of-12-patients
#19
Charlotte Hurabielle, Emilie Sbidian, Helmut Beltraminelli, Brigitte Bouchindhomme, Catherine Chassagne-Clément, Brigitte Balme, Céline Bossard, Marie-Hélène Delfau-Larue, Pierre Wolkenstein, Olivier Chosidow, Catherine Cordonnier, Andrea Toma, Cécile Pautas, Nicolas Ortonne
Eruption of lymphocyte recovery (ELR) may occur during bone marrow aplasia after chemotherapies. We reviewed the clinical and pathologic features of 12 patients (male/female: 7/5, median age: 61years) with an atypical ELR histologically mimicking a primary cutaneous T-cell lymphoma (CTCL) such as Sézary Syndrome or CD30+ T-cell lymphoproliferative disorder (LPD). All the patients displayed an erythematous maculopapular eruption on the trunk and the limbs, associated with fever. All but one had received a polychemotherapy for an acute myeloid leukemia (n=10) or an urothelial carcinoma (n=1) before the occurrence of the skin eruption...
October 28, 2017: Human Pathology
https://www.readbyqxmd.com/read/29091516/bosutinib-versus-imatinib-for-newly-diagnosed-chronic-myeloid-leukemia-results-from-the-randomized-bfore-trial
#20
Jorge E Cortes, Carlo Gambacorti-Passerini, Michael W Deininger, Michael J Mauro, Charles Chuah, Dong-Wook Kim, Irina Dyagil, Nataliia Glushko, Dragana Milojkovic, Philipp le Coutre, Valentin Garcia-Gutierrez, Laurence Reilly, Allison Jeynes-Ellis, Eric Leip, Nathalie Bardy-Bouxin, Andreas Hochhaus, Tim H Brümmendorf
Purpose Bosutinib is a potent dual SRC/ABL kinase inhibitor approved for adults with Philadelphia chromosome-positive chronic myeloid leukemia (CML) resistant and /or intolerant to prior therapy. We assessed the efficacy and safety of bosutinib versus imatinib for first-line treatment of chronic-phase CML. Methods In this ongoing, multinational, phase III study, 536 patients with newly diagnosed chronic-phase CML were randomly assigned 1:1 to receive 400 mg of bosutinib once daily (n = 268) or imatinib (n = 268)...
November 1, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
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