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https://www.readbyqxmd.com/read/28936212/how-viruses-contribute-to-the-pathogenesis-of-hemophagocytic-lymphohistiocytosis
#1
Ellen Brisse, Carine H Wouters, Graciela Andrei, Patrick Matthys
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening, hyperinflammatory syndrome, characterized by the uncontrolled activation of macrophages and T cells, eliciting key symptoms such as persistent fever, hepatosplenomegaly, pancytopenia, hemophagocytosis, hyperferritinemia, and coagulopathy. Viral infections are frequently implicated in the onset of active HLH episodes, both in primary, genetic HLH as in the secondary, acquired form. Infections with herpesviruses such as Epstein-Barr virus and cytomegalovirus are the most common...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28935394/case-report-an-hiv-patient-presenting-with-pancytopenia-hepatic-failure-and-coagulopathy-a-rare-small-cell-liver-carcinoma-with-diffuse-splenic-and-bone-marrow-metastasis-diagnosed-at-autopsy
#2
Joshua T Byers, Alejandro Mendoza, Daniel Wu, Jason S Kahlon, Xin Qing, Samuel W French
A 34-year old male with a giant condyloma acuminatum of the anus secondary to HIV infection presented to the emergency department with a persistent nose bleed lasting 2-3days, acute anemia, thrombocytopenia, and coagulopathy. The patient also had significant hepatosplenomegaly and elevated liver enzymes which were a new finding since the patient's last hospitalization 1-2month prior to the current admission. A bone marrow biopsy showed diffuse infiltration by carcinoma with neuroendocrine features. The patient quickly developed multi-organ injury, decompensated, and died...
September 18, 2017: Experimental and Molecular Pathology
https://www.readbyqxmd.com/read/28932375/fever-of-unknown-origin-in-a-male-patient-with-systemic-lupus-erythematosus
#3
Duminda Bandara Basnayake, Thamara Kannangara, Laknath Welagedara, Vindhya Bandara, Janitha Herath
BACKGROUND: Systemic lupus erythematosus (SLE) is an inflammatory autoimmune disorder which is uncommon in men. It has a wide variety of clinical presentations. CASE REPORT: We report a 21-year-old male presented with one month history of fever, loss of appetite, weight loss and reduced hair growth with an examination revealing an oral ulcer, cervical and axillary lymphadenopathy simulating hematological malignancy. Investigations showed pancytopenia, positive anti-nuclear factor and double-stranded DNA, high erythrocyte sedimentation rate with normal C-reactive protein levels and hypocomplementemia...
2017: Caspian Journal of Internal Medicine
https://www.readbyqxmd.com/read/28927162/myelodysplastic-syndrome-unclassifiable-mds-u-with-1-blasts-is-a-distinct-subgroup-of-mds-u-with-a-poor-prognosis
#4
Elizabeth Margolskee, Robert P Hasserjian, Duane Hassane, Wayne Tam, Susan Mathew, Chi Young Ok, Sa A Wang, Jean Oak, Daniel A Arber, Attilio Orazi
Objectives: Three situations qualify as myelodysplastic syndrome, unclassifiable (MDS-U): (1) refractory cytopenia with dysplasia and 1% blasts in peripheral blood (BL), (2) pancytopenia with unilineage dysplasia (Pan), and (3) persistent cytopenia, less than 5% bone marrow blasts, and less than 10% dysplastic cells and presence of MDS-defining cytogenetic abnormalities (CG). We compared the clinicopathologic features and mutational profiles for these three groups. Methods: MDS-U cases were reviewed at four major academic institutions...
July 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28924117/successful-treatment-of-herpes-simplex-virus-hsv-1-associated-hemophagocytic-lymphohistiocytosis-hlh-with-acyclovir-a-case-report-and-literature-review
#5
Tomohiro Yabushita, Satoshi Yoshioka, Yusuke Koba, Yuichirou Ono, Nobuhiro Hiramoto, Sumie Tabata, Munehiro Itou, Norio Shimizu, Keisuke Tomii, Takayuki Ishikawa
Hemophagocytic lymphohistiocytosis (HLH) associated with herpes simplex virus (HSV)-1 infection (HSV-1-HLH) is uncommon and is potentially fatal without appropriate treatment. We herein report the case of an adult patient with HSV-1-HLH who was successfully treated with acyclovir. A 69-year-old man developed fever, pancytopenia and liver enzyme elevation after the resolution of pneumonia. These findings and the presence of hemophagocytosis in the patient's bone marrow were consistent with a diagnosis of HLH...
September 15, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28917574/-apparent-worsening-of-psoriasis-lesions-revealing-methotrexate-overdosage
#6
R Haber, B Baroudjian, M Battistella, M Bagot, A Petit
BACKGROUND: Methotrexate (MTX) is an antimetabolite drug used in the treatment of cancers and autoimmune diseases and frequently in dermatology for cutaneous and/or arthritic psoriasis. Toxicities due to MTX overdosage are mainly cutaneous, hepatic and hematologic. Herein, we report a case of MTX overdosage presenting as an erosive and an inflammatory flare of preexisting psoriatic plaques and with new palmar lesions. PATIENTS AND METHODS: A 51-year-old male with a 6-year history of plaque psoriasis resistant to topical corticosteroids was started for the first time on MTX 20mg weekly...
September 13, 2017: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/28912991/t-cell-histiocyte-rich-large-b-cell-lymphoma-presenting-as-hemophagocytic-lymphohistiocytosis-an-uncommon-presentation-of-a-rare-disease
#7
Uroosa Ibrahim, Gwenalyn Garcia, Amina Saqib, Shafinaz Hussein, Qun Dai
T cell histiocyte rich large B cell lymphoma (THRLBCL) is a rare subtype of non-Hodgkin's lymphoma characterized by malignant B cells with reactive T lymphocytes. The pathophysiology is thought to involve cytokine-mediated evasion of T cell immune response by malignant B cells. It usually presents at an advanced stage with extranodal involvement. An extremely unusual manifestation of the disease is hemophagocytic lymphohistiocytosis (HLH) which is a hyperinflammatory disorder. We present a case of a 43-year-old male who presented with recurrent fever and recent radiologic imaging showing splenomegaly and right inguinal lymphadenopathy...
2017: Case Reports in Oncological Medicine
https://www.readbyqxmd.com/read/28906499/-incomplete-poems-syndrome-with-multicentric-castleman-s-disease
#8
P González de la Aleja, M García-Navarro, R Sánchez-Rodríguez, J M Ramos-Rincón
Castleman's disease (CD) is an atypical lymphoproliferative disorder of unknown cause, characterized by non-clonal nodal hyperplastic growth. Two forms of clinical presentation are currently recognized, one localized and the other multicentric, and four histopathologic variants. It is characterized by generalized lymphadenopathy, hepatosplenomegaly, fever and night sweats. CD may present severe pancytopenia, multi-organ failure, lymphoma evolution and it can sometimes be associated with paraneoplastic syndromes such as POEMS syndrome...
September 14, 2017: Anales del Sistema Sanitario de Navarra
https://www.readbyqxmd.com/read/28902383/-bcgitis-with-involvement-of-lung-liver-and-bone-marrow%C3%A2-after-immunotherapy-of-urothelial-cancer
#9
Hilte Friederike Geerdes-Fenge, Franziska Stubbe, Micha Löbermann, Philipp Warnke, Andreas Erbersdobler, Emil Christian Reisinger
Medical history A 77-year-old patient with transurethral resection of a bladder tumor was transferred due to persistent fever and progressive dyspnea despite antibiotic therapy for suspected urinary tract infection. Repeating the medical history revealed that a BCG immunotherapy of his non-muscle-invasive bladder carcinoma was performed the day before fever developed. Therefore, BCGitis was suspected. Examinations Laboratory parameters showed pancytopenia, elevated liver enzymes, eleveated C-reactive protein and hypoxemia...
September 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28894565/hematological-characteristics-of-yemeni-adults-and-children-with-visceral-leishmaniasis-could-eosinopenia-be-a-suspicion-index
#10
Jameel Al-Ghazaly, Waled Al-Dubai, Munasser Abdullah, Leila Al-Gharasi
BACKGROUND AND OBJECTIVES: Delay in the diagnosis of visceral leishmaniasis (VL) particularly in non-endemic areas is associated with higher mortality. In our experience, we found that marked bone marrow eosinopenia was a very frequent accompaniment of VL and might be a useful clue for the diagnosis, which indicates the opportunity for further morphological assessment. The aim of this study was to describe the hematological characteristics including peripheral blood and bone marrow findings of Yemeni adults and children with VL...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28894557/clinico-pathological-spectrum-and-novel-karyotypic-findings-in-myelodysplastic-syndrome-experience-of-tertiary-care-center-in-india
#11
Ruchi Gupta, Khaliqur Rahman, Manish Kumar Singh, Surabhi Kumari, Geeta Yadav, Soniya Nityanand
BACKGROUND: Myelodysplastic syndrome (MDS) is a heterogeneous disorder characterized clinically by the presence of cytopenia/s. Limited data are available about the morphological spectrum and cytogenetic profile of Indian MDS patients. The aim of the study was to ascertain the clinico-pathological, morphological and cytogenetic spectrum of Indian MDS patients. MATERIAL AND METHODS: A retrospective analysis of all patients diagnosed with MDS from June 2012 to December 2016 was performed...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28883247/hepatic-and-gastric-involvement-in-a-case-of-systemic-sarcoidosis-presenting-with-rupture-of-esophageal-varices
#12
Hiroaki Saito, Masayasu Ohmori, Masaya Iwamuro, Takehiro Tanaka, Nozomu Wada, Tetsuya Yasunaka, Akinobu Takaki, Hiroyuki Okada
A 46-year-old woman presented with massive hematemesis, caused by the rupture of esophageal varices. The laboratory investigations showed pancytopenia, and imaging tests revealed hepatosplenomegaly and ascites. A diagnosis of systemic sarcoidosis was made based on biopsies of the liver, stomach, lungs, heart, and skin. Although fat deposition was predominant, non-caseating granuloma and cirrhotic changes were found in the liver. Non-caseating granuloma was also identified in a biopsy specimen from minute depressions of the gastric folds...
September 6, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28875006/aberrant-methylation-mediated-suppression-of-apaf1-in-myelodysplastic-syndrome
#13
Farhad Zaker, Nahid Nasiri, Naser Amirizadeh, Seyed Mohsen Razavi, Marjan Yaghmaie, Ladan Teimoori-Toolabi, Ali Maleki, Masoumeh Bakhshayesh
Background: Myelodysplastic syndromes (MDSs) include a diverse group of clonal bone marrow disorders characterized by ineffective hematopoiesis and pancytopenia. It was found that down regulation of APAF1, a putative tumor suppressor gene (TSG), leads to resistance to chemotherapy and disease development in some cancers. In this study, we investigated the relation of APAF1 methylation status with its expression and clinicopathological factors in myelodysplastic syndrome (MDS) patients. Materials andMethods: Methylation Sensitive-High Resolution Melting Curve Analysis (MS-HRM) was employed in studying the methylation of CpG islands in the APAF1promoter region in MDS...
April 1, 2017: International Journal of Hematology-oncology and Stem Cell Research
https://www.readbyqxmd.com/read/28874083/azathioprine-induced-pancytopenia-and-septic-complications-a-probable-cause-of-death
#14
Bijoy K Panda, Siddhi Umarje, Arundhati Diwan
Azathioprine, an immunosuppressant which is widely used in the management of the autoimmune neuromuscular disorder. Myasthenia gravis is known to cause myelotoxicity. A 55-year-old male recently diagnosed with myasthenia gravis and chronic kidney disease was put on azathioprine (100 mg/d) along with pyridostigmine and prednisolone. When the treatment was initiated, the hematological reports revealed normal levels of blood count. However, approximately within 3 weeks of continuing the prescribed drugs, the patient was readmitted for complaints of loose watery stools, weakness, and giddiness...
January 1, 2017: Journal of Pharmacy Practice
https://www.readbyqxmd.com/read/28868793/chronic-steroid-response-pancytopenia-and-increased-bone-density-due-to-thromboxane-synthase-deficiency
#15
Richa Sharma, Elizabeth Sierra Potchanant, Jennifer E Schwartz, Grzegorz Nalepa
Diagnosis of bone marrow failure (BMF) disorders is challenging but essential for optimal patient management. Here, we report a young adult from nonconsanguineous parents with progressive pancytopenia since childhood, bone pain, increased bone density, and haphazard ossification replacing hematopoiesis within the bone marrow. Sequencing revealed two novel biallelic variants of unknown significance within the thromboxane A synthase gene, TBXAS1 (c.266T > C; c.989T > C), bioinformatically predicted to disrupt the protein...
September 4, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28859059/phase-1-safety-pharmacokinetic-and-pharmacodynamic-study-of-the-cyclin-dependent-kinase-inhibitor-dinaciclib-administered-every-three-weeks-in-patients-with-advanced-malignancies
#16
Monica M Mita, Alain C Mita, Jennifer L Moseley, Jennifer Poon, Karen A Small, Ying-Ming Jou, Paul Kirschmeier, Da Zhang, Yali Zhu, Paul Statkevich, Kamelesh K Sankhala, John Sarantopoulos, James M Cleary, Lucian R Chirieac, Scott J Rodig, Rajat Bannerji, Geoffrey I Shapiro
BACKGROUND: Dinaciclib is a potent inhibitor of cell cycle and transcriptional cyclin-dependent kinases. This Phase 1 study evaluated the safety, tolerability and pharmacokinetics of various dosing schedules of dinaciclib in advanced solid tumour patients and assessed Pharmacodynamic and preliminary anti-tumour activity. METHODS: In part 1, patients were enrolled in escalating cohorts of 2-h infusions administered once every 3 weeks, utilising an accelerated titration design until a recommended phase 2 dose (RP2D) was defined...
August 31, 2017: British Journal of Cancer
https://www.readbyqxmd.com/read/28852709/ataxia-pancytopenia-syndrome-with-samd9l-mutations
#17
Sorina Gorcenco, Jonna Komulainen-Ebrahim, Karin Nordborg, Maria Suo-Palosaari, Sten Andréasson, Johanna Krüger, Christer Nilsson, Ulrika Kjellström, Elisa Rahikkala, Dominik Turkiewicz, Mikael Karlberg, Lars Nilsson, Jörg Cammenga, Ulf Tedgård, Josef Davidsson, Johanna Uusimaa, Andreas Puschmann
OBJECTIVE: We describe the neurologic, neuroradiologic, and ophthalmologic phenotype of 1 Swedish and 1 Finnish family with autosomal dominant ataxia-pancytopenia (ATXPC) syndrome and SAMD9L mutations. METHODS: Members of these families with germline SAMD9L c.2956C>T, p.Arg986Cys, or c.2672T>C, p.Ile891Thr mutations underwent structured interviews and neurologic and ophthalmologic examinations. Neuroimaging was performed, and medical records were reviewed...
October 2017: Neurology. Genetics
https://www.readbyqxmd.com/read/28840066/pituitary-carcinoma-a-case-report-and-discussion-of-potential-value-of-combined-use-of-ga-68-dotatate-and-f-18-fdg-pet-ct-scan-to-better-choose-therapy
#18
Heraldo Mendes Garmes, José Barreto Campello Carvalheira, Fabiano Reis, Luciano de Souza Queiroz, Mateus Dal Fabbro, Vanessa de Fatima Porto Souza, Allan de Oliviera Santos
BACKGROUND: Pituitary carcinoma is extremely rare and carries a very poor prognosis. In most cases, apparently indolent tumors become malignant; however, there are no satisfactory biomarkers for predicting tumor behavior. Thus, scientific advances in the search for new biological markers, diagnostic methods, and therapies are needed to improve the prognosis of these patients. CASE DESCRIPTION: We report the case of a woman with initial diagnosis of nonfunctioning pituitary adenoma which evolved to carcinoma after 4 years...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28834694/ruxolitinib-for-secondary-hemophagocytic-lymphohistiocytosis-first-case-report
#19
Jonathan H Sin, Mark L Zangardi
Hemophagocytic lymphohistiocytosis (HLH) is an immune-mediated disorder resulting in hyper-activation of inflammatory cytokines. If left untreated, the uncontrolled inflammatory response can lead to significant tissue injury and potentially life-threatening multi-organ dysfunction. Conventional immunosuppressive agents are available for the management of HLH, including dexamethasone, cyclosporine, and etoposide; however, patients may not respond to these therapies. Clinicians may turn toward alternative pharmacologic agents that likely have less clinical evidence...
August 16, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28831382/capnocytophaga-canimorsus-sepsis-in-a-methotrexate-treated-patient-with-rheumatoid-arthritis
#20
Shinobu Tamura, Asumi Koyama, Yusuke Yamashita, Chieko Shiotani, Hiromichi Nakamoto, Chiaki Nakamoto, Michio Suzuki, Yoshio Nakano, Koichi Imaoka, Takashi Sonoki, Tokuzo Fujimoto
Capnocytophaga canimorsus is a gram-negative rod that can be transmitted primarily by dog bites. This life-threatening organism commonly causes sepsis in patients with splenectomy or alcoholism. A 53-year-old rheumatoid arthritis male treated with methotrexate (MTX) for 5 years was admitted for a 4-day history of fever and dyspnea. He had been bitten on a finger by the family dog 4 days before onset. Laboratory tests revealed pancytopenia, acute renal failure, and evidence of disseminated intravascular coagulation, and he subsequently developed acute respiratory distress syndrome...
2017: IDCases
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