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https://www.readbyqxmd.com/read/28654467/a-young-woman-with-an-erythematous-rash-fever-tachycardia-and-pancytopenia
#1
Rachel McAndrew, Lindsay Thelin, Ammar Ahmed
No abstract text is available yet for this article.
June 20, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28649693/from-febrile-pancytopenia-to-hemophagocytic-lymphohistiocytosis-associated-organ-dysfunction
#2
Joseph A Carcillo, Bradley Podd, Dennis W Simon
No abstract text is available yet for this article.
June 26, 2017: Intensive Care Medicine
https://www.readbyqxmd.com/read/28637584/decreased-tim-3-expression-of-peripheral-blood-natural-killer-cells-in-patients-with-severe-aplastic-anemia
#3
Tian Zhang, Xin Yuan, Chunyan Liu, Yi Li, Hui Liu, Lijuan Li, Kai Ding, Ting Wang, Honglei Wang, Zonghong Shao, Rong Fu
Severe aplastic anemia (SAA) is an autoimmune disease characterized by severe pancytopenia and bone marrow failure. In our previous studies, we found natural killer (NK) cells were aberrant in SAA patients. T cell immunoglobulin mucin-3 (TIM-3), an important regulator of immunity, is widely detected on NK cells and may contribute as a marker of activation and maturation of NK cells. In this study, we found that SAA untreated patients had lower TIM-3 expression on NK cells and CD56(dim) NK subsets compared with normal controls, and were correlated with the severity of pancytopenia of SAA...
April 24, 2017: Cellular Immunology
https://www.readbyqxmd.com/read/28628471/haematological-involvement-associated-with-a-mild-autoinflammatory-phenotype-in-two-patients-carrying-the-e250k-mutation-of-pstpip1
#4
Elena Belelli, Chiara Passarelli, Manuela Pardeo, Dirk Holzinger, Fabrizio De Benedetti, Antonella Insalaco
OBJECTIVES: Hyperzincaemia/hypercalprotectinemia (Hz/Hc) syndrome is a recently described condition caused by a specific de novo mutation (E250K) affecting PSTPIP1 gene. It has a phenotype distinct from classical pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome that includes severe systemic and cutaneous inflammation, hepatosplenomegaly, arthritis without sequelae, pancytopenia and failure to thrive. METHODS: We describe an 8-year-old boy who presented recurrent right knee swelling mimicking septic arthritis and persistent bone marrow involvement, without cutaneous involvement...
June 19, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28626190/miliary-tuberculosis-in-a-young-woman-with-hemophagocytic-syndrome-a-case-report-and-literature-review
#5
Mina Asaji, Kazunori Tobino, Koujin Murakami, Yuki Goto, Takuto Sueyasu, Saori Nishizawa, Kohei Yoshimine, Miyuki Munechika, Yuki Ko, Yuki Yoshimatsu, Kosuke Tsuruno, Hiromi Ide, Hiroyuki Miyajima, Noriyuki Ebi
We herein report a rare case of miliary tuberculosis-associated hemophagocytic syndrome (HPS) complicated with respiratory failure. A 19-year-old Japanese woman with a fever, general malaise, and chest radiograph abnormalities was referred to our hospital. After admission, she developed respiratory failure with pancytopenia. A histological examination of lung and bone marrow biopsy samples revealed noncaseating granulomas without evidence of acid-fast bacilli or lymphoma. In addition, a bone marrow biopsy showed marked histiocyte hyperplasia with hemophagocytosis, and a bronchoalveolar lavage fluid culture grew Mycobacterium tuberculosis...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28626188/discoid-lupus-erythematosus-complicated-with-pregnancy-induced-hemophagocytic-syndrome
#6
Hideto Takada, Naoki Kimura, Yoko Yoshihashi-Nakazato, Kimito Kawahata, Hitoshi Kohsaka
A 35-year-old woman with discoid lupus erythematosus (DLE) was admitted at 11 weeks' gestation with a persistent fever. Laboratory studies revealed pancytopenia, elevated liver enzymes, and hyperferritinemia. Bone marrow aspiration confirmed the diagnosis of hemophagocytic syndrome (HPS). She had no findings of infection or active systemic lupus erythematosus. The administration of high-dose corticosteroids resolved the clinical and laboratory findings. She delivered a healthy baby at 35 weeks' gestation. This case suggests that DLE can be a predisposing factor for pregnancy-induced HPS...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28620611/dogs-with-acute-myeloid-leukemia-have-clonal-rearrangements-in-t-and-b-cell-receptors
#7
Tracy Stokol, Gabrielle A Nickerson, Martha Shuman, Nicole Belcher
Clonality testing for rearrangements in the complementarity-determining region 3 of the immunoglobulin heavy chain of B lymphocytes (B cell receptor) and the T cell receptor of T lymphocytes helps distinguish between clonal and non-clonal expansions of lymphocytes. There are rare reports of clonally rearranged T and B cell receptors in dogs with acute myeloid leukemia (AML). Our objective was to determine the frequency of clonally rearranged T and B cell receptors in dogs with AML. Archived slides from historical cases of AML (from January 2010 to June 2013) and slides or liquid specimens [blood, bone marrow (BM), body cavity fluid, or tissue aspirates] from cases of AML diagnosed between June 2013 and February 2017 were used in the study...
2017: Frontiers in Veterinary Science
https://www.readbyqxmd.com/read/28620416/antiphospholipid-syndrome-multiple-manifestations-in-a-single-patient-a-high-suspicion-is-still-needed
#8
Uroosa Ibrahim, Shiksha Kedia, Gwenalyn Garcia, Jean Paul Atallah
Antiphospholipid Syndrome (APS) is an autoimmune disorder with clinical and laboratory features of vascular thrombosis, pregnancy loss, and persistent antiphospholipid antibodies (aPLs). The pathophysiology is thought to involve the activation of endothelial cells, monocytes, platelets, and complement by aPLs. Disease can range from asymptomatic to rapidly fatal catastrophic APS. We present a case of a 34-year-old male referred for pancytopenia and splenomegaly. On examination, he had decreased sensation and 4/5 power in the left upper extremity...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28598057/macrophage-activation-syndrome
#9
P Babu Raj, B L Harikrishnan, Roshan Mampilly, R Anand
Macrophage Activation Syndrome (MAS) occurs as a severe life-threatening complication of several chronic rheumatic diseases. It is more frequent with systemic onset juvenile arthritis and adult onset Still's disease.1 It can be primary, infection related, malignancy associated or autoimmune3. We report a case of Macrophage Activation Syndrome presenting as pyrexia of unknown origin (PUO) and pancytopenia in the absence of any known triggering factor.
May 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28596664/bullous-pyoderma-gangrenosum-associated-with-hairy-cell-leukemia-and-its-complete-response-to-cladribine-therapy
#10
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/28590296/18f-fdg-superscan-caused-by-extensive-bone-marrow-involvement-in-hemophagocytic-lymphohistiocytosis
#11
W Phillip Law, Shannon Emmett, Peter Jackson
An 82-year-old woman who initially presented with pulmonary infection had persistent pancytopenia, which presented a diagnostic dilemma requiring multiple bone marrow biopsies for eventual diagnosis of hemophagocytic lymphohistiocytosis. This case highlights the utility of FDG PET in (a) focusing attention on the bone marrow and reticuloendothelial system as the primary site of pathology and (b) excluding underlying malignancy and infection in this rare but potentially fatal hyperinflammatory condition caused by a highly stimulated but ineffective immune response...
June 5, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28588869/mycobacterium-genavense-invading-the-bone-marrow-in-a-hiv-positive-patient
#12
Christianne Bourlon, Cesar Vargas-Serafín, Xavier López-Karpovitch
Nontuberculous mycobacteria infrequently cause disseminated infections in immunocompetent hosts. However, they are increasingly being recognized in immunocompromised patients. We present the case of a 40-year-old HIV-positive male presenting with lymphadenopathies and pancytopenia in whom disseminated infection, with bone marrow involvement by Mycobacterium genavense (M. genavense) was diagnosed.
June 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28587063/a-relative-deficiency-of-lysosomal-acid-lypase-activity-characterizes-non-alcoholic-fatty-liver-disease
#13
Francesco Tovoli, Lucia Napoli, Giulia Negrini, Sergio D'Addato, Giulia Tozzi, Jessica D'Amico, Fabio Piscaglia, Luigi Bolondi
Lysosomal acid lipase (LAL) is a key enzyme in lipid metabolism. Initial reports have suggested a role for a relative acquired LAL deficiency in non-alcoholic fatty liver disease (NAFLD)-however, it is still unclear whether this mechanism is specific for NAFLD. We aimed to determine LAL activity in a cohort of NAFLD subjects and in a control group of hepatitis C virus (HCV)-infected patients, investigating the role of liver cirrhosis. A total of 81 patients with a diagnosis of NAFLD, and 78 matched controls with HCV-related liver disease were enrolled...
May 25, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28584963/steroid-resistant-autoimmune-myelofibrosis-in-a-patient-with-autoimmune-hepatitis-and-evans-syndrome-complicated-with-increased-expression-of-tgf-%C3%AE-in-the-bone-marrow-a-case-report
#14
Hiroshi Ohkawara, Miki Furukawa, Kazuhiko Ikeda, Akiko Shichishima-Nakamura, Masahiko Fukatsu, Takahiro Sano, Koki Ueda, Satoshi Kimura, Risa Kanai, Yuka Oka, Fumi Murakami, Osamu Suzuki, Yuko Hashimoto, Kazuei Ogawa, Takayuki Ikezoe
We here report a 47-year-old female with autoimmune myelofibrosis (AIMF) associated with liver damage caused by autoimmune hepatitis and Evans syndrome. Bone marrow biopsy revealed hypocellular marrow with grade 2 reticulin fibrosis and increased levels of B lymphocytes (CD20(+)), T lymphocytes (CD3(+), CD8(+)), and plasma cells (CD138(+)). Immunohistochemical analysis revealed increased expression of transforming growth factor-β (TGF-β) in infiltrating lymphocytes and macrophages in the bone marrow. She was initially treated with oral prednisolone (PSL) for 2 months, which had a limited effect...
June 5, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28573901/a-case-report-of-parvovirus-b19-infection-in-a-renal-allograft
#15
Diana M Oramas, Suman Setty, Vijay Yeldandi, Julio Cabrera, Tushar Patel
Parvovirus B19 infection is undiagnosed in recipients undergoing solid organ transplantation. It is usually responsible for unexplained acute and chronic red blood cell aplasia that does not respond to erythropoietin therapy. Cases of parvovirus B19 infection associated with pancytopenia, solid organ dysfunction, and allograft rejection have been described in the literature. The deterioration of the immune system as a result of severe immunotherapy favors the reactivation of a previous infection or the acquisition of a new one...
June 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28562510/pancytopenia-and-hypothyroidism-in-a-patient-with-leukemic-infiltration-of-the-thyroid-as-the-first-presentation-of-acute-lymphoblastic-leukemia
#16
Christiaan F Mooij, Janiëlle A E M van Alfen-van der Velden, Romana T Netea-Maier, Roel Ten Broek, Martin Gotthardt, Melanie M Hagleitner
We report the case of a 16-year-old female patient with hypothyroidism, goiter, and pancytopenia. Biopsy of the thyroid showed leukemic infiltration. After confirmation of the diagnosis of B-lymphoblastic leukemia, treatment was started. Histologic follow-up at day 33 and 79 showed no residual signs of leukemic infiltration. Hypothyroidism persisted despite successful antileukemic treatment. Leukemic infiltration of the thyroid should be considered as a differential diagnosis in patients with hypothyroidism, goiter, and pancytopenia...
May 29, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28559663/consecutive-sessions-of-rescue-balloon-atrial-septostomy-for-an-idiopathic-pulmonary-arterial-hypertension-patient-with-refractory-right-heart-failure-usefulness-of-intracardiac-echocardiography-guidance
#17
Yu-Wei Chen, Hung-Chih Pan, Kuo-Yang Wang, Kae-Woei Liang
For idiopathic pulmonary artery hypertension (PAH) patients with end-stage right heart failure who received maximal medical therapy, balloon atrial septostomy (BAS) is recommended by most guidelines as a palliative therapy or a bridging treatment before lung transplantation. In this report, we described a 32-year-old woman with idiopathic PAH, who received maximal PAH-specific medical treatment, including intravenous prostacyclin, but still suffered from refractory right heart failure. The markedly enlarged right atrium (RA), high mean RA pressure of 23 mmHg, low systemic arterial oxygen saturation of 86% and concomitant pancytopenia all increased the patient's risk for BAS...
May 2017: Acta Cardiologica Sinica
https://www.readbyqxmd.com/read/28549617/hematopathological-alterations-of-major-tumor-suppressor-cascade-vital-cell-cycle-inhibitors-and-hematopoietic-niche-components-in-experimental-myelodysplasia
#18
Ritam Chatterjee, Shubhangi Gupta, Sujata Law
Myelodysplastic syndrome (MDS) is a poorly understood dreadful hematopoietic disorder that involves maturational defect and abnormalities in blood cell production leading to dysplastic changes and peripheral blood pancytopenia. The present work aims in establishing the mechanistic relationship of the expressional alterations of major tumor suppressor cascade, vital cell cycle inhibitors and hematopoietic microenvironmental components with the disease pathophysiologies. The study involves the development of N-N' Ethylnitrosourea (ENU) induced mouse model of MDS, characterization of the disease with blood film and bone marrow smear studies, scanning electron microscopic observation, mitochondrial membrane potential determination, flowcytometric analysis of osteoblastic and vascular niche components along with the expressional study of cleaved caspase-3, PCNA, Chk-2, p53, Ndn, Gfi-1, Tie-2, Sdf-1, Gsk-3β, p18 and Myt-1 in the bone marrow compartment...
May 23, 2017: Chemico-biological Interactions
https://www.readbyqxmd.com/read/28545978/benralizumab-for-patients-with-mild-to-moderate-persistent-asthma-bise-a-randomised-double-blind-placebo-controlled-phase-3-trial
#19
Gary T Ferguson, J Mark FitzGerald, Eugene R Bleecker, Michel Laviolette, David Bernstein, Craig LaForce, Lyndon Mansfield, Peter Barker, Yanping Wu, Maria Jison, Mitchell Goldman
BACKGROUND: Benralizumab is a humanised, anti-interleukin 5 receptor α monoclonal antibody that directly and rapidly depletes eosinophils, reduces asthma exacerbations, and improves lung function for patients with severe eosinophilic asthma. The objective of this trial was to assess the safety and efficacy of benralizumab for patients with mild to moderate, persistent asthma. METHODS: In this randomised, double-blind, placebo-controlled, phase 3 trial, we recruited patients aged 18-75 years, weighing at least 40 kg, and with a postbronchodilator reversibility in forced expiratory volume in 1 s (FEV1) of at least 12% at screening, from 52 clinical research centres in six countries...
May 22, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/28538514/long-term-outcome-of-4-patients-with-transcobalamin-deficiency-caused-by-2-novel-tcn2-mutations
#20
Marwan Nashabat, Gustavo Maegawa, Peter H Nissen, Ebba Nexo, Hussain Al-Shamrani, Mohammed Al-Owain, Majid Alfadhel
Cobalamin (vitamin B12 [Cbl]) is an essential cofactor for many biochemical pathways. Transcobalamin (TC) is required to internalize Cbl into the cells through membrane receptor-mediated endocytosis. Cbl is then processed in the cytoplasm and mitochondria by complementation factors leading to its active metabolites; methylcobalamin and 5-deoxyadenosyl-cobalamin. Deficiency of TC results in an elevation in methylmalonic acid and homocysteine. Patients usually present with macrocytic anemia, pancytopenia, failure to thrive, gastrointestinal symptoms, and neurological dysfunction...
May 22, 2017: Journal of Pediatric Hematology/oncology
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