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https://www.readbyqxmd.com/read/28648734/drug-induced-hemolytic-anemia-pharmacological-aspects
#1
D Renard, A Rosselet
Drug-induced hemolytic anemia is a very rare but potentially lethal adverse drug reaction, which can take the form of oxidative damage to vulnerable erythrocytes (as in glucose-6-phosphate dehydrogenase deficiency), drug-induced thrombotic microangiopathy, or immune-mediated hemolytic anemia. For each form, distinctive drugs are documented as potential triggers. When a formal diagnosis of hemolytic anemia is made following drug administration, a structured approach is recommended to assess the plausibility of an adverse drug reaction based on chronological sequence, epidemiological data, objective evidence (when available), and ruling out of non-drug causes...
June 22, 2017: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/28646944/oncocytic-adrenocortical-carcinoma-a-rare-adrenal-tumor-subtype
#2
Emma Sumner, Behram Cenk Acar, Matthew R Acker
Oncocytic tumors arising from the adrenal gland are rare. Oncocytic adrenal neoplasms (OAN) may mimic adrenocortical carcinoma (ACC) at presentation, and can only be definitively diagnosed histologically. Most OANs are benign, and carry a favorable prognosis. We report on an 83-year-old female who, while being investigated for anemia and weight loss, was found to have a 23 cm adrenal mass concerning for ACC. Adrenalectomy and histopathology confirmed a malignant OAN, based on the Lin-Weiss-Bisceglia criteria...
June 2017: Canadian Journal of Urology
https://www.readbyqxmd.com/read/28642830/a-rare-case-of-esophageal-adenocarcinoma-with-urinary-bladder-metastasis
#3
Heather Katz, Rahoma E Saad, Krista Denning, Toni O Pacioles
Metastatic esophageal adenocarcinoma to the urinary bladder is extremely rare. We describe a previously healthy 49-year-old female with recent diagnosis of adenocarcinoma of the gastroesophageal junction with metastatic disease to the liver. Biopsy was positive for human epidermal growth factor receptor 2 (HER2) by Fluorescence In Situ Hybridization (FISH). She received six cycles of Cisplatin, 5-Fluorouracil, and Herceptin and subsequently developed symptomatic anemia and hematuria. Cystoscopy with retroflexion was performed and she received a transurethral resection of bladder tumor with fulguration...
2017: Case Reports in Gastrointestinal Medicine
https://www.readbyqxmd.com/read/28636757/babesiosis-an-unusual-cause-of-sepsis-after-kidney-transplantation-and-review-of-the-literature
#4
Imtiaz Ather, Negiin Pourafshar, Denise Schain, Asmita Gupte, Michael J Casey
We report a unique case of babesiosis presenting as sepsis after kidney transplantation. A 70-year-old female kidney transplant recipient presented with fever, hemolytic anemia, and acute kidney injury, and met three of four systemic inflammatory response syndrome (SIRS) criteria. Serology was positive for Babesia microti, confirmed by polymerase chain reaction. The patient was treated with atovaquone and azithromycin and made a full recovery. Reports of babesiosis after solid organ transplantation are rare, with only four prior cases reported in the literature...
June 21, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/28633156/generalized-indeterminate-cell-histiocytosis-presenting-as-eroded-papules-and-crusts
#5
Yaping Li, Harrison Xiao Bai, Chang Su, Guiying Zhang
Indeterminate cell histiocytosis (ICH) is a very rare histiocytic disease. We reported a case of generalized ICH presenting as eroded papules and crusts, accompanied with intermittent fever, pulmonary disorder, lymphadenectasis in the mediastinum, and the retroperitoneum. The histopathology indicated a neoplasm that was composed of small nests of large cells with ample pale cytoplasm. Immunohistochemistry results and the absence of Birbeck granules led us to the diagnosis of ICH. Four months later, the patient died of anemia, ascites, and swelling of the limbs...
July 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28630090/t-cell-transcriptomes-from-paroxysmal-nocturnal-hemoglobinuria-patients-reveal-novel-signaling-pathways
#6
Kohei Hosokawa, Sachiko Kajigaya, Keyvan Keyvanfar, Wangmin Qiao, Yanling Xie, Danielle M Townsley, Xingmin Feng, Neal S Young
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder originating from hematopoietic stem cells and is a life-threating disease characterized by intravascular hemolysis, bone marrow (BM) failure, and venous thrombosis. The etiology of PNH is a somatic mutation in the phosphatidylinositol glycan class A gene (PIG-A) on the X chromosome, which blocks synthesis of the glycolipid moiety and causes deficiency in GPI-anchored proteins. PNH is closely related to aplastic anemia, in which T cells mediate destruction of BM...
June 19, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28627616/distinct-cellular-phenotype-linked-to-defective-dna-interstrand-crosslink-repair-and-homologous-recombination
#7
Aleksandra M Gorniewska, Katarzyna Kluzek, Lidia Gackowska, Izabela Kubiszewska, Malgorzata Z Zdzienicka, Aneta Bialkowska
Repair of DNA interstrand crosslinks (ICLs) predominantly involves the Fanconi anemia (FA) pathway and homologous recombination (HR). The HR repair system eliminates DNA double strand breaks (DSBs) that emerge during ICLs removal. The current study presents a novel cell line, CL‑V8B, representing a new complementation group of Chinese hamster cell mutants hypersensitive to DNA crosslinking factors. CL‑V8B exhibits increased sensitivity to various DNA‑damaging agents, including compounds leading to DSBs formation (bleomycin and 6‑thioguanine), and is extremely sensitive to poly (ADP-ribose) polymerase inhibitor (>400‑fold), which is typical for HR‑defective cells...
June 15, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28627524/screening-for-mutations-in-two-exons-of-fancg-gene-in-pakistani-population
#8
Ujala Aymun, Saima Iram, Iram Aftab, Saba Khaliq, Ali Nadir, Ahmed Nisar, Shahida Mohsin
BACKGROUND: Fanconi anemia is a rare autosomal recessive disorder of genetic instability. It is both molecularly and clinically, a heterogeneous disorder. Its incidence is 1 in 129,000 births and relatively high in some ethnic groups. Sixteen genes have been identified among them mutations in FANCG gene are most common after FANCA and FANCC gene mutations. OBJECTIVE: To study mutations in exon 3 and 4 of FANCG gene in Pakistani population. METHODS: Thirty five patients with positive Diepoxybutane test were included in the study...
June 2017: Biomedical Papers of the Medical Faculty of the University Palacký, Olomouc, Czechoslovakia
https://www.readbyqxmd.com/read/28626540/acute-crises-and-complications-of-sickle-cell-anemia-among-patients-attending-a-pediatric-tertiary-unit-in-kinshasa-democratic-republic-of-congo
#9
Michel Ntetani Aloni, Bertin Tshimanga Kadima, Pépé Mfutu Ekulu, Aléine Nzazi Budiongo, René Makuala Ngiyulu, Jean Lambert Gini-Ehungu
In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated to affect 30,000 to 40,000 neonates per year. However, there is paucity of data on acute clinical manifestations in sickle cell children. In these circumstances, it is difficult to develop a health care policy for an adequate management of sickle cell patients. This was a seven years' retrospective study of children admitted with acute sickle cell crisis in the Department of Pediatrics in University Hospital of Kinshasa, Kinshasa, the Democratic Republic of Congo...
June 1, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28626212/a-huge-subcutaneous-hematoma-in-an-adult-with-kasabach-merritt-syndrome
#10
Kuan-Lin Wu, Chiung-Ying Liao, Chen-Kuang Chang, Shang-Yun Ho, Yeu-Sheng Tyan, Yuan-Chun Huang
BACKGROUND Kasabach-Merritt syndrome is a potentially fatal disease that consists of hemangioma(s) with thrombocytopenia, microangiopathic hemolytic anemia, and coagulopathy. Extensive hemangiomatosis is rare. We present the radiological features and treatment strategy of a young adult suffering from Kasabach-Merritt syndrome with widespread hemangiomas and an infected huge hematoma in the right thigh. CASE REPORT A 33-year-old Taiwanese male presented with a painful 20-cm mass over his right thigh and gross hematuria for 2 days...
June 19, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28623553/-kaposi-s-disease-in-an-hiv-positive-child-with-probable-contamination-from-his-grandmother
#11
A Dicko, Y Fofana, A Traoré, S Berthé, S Touré, C Lamissa, B Guindo, A Keita, O Faye
Kaposi's disease in children with HIV is rarely reported in everyday practice. This is a case study of cutaneous Kaposi's disease revealing HIV in a 5-year-old child with polymorphic eruption of papules and nodules on the face, trunk, back, and limbs. Histopathological examination confirmed the diagnosis of Kaposi's disease. The child's HIV serology was positive with a CD4 count of 240/mm3, normochromic and normocytic anemia, and a hemoglobin level at 8.5 g/dl. It was found that the child, after early weaning from his HIV-negative mother, had repeatedly suckled his healthy grandmother, who had no skin lesions but was HIV1 positive...
June 16, 2017: Bulletin de la Société de Pathologie Exotique
https://www.readbyqxmd.com/read/28620491/pseudo-thrombotic-thrombocytopenic-purpura-presenting-as-multi-organ-dysfunction-syndrome-a-rare-complication-of-pernicious-anemia
#12
Saroj Kandel, Nibash Budhathoki, Shanta Pandey, Bikash Bhattarai, Aam Baqui, Ramesh Pandey, Divya Salhan, Danilo Enriquez, Joseph Quist, Frances M Schmidt
OBJECTIVE: We present a rare case of pernicious anemia presented as multi-organ dysfunction syndrome, later found to have pseudo-thrombotic thrombocytopenic purpura. METHODS: An 86-year-old female presented with respiratory distress, altered mental status, acute renal failure and was intubated in emergency room. She was found to have severe anemia, thrombocytopenia, high lactate, high lactate dehydrogenase and low haptoglobin. Peripheral smear revealed multilobulated neutrophils with schistocytes, poikilocytes and anisocytes...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28620416/antiphospholipid-syndrome-multiple-manifestations-in-a-single-patient-a-high-suspicion-is-still-needed
#13
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/28619392/platelet-satellitism-in-autoimmune-hemolytic-anemia
#14
REVIEW
I Ghariani, N Braham, S Hamzaoui, L Bekir
Platelet satellitism is a rare phenomenon observed in blood smears obtained from blood anticoagulated with EDTA with an estimated frequency at 0.008%. It is characterized by platelet rosetting around neutrophils and in rare cases around other blood cells, which may causes pseudothrombocytopenia. Our case involves a 26-year-old female patient with autoimmune hemolytic anemia (AIHA). Blood smear with EDTA as an anticoagulant had platelet satellitism whereas the phenomenon was not observed in tubes with different anticoagulants (citrate, heparin) and capillary blood...
June 12, 2017: Current Research in Translational Medicine
https://www.readbyqxmd.com/read/28617164/liver-transplantation-using-graft-from-a-donor-with-aplastic-anemia
#15
Soheila Milani, Mohsen Aliakbarian, Ebrahim Khaleghi
Liver transplantation (LT) is a life-saving procedure in patients with end-stage liver disease. The number of patients in the waiting list for LT has steadily increased over time, so removing liver from deceased donors with unusual disorders could possibly be used for transplantation. We describe a case of LT from a donor with "aplastic anemia" to a patient with liver failure due to hepatitis C. Aplastic anemia is a syndrome of bone marrow failure. Aplastic anemia is one of the most common conditions linked to transfusion dependence, and long-term repeated transfusion inevitably results in iron overload...
June 2017: Progress in Transplantation
https://www.readbyqxmd.com/read/28616085/helicobacter-pylori-infection-old-and-new
#16
REVIEW
S Diaconu, A Predescu, A Moldoveanu, C S Pop, C Fierbințeanu-Braticevici
Helicobacter pylori is a spiral-shaped bacterium that grows in the digestive tract and may be present in more than half of the world's population. The clinical features of Helicobacter pylori range from asymptomatic gastritis to gastrointestinal malignancy. Mucosa-associated lymphoid tissue (MALT) lymphoma is a low-grade B-cell marginal zone lymphoma and Helicobacter pylori has been detected in more than 75% of the patients with MALT lymphoma. Many tests for the detection of Helicobacter pylori are available, including antibody tests, urea breath tests, stool antigen tests and endoscopic biopsies...
April 2017: Journal of Medicine and Life
https://www.readbyqxmd.com/read/28614581/clinical-biochemical-and-genetic-characterization-of-north-american-patients-with-erythropoietic-protoporphyria-and-x-linked-protoporphyria
#17
Manisha Balwani, Hetanshi Naik, Karl E Anderson, D Montgomery Bissell, Joseph Bloomer, Herbert L Bonkovsky, John D Phillips, Jessica R Overbey, Bruce Wang, Ashwani K Singal, Lawrence U Liu, Robert J Desnick
Importance: Autosomal recessive erythropoietic protoporphyria (EPP) and X-linked protoporphyria (XLP) are rare photodermatoses presenting with variable degrees of painful phototoxicity that markedly affects quality of life. The clinical variability, determinants of severity, and genotype/phenotype correlations of these diseases are not well characterized. Objective: To describe the baseline clinical characteristics, genotypes, and determinants of disease severity in a large patient cohort with EPP or XLP...
June 14, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28611626/angioimmunoblastic-t-cell-lymphoma-mimicking-drug-reaction-with-eosinophilia-and-systemic-symptoms-dress-syndrome
#18
Joanna Mangana, Emmanuella Guenova, Katrin Kerl, Mirjana Urosevic-Maiwald, Valerie C Amann, Cornelia Bayard, Reinhard Dummer, Lars E French
Angioimmunoblastic T-cell lymphoma (AITCL) is a rare, aggressive lymphoma which derives from follicular helper T cells, commonly affecting the elderly population. It accounts for 2% of all non-Hodgkin lymphomas, with a reported 5-year overall survival rate of less than 30%. Very often, the clinical picture of AITCL encompasses systemic symptoms such as generalized lymphadenopathy, hepatosplenomegaly, skin rash, anemia, and polyclonal hypergammaglobulinemia. Here we report on the case of a female patient who presented with clinical features resembling drug reaction with eosinophilia and systemic symptoms (DRESS syndrome) prior to the definitive diagnosis of AITCL...
January 2017: Case Reports in Dermatology
https://www.readbyqxmd.com/read/28610705/anastomotic-ulcers-in-short-bowel-syndrome-new-suggestions-from-a-multidisciplinary-approach
#19
Fabio Fusaro, Renato Tambucci, Erminia Romeo, Pietro Bagolan, Luigi Dall'Oglio, Stefano Ceccarelli, Paola Francalanci, Dominique Hermans, Andrea Pietrobattista, Antonella Diamanti, Filippo Torroni, Paola De Angelis
BACKGROUND AND AIMS: Anastomotic ulceration (AU) is a rare potential life-threatening complication that may occur after intestinal resection. The diagnosis is often delayed after a long-lasting history of refractory anemia. The pathogenesis remains unknown and there are no established therapies. The aim of the study was to analyze the medical history of children with short bowel syndrome (SBS) who were experiencing AU. METHODS: Records of SBS children were retrospectively reviewed...
June 3, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28605161/the-use-of-therapeutic-plasma-exchange-to-reduce-serum-bilirubin-in-a-dog-with-kernicterus
#20
Tricia Tovar, Sarah Deitschel, Christine Guenther
OBJECTIVE: To describe the use of a manual method of therapeutic plasma exchange to reduce total serum bilirubin, manage kernicterus, and halt progression of neurological dysfunction in a dog with immune-mediated hemolytic anemia (IMHA). CASE SUMMARY: A 5-year-old male neutered Lhasa Apso diagnosed with IMHA developed acute onset neurologic signs consistent with kernicterus. Manual therapeutic plasma exchange was performed in an attempt to reduce total serum bilirubin...
June 12, 2017: Journal of Veterinary Emergency and Critical Care
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