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https://www.readbyqxmd.com/read/28331646/conversion-of-low-flow-priapism-to-high-flow-state-using-t-shunt-with-tunneling
#1
Neil A Mistry, Nicholas N Tadros, Jason C Hedges
Introduction. The three types of priapism are stuttering, arterial (high-flow, nonischemic), and venoocclusive (low-flow, ischemic). These are usually distinct entities and rarely occur in the same patient. T-shunts and other distal shunts are frequently combined with tunneling, but a seldom recognized potential complication is conversion to a high-flow state. Case Presentation. We describe 2 cases of men who presented with low-flow priapism episodes that were treated using T-shunts with tunneling that resulted with both men having recurrent erections shortly after surgery that were found to be consistent with high-flow states...
2017: Case Reports in Urology
https://www.readbyqxmd.com/read/28328837/atypical-presentation-of-paroxysmal-nocturnal-hemoglobinuria-treated-by-eculizumab-a-case-report
#2
Anne Quinquenel, Quentin Maestraggi, Carinne Lecoq-Lafon, Peffault de Latour Régis, Alain Delmer, Amélie Servettaz
RATIONALE: Paroxysmal nocturnal hemoglobinuria (PNH) is a nonmalignant acquired hematopoietic stem cell disease, which can be revealed by hemolytic anemia, thromboembolism, or bonemarrow failure. Thrombosis can occur at any site, but coronary thrombosis is extremely rare. Controlled trials have demonstrated that eculizimab, an inhibitor of the terminal complement cascade, was able to reduce both hemolysis and thrombosis, but its efficacy in cases of PNH with coronary thrombosis is unknown...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28323276/open-capsule-budesonide-for-refractory-celiac-disease
#3
Saurabh S Mukewar, Ayush Sharma, Alberto Rubio-Tapia, Tsung-Teh Wu, Bana Jabri, Joseph A Murray
OBJECTIVES: Refractory celiac disease (RCD) is a rare condition often associated with poor prognosis. Various immunosuppressive medications (IMs) have been used with modest success. We describe outcomes in patients treated with open-capsule budesonide (OB), including those for whom IM treatment failed. METHODS: We identified RCD patients treated with OB at Mayo Clinic, Rochester, Minnesota from 2003 to 2015. Demographic, serologic, and clinical variables were analyzed...
March 21, 2017: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/28321090/splenic-marginal-zone-lymphoma-complicated-by-cold-agglutinin-disease
#4
Kiyosumi Ochi, Kazuaki Yokoyama, Nobuhiro Ohno, Yasunori Ota, Arinobu Tojo
Splenic marginal zone lymphoma (SMZL) is a rare low-grade B-cell lymphoma accounting for less than 1% of lymphoid neoplasms and is often associated with autoimmune disorders. A 48-year-old woman presented with severe anemia due to steroid-refractory cold agglutinin disease (CAD), and was referred to our hospital for management of progressive systemic illness and high fever. On admission, she showed elevated serum soluble IL-2R and mild splenomegaly. PET/CT revealed FDG accumulation in the spleen and bone. She was pathologically diagnosed as having splenic marginal zone lymphoma by splenectomy and received 8 cycles of rituximab every 2 weeks, resulting in marked improvement of anemia...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28321074/epstein-barr-virus-associated-lymphoproliferative-disorder-with-encephalitis-following-anti-thymocyte-globulin-for-aplastic-anemia-resolved-with-rituximab-therapy-a-case-report-and-literature-review
#5
Kiyomi Mashima, Shingo Yano, Hiroki Yokoyama, Takeshi Saito, Tomohito Machishima, Takaki Shimada, Yuichi Yahagi, Shinobu Takahara, Katsuki Sugiyama, Yoji Ogasawara, Jiro Minami, Yutaro Kamiyama, Atsushi Katsube, Kazuhito Suzuki, Sayaka Ohshima, Hisashi Yamada, Noriko Usui, Keisuke Aiba
Epstein-Barr virus (EBV)-associated lymphoproliferative disorders (LPDs) sometimes occur following Anti-thymocyte globulin (ATG) administration for allogenic stem cell transplantation but are rare in aplastic anemia (AA) patients. A 55-year-old woman with AA following ATG developed refractory fever and was diagnosed with EBV-LPD. She was successfully treated with weekly rituximab monotherapy; however, she developed EBV encephalitis. She was admitted to the intensive care unit and finally recovered from unconsciousness...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28299229/a-giant-brunneroma-causing-gastrointestinal-bleeding-and-severe-anemia-requiring-transfusion-and-surgery
#6
Nicola C Frenkel, Miangela M Laclé, Inne H M Borel Rinkes, Izaak Q Molenaar, Jeroen Hagendoorn
Brunner's gland hamartoma, also called hyperplasia, adenoma, and Brunneroma, is an extremely rare benign proliferative lesion of Brunner's glands in the duodenum. While being mostly small and asymptomatic, they can result in gastrointestinal bleeding and obstruction. We report the case of a 54-year-old man presenting with melena and severe anemia requiring blood transfusion. CT scans showed a large mass of 8 cm in diameter, presumably arising in the duodenum. Endoscopic biopsies were not conclusive. As we were unable to determine the nature of the mass preoperatively and due to the severe symptoms, its size, and the uncertain malignant potential, a classic Whipple procedure was performed...
2017: Case Reports in Surgery
https://www.readbyqxmd.com/read/28299211/epistaxis-as-a-common-presenting-symptom-of-glanzmann-s-thrombasthenia-a-rare-qualitative-platelet-disorder-illustrative-case-examples
#7
Michael Recht, Meera Chitlur, Derek Lam, Syana Sarnaik, Madhvi Rajpurkar, David L Cooper, Sriya Gunawardena
Children often present to emergency departments (EDs) with uncontrollable nose bleeding. Although usually due to benign etiologies, epistaxis may be the presenting symptom of an inherited bleeding disorder. Whereas most bleeding disorders are detected through standard hematologic assessments, diagnosing rare platelet function disorders may be challenging. Here we present two case reports and review diagnostic and management challenges of platelet function disorders with a focus on Glanzmann's thrombasthenia (GT)...
2017: Case Reports in Emergency Medicine
https://www.readbyqxmd.com/read/28291558/-primary-epithelioid-adrenal-angiosarcoma-mimicking-undifferentiated-carcinoma
#8
Samira Miladi, Yaëlle Harrar, Maxime Battistella, Philippe Bertheau, Véronique Meignin, Brigitte Roche, Fatiha Amira Bouhidel
The adrenal primary epithelioid angiosarcoma (ASE) is a rare malignant tumor which can be histologically confused with other neoplasms. We report one case in a 79-year-old man who underwent adrenal tumor surgery for a mass fortuitly discovered by imaging for examination of an inflammatory syndrome associated with anemia. The histological diagnosis was difficult because of the undifferentiated and epithelioid appearance of tumor cells, whose immunohistochemical epithelial markers positivity led to frequent confusion with a metastatic carcinoma...
March 11, 2017: Annales de Pathologie
https://www.readbyqxmd.com/read/28288851/clinically-distinct-presentations-of-copper-deficiency-myeloneuropathy-and-cytopenias-in-a-patient-using-excessive-zinc-containing-denture-adhesive
#9
Sahara J Cathcart, Alina G Sofronescu
OBJECTIVES: While copper deficiency has long been known to cause cytopenias, copper deficiency myeloneuropathy is a more recently described entity. Here, we present the case of two clinically distinct presentations of acquired copper deficiency syndromes secondary to excessive use of zinc-containing denture adhesive over five years: myeloneuropathy and severe macrocytic anemia and neutropenia. METHODS: Extensive laboratory testing and histologic evaluation of the liver and bone marrow, were necessary to rule out other disease processes and establish the diagnosis of copper deficiency...
March 10, 2017: Clinical Biochemistry
https://www.readbyqxmd.com/read/28286631/hemoglobin-willamette-%C3%AE-51pro-%C3%A2-arg-case-report-and-literature-review
#10
Orivaldo Alves Barbosa, Matheus Martins de Sousa Dias, Saymon Medeiros Távora, Gentil Claudino de Galiza Neto, Jacqueline Holanda de Souza, Herivaldo Ferreira da Silva
We report a case of hemoglobin (Hb) Willamette (β51 Pro → Arg) in the Hematology Department of a tertiary hospital in Fortaleza, Northeast of Brazil. A literature review of the cases described in health sciences databases using as a descriptor Hb Willamette was performed, revealing 12 reported cases, of which only one presented with anemia. Herein, we describe a case of a female 29 years old, with hemoglobinopathy Willamette presenting clinically with anemia, having the lowest hemoglobin rate of the published cases...
February 23, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28286630/a-fatal-case-of-immune-hyperhemolysis-with-bone-marrow-necrosis-in-a-patient-with-sickle-cell-disease
#11
Matthew S Karafin, Arun Singavi, Susan T Johnson, Joshua J Field
In patients with sickle cell disease, hyperhemolysis is a rare but life-threatening complication of transfusion. In this case report, we describe a 61 year-old woman with hemoglobin sickle cell (SC) disease and history of alloimmunization who developed hyperhemolysis associated with a transfusion. She was found to have a warm and a clinically-significant cold autoantibody. Severe anemia (Hb 2.7 g/dL) with reticulocytopenia and thrombocytopenia prompted a bone marrow biopsy, which demonstrated extensive bone marrow necrosis...
February 23, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28283014/-fungemia-and-septic-arthritis-caused-by-saprochaete-capitata-in-a-patient-with-fanconi-aplastic-anemia-a-case-report
#12
Ömür Mustafa Parkan, Mustafa Altay Atalay, Ayşe Nedret Koç, Çiğdem Pala, Gonca Aydemir, Leylagül Kaynar
Saprochaete capitata (formerly known as Blastoschizomyces capitatus, Trichosporon capitatum, Geotrichum capitatum) is a rare but emerging yeast-like fungus. It is commonly found in environmental sources and can be isolated from skin, gastrointestinal system and respiratory tract of healthy individuals as well. It mainly infects patients with hematological malignancies such as acute myeloid leukemia (AML), especially in the presence of neutropenia; and mortality rates are high in those patients. Although the data about the in vitro antifungal susceptibility are limited, it is being reported that amphotericin B and voriconazole are more effective on S...
January 2017: Mikrobiyoloji Bülteni
https://www.readbyqxmd.com/read/28277850/diagnosis-and-management-of-catastrophic-antiphospholipid-syndrome
#13
Or Carmi, Maya Berla, Yehuda Shoenfeld, Yair Levy
Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening disease. In 1992, Asherson defined it as a widespread coagulopathy related to the antiphospholipid antibodies (aPL). CAPS requires rapid diagnosis and prompt initiation of treatment. Areas covered: This paper discusses all aspects of CAPS, including its pathophysiology, clinical manifestations, diagnostic approaches, differential diagnoses, management and treatment of relapsing CAPS, and its prognosis. To obtain the information used in this review, scientific databases were searched using the key words antiphospholipid antibodies, catastrophic antiphospholipid syndrome, hemolytic anemia, lupus anticoagulant, and thrombotic microangiopathic hemolytic anemia...
March 1, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28277473/clinical-utility-of-hemoglobin-testing-after-minimally-invasive-sacrocolpopexy
#14
Erin Seifert Lavelle, Lauren Giugale, Sears Sarah, Charelle M Carter-Brooks, Jonathan P Shepherd
OBJECTIVE: To determine the clinical utility of routine postoperative hemoglobin screening after minimally invasive sacrocolpopexy. METHODS: This is a retrospective chart review of women undergoing minimally invasive sacrocolpopexy between 2009 and 2015 at a large academic center where postoperative hemoglobin assessment is performed as routine practice. Demographic and perioperative data, pre- and postoperative hemoglobin values, and clinical signs and symptoms of potential postoperative anemia were extracted...
March 9, 2017: Female Pelvic Medicine & Reconstructive Surgery
https://www.readbyqxmd.com/read/28275334/clinical-significance-of-an-alloantibody-against-the-kell-blood-group-glycoprotein
#15
Stella Maris Mattaloni, Carine Arnoni, Rosario Céspedes, Claudia Nonaka, Carolina Trucco Boggione, Melina Eliana Luján Brajovich, Andrea Trejo, Néstor Zani, Claudia Silvia Biondi, Lilian Castilho, Carlos Miquel Cotorruelo
BACKGROUND: Kell null (K0) individuals can produce anti-Ku, an antibody against many epitopes in the Kell glycoprotein, after transfusion and/or pregnancy. Since sensitized K0 patients are rare, little is known about anti-Ku clinical relevance and in particular about its association to hemolytic disease of the fetus and newborn. CASE REPORT: This work describes a case of neonatal hyperbilirubinemia due to immune-mediated erythrocyte destruction by an alloantibody directed against the Kell glycoprotein...
January 2017: Transfusion Medicine and Hemotherapy
https://www.readbyqxmd.com/read/28273032/the-efficacy-of-modified-docetaxel-cisplatin-5-fluorouracil-regimen-as-first-line-treatment-in-patients-with-alpha-fetoprotein-producing-gastric-carcinoma
#16
Yakup Bozkaya, Mutlu Doğan, Ozan Yazıcı, Gökmen Umut Erdem, Nebi Serkan Demirci, Nurullah Zengin
Alpha-fetoprotein producing gastric carcinoma (AFP-PGC) is a rare cancer for which limited data on the clinicopathological features and treatment modalities exist. The aim of this study was to compare the efficacy of modified docetaxel-cisplatin-5-fluorouracil (mDCF) as the first-line chemotherapy regimen in metastatic AFP-PGC and non-AFP-PGC. The patients diagnosed with metastatic gastric cancer who were given mDCF as first-line therapy were retrospectively reviewed. The patients with a basal serum AFP level over 9 ng/ml were defined as AFP-PGC patients...
February 25, 2017: Bosnian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/28267090/a-rare-cause-of-neonatal-hemolytic-anemia-glutathione-synthetase-deficiency
#17
Pembe Soylu Ustkoyuncu, Fatma Türkan Mutlu, Aslihan Kiraz, Zuhal Tag Balkis, Sibel Yel
BACKGROUND: Isolated hemolysis or hemolytic anemia and 5-oxoprolinuria are 2 distinct medical conditions in the clinical spectrum associated with glutathione synthetase deficiency. CLINICAL OBSERVATION: A 1-day-old female baby presented with anemia and respiratory distress. Her hemoglobin level was 9.5 g/dL and the total serum bilirubin level was 5.6 mg/dL. Metabolic acidosis was detected in her blood gas analysis. Metabolic acidosis recurred despite treatment and further investigation was required...
March 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28267085/hemolytic-anemia-due-to-right-ventricular-to-pulmonary-artery-conduit-stenosis
#18
Sudha Rao, Julie A Creaden, Shunyou Gong, Cynthia Rigsby, John M Costello
Hemolytic anemia is a well-recognized complication in patients with left-sided mechanical heart valves. It is rare to see hemolysis with a bioprosthetic valve in the right ventricular outflow tract. We report a 4-year-old-girl with history of truncus arteriosus status-post repair who developed hemolytic anemia as a result of a calcified and obstructed bioprosthetic right ventricular to pulmonary artery-valved conduit. The hemolysis was alleviated by replacing the obstructed conduit with a larger valved conduit...
March 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28266038/using-direct-antiglobulin-test-results-to-reduce-unnecessary-cold-agglutinin-testing
#19
Craig B Wilen, Garrett S Booth, Brenda J Grossman, William J Lane, Penny C Szklarski, Ronald Jackups
BACKGROUND: Cold agglutinin disease (CAD) is a rare autoimmune hemolytic anemia mediated by autoantibodies that preferentially react at 4°C. Laboratory testing for cold-reactive autoantibodies is laborious and may not be ordered judiciously, particularly in patients with a negative direct antiglobulin test (DAT). We sought to determine whether a negative DAT using anti-human complement (anti-C3) rules out elevated cold agglutinin (CA) titers and the diagnosis of CAD. STUDY DESIGN AND METHODS: We performed a retrospective study of patients with a CA test performed at three major academic medical centers: Barnes-Jewish Hospital (2003-2014), Vanderbilt University Medical Center (2007-2009), and Massachusetts General Hospital (2009-2014)...
March 7, 2017: Transfusion
https://www.readbyqxmd.com/read/28261784/fatal-folic-acid-toxicity-in-humans
#20
Gerard Pradeep Devnath, Senthil Kumaran, R Rajiv, Kusa Kumar Shaha, Ashok Nagaraj
Folic acid is B-9 vitamin. Folic acid is prescribed commonly for pregnant women to prevent neural tube defects in the fetus, patients under chemotherapy, pernicious anemia and to reduce the risk of stroke and cardiovascular disease. Acute or chronic ingestion of a large dose of folic acid generally manifests as neurological complications, which are reversible. In this present case, a 23-year-old pregnant woman committed suicide by consuming folic acid tablets and succumbed to death within 36 h. Postmortem toxicological analysis detected folic acid in viscera...
March 6, 2017: Journal of Forensic Sciences
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