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hemolytic anemia treatment

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https://www.readbyqxmd.com/read/28649613/early-onset-parkinsonism-in-a-pedigree-with-phosphoglycerate-kinase-deficiency-and-a-heterozygous-carrier-do-pgk-1-mutations-contribute-to-vulnerability-to-parkinsonism
#1
Satoshi Sakaue, Takashi Kasai, Ikuko Mizuta, Masaya Suematsu, Shinya Osone, Yumiko Azuma, Toshihiko Imamura, Takahiko Tokuda, Hitoshi Kanno, Omar M A El-Agnaf, Masafumi Morimoto, Masanori Nakagawa, Hajime Hosoi, Toshiki Mizuno
Phosphoglycerate kinase 1 (PGK-1) is a glycolytic enzyme encoded by PGK-1, which maps to the X chromosome. PGK-1 deficiency causes X-linked recessive hereditary chronic hemolytic anemia, myopathy, and neurological disorders due to insufficient ATP regeneration. Early-onset parkinsonism has occasionally been reported as a neurological complication of this condition. However, heterozygous carriers of PGK-1 deficiency were thought to be neurologically asymptomatic. Here, we report a boy with PGK-1 deficiency and his mother, a carrier of a heterozygous mutation in PGK-1, both of whom presented with early-onset parkinsonism...
2017: NPJ Parkinson's Disease
https://www.readbyqxmd.com/read/28641660/-comparison-of-laboratory-indexes-and-therapeutic-efficacy-between-two-kinds-of-different-rbc-transfusion-in-aiha-patients
#2
Jing-Jing Gao, Xiong-Peng Zhu, Ming-Quan Wang
OBJECTIVE: To investigate the effect of 2 kinds of red blood cells (RBC) on the laboratorial indexes and therapeutic efficacy of patients with autoimmune hemolytic anemia (AIHA). METHODS: The clinical data of 120 patients with AIHA from June 2015 to June 2016 were analyzed retrospectively. These 120 patients were divided into A goup and B group. The patients in A group (60 cases) were infused with washed RBC, while the patients in B group (60 cases) were infused with WBC-deplated RBC...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28639307/anti-d-in-a-mother-hemizygous-for-the-variant-rhd-dnb-gene-associated-with-hemolytic-disease-of-the-fetus-and-newborn
#3
Kelli M Quantock, Genghis H Lopez, Catherine A Hyland, Yew-Wah Liew, Robert L Flower, Frans J Niemann, Arthur Joyce
BACKGROUND: Individuals with the partial D phenotype when exposed to D+ red blood cells (RBCs) carrying the epitopes they lack may develop anti-D specific for the missing epitopes. DNB is the most common partial D in Caucasians and the clinical significance for anti-D in these individuals is unknown. STUDY DESIGN AND METHODS: This article describes the serologic genotyping results and clinical manifestations in two group D+ babies of a mother presenting as group O, D+ with alloanti-D...
June 21, 2017: Transfusion
https://www.readbyqxmd.com/read/28626544/atypical-hemolytic-uremic-syndrome-a-brief-review
#4
REVIEW
Kuixing Zhang, Yuxin Lu, Kevin T Harley, Minh-Ha Tran
Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The histopathologic lesions of aHUS include thrombotic microangiopathy involving the glomerular capillaries and thrombosis involving arterioles or interlobar arteries. Extra-renal manifestations occur in up to 20% of patients. The majority of aHUS is caused by complement system defects impairing ordinary regulatory mechanisms. Activating events therefore lead to unbridled, ongoing complement activity producing widespread endothelial injury...
June 1, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28626212/a-huge-subcutaneous-hematoma-in-an-adult-with-kasabach-merritt-syndrome
#5
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/28621877/autoimmune-hemolytic-anemia-in-solid-organ-transplantation-the-role-of-immunosuppression
#6
REVIEW
Theoni Kanellopoulou
Hemolysis after solid organ transplantation can be caused by both immune and non-immune mediated mechanisms and the evaluation must take into account issues distinctive to the post-transplant period. Autoimmune hemolytic anemia usually occurs within the first year and has been attributed to immunosuppressive treatment, infections or underlying post-transplant lymphoproliferative disorder. Review of the literature revealed 59 cases with autoimmune hemolytic anemia mostly in children after liver transplantation...
June 16, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28612003/discontinuation-of-peritoneal-dialysis-after-late-initiation-of-eculizumab-in-a-case-of-familial-atypical-hemolytic-uremic-syndrome-a-case-report
#7
Rafael Alonso Valente, Giannina Elena García Rodríguez, Yanina García Marcote, Manuel Fidalgo Díaz, Vanesa Becerra Mosquera, Daniel Novoa García, Teresa Cordal Martínez, Cándido Díaz Rodríguez
BACKGROUND: Atypical hemolytic-uremic syndrome is caused by a thrombotic microangiopathy and manifests itself with hemolytic anemia, thrombocytopenia, and organ ischemia. Its etiology is a mutation affecting the genes encoding for proteins of the complement system. Early treatment with eculizumab (8.6 months from the moment of presentation), a humanized monoclonal antibody against complement, is shown to be effective in controlling symptoms and reversing organ damage. We present a patient with a mutation not previously described in the literature...
January 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28600054/prenatal-treatment-of-severe-fetal-hemolytic-disease-due-to-anti-m-alloimmunization-by-serial-intrauterine-transfusions
#8
Lin Li, Linhuan Huang, Guangping Luo, Yanmin Luo, Qun Fang
OBJECTIVE: Fetal hemolytic disease is a common cause of fetal hydrops and fetal morbidity and mortality. Despite its relatively low frequency, the anti-M IgG antibody is one of the causes of severe fetal anemia and intrauterine death; only a few cases have been reported. CASE REPORT: This is a case of a pregnant woman with a history of three intrauterine deaths. A diagnosis of severe fetal anemia attributed to anti-M alloimmunization was confirmed in her fifth pregnancy...
June 2017: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28598573/successful-treatment-of-tacrolimus-related-pure-red-cell-aplasia-and-autoimmune-hemolytic-anemia-with-rituximab-in-a-pediatric-cardiac-transplant-patient
#9
Chenue Abongwa, Ghada Abusin, Ayman El-Sheikh
Acquired pure red cell aplasia (PRCA) and autoimmune hemolytic anemia (AIHA) are rare complications of immunosuppression in pediatric solid organ transplant patients. We report a 14-month-old female child who developed Coombs positive hemolytic anemia and reticulocytopenia while on tacrolimus after cardiac transplantation. She was successfully treated with rituximab after failing treatment with corticosteroids and intravenous immunoglobulins. Clinicians should consider PRCA differential diagnosis in a patient presenting with reticulocytopenia and hemolysis...
June 9, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28596226/autoimmune-hemolytic-anemia-associated-with-trimethoprim-sulfamethoxazole-use
#10
Jillian Frieder, Jason Aboudi Mouabbi, Rami Zein, Tariq Hadid
PURPOSE: A case report of drug-induced immune hemolytic anemia (DIIHA) triggered by exposure to trimethoprim-sulfamethoxazole is presented along with a brief review of the pathophysiology of DIIHA and diagnostic considerations. SUMMARY: A 58-year-old woman recently initiated on trimethoprim-sulfamethoxazole for treatment of a urinary tract infection presented to the emergency department with generalized weakness and fatigue. Initial laboratory studies were significant for the following values: hemoglobin concentration, 5...
June 15, 2017: American Journal of Health-system Pharmacy: AJHP
https://www.readbyqxmd.com/read/28595228/-delayed-hemolytic-anemia-associated-with-anti-jk3-antibody-following-cardiovascular-surgery-report-of-a-case
#11
Takayuki Nishimoto, Yukihiro Bonkohara, Masaki Iijima
We report a 73-year-old man who underwent total aortic arch replacement with an open stent graft for aortic arch aneurysm. The patient received blood transfusion intraoperatively without any signs of hemolysis. However, on post-operative day 16, he developed hemoglobinuria, and was found to have anemia, elevated serum lactate dehydrogenase, elevated total bilirubin, and decreased serum haptoglobin. Initially, the cause for these findings was unknown. Upon further testing, however, antibodies against the high frequency antigen, anti-Jk3 was identified...
June 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28593773/-hellp-syndrome-requiring-therapeutic-plasma-exchange-due-to-progression-to-multiple-organ-dysfunction-syndrome-with-predominant-encephalopathy-respiratory-and-renal-insufficiency
#12
M Trávniková, J Gumulec, Z Kořístek, M Navrátil, M Janáč, J Pelková, P Šuráň, E Doležálková, O Šimetka
OBJECTIVE: Case report of woman with twin pregnancy complicated by HELLP syndrome which progressed to multiple organ dysfunction syndrome with predominant encephalopathy, renal and respiratory insufficiency with the need to perform repeated therapeutic plasma exchange. DESIGN: Case report. SETTING: Department of gynecology and obstetrics, University Hospital in Ostrava; Departmet of hematooncology, University Hospital in Ostrava; Department of gynecology and obstetrics, Vsetín hospital; Department of hematology and transfusion, Vsetín Hospital...
2017: Ceská Gynekologie
https://www.readbyqxmd.com/read/28578979/hiv-associated-kidney-diseases-clarifying-concordance-between-renal-failure-in-hiv-infection-and-histopathologic-manifestations-at-kidney-biopsy
#13
REVIEW
Carla L Ellis
Patients with HIV infection have a wide spectrum of renal diseases. Some are known to be the direct effect of the viral infection while others are renal diseases that also occur in uninfected populations. HIV associated nephropathy (HIVAN) is considered to be a subtype of primary focal and segmental glomerulosclerosis that is distinct in HIV infected patients. It is more frequent in the African-American population and associated with mutations of the apolipoprotein L1 (APOL1) gene. HIV associated immune complex kidney disease (HIVICD) encompasses a spectrum of HIV associated renal diseases characterized by the presence of immune complex deposition within glomeruli...
July 2017: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28568311/defining-the-clinical-relevance-of-red-blood-cell-autoantibodies-by-monocyte-monolayer-assay
#14
Marina C A V Conrado, Amanda N D'Avila, Juliana B Vieira, Silvia L Bonifacio, Francisco C A Gomes, Marcia R Dezan, Valeria B Oliveira, Ingrid H Ribeiro, Luciana T C M Tucunduva, Alfredo Mendrone-Júnior, Vanderson Rocha, Carla L Dinardo
BACKGROUND: The Monocyte Monolayer Assay (MMA) is an in vitro simulation of red blood cell (RBC) alloantibody behavior. It has been classically applied to predict the risks of post-transfusion hemolytic reactions when transfusing incompatible RBC units. Quantifying erythrophagocytosis by MMA may be an interesting option for situations where there is doubt whether a RBC autoantibody is mediating significant hemolysis. Here, we present three situations involving RBC autoantibodies in which the MMA was decisive for clarifying the diagnosis and choosing the best clinical treatment...
May 31, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28550351/diagnostic-and-treatment-guidelines-for-thrombotic-thrombocytopenic-purpura-ttp-2017-in-japan
#15
Masanori Matsumoto, Yoshihiro Fujimura, Hideo Wada, Koichi Kokame, Yoshitaka Miyakawa, Yasunori Ueda, Satoshi Higasa, Takanori Moriki, Hideo Yagi, Toshiyuki Miyata, Mitsuru Murata
Thrombotic thrombocytopenic purpura (TTP) can rapidly progress into a life-threatening condition, thus the importance of appropriate diagnosis and treatment cannot be overstated. Until recently, TTP has mainly been diagnosed by clinical findings such as thrombocytopenia and non-immune hemolytic anemia. In addition to these clinical findings, however, reduced activity of a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) below 10% has been accepted internationally as a diagnostic criterion for TTP...
July 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28542880/suicidal-death-of-erythrocytes-in-cancer-and-its-chemotherapy-a-potential-target-in-the-treatment-of-tumor-associated-anemia
#16
REVIEW
Elisabeth Lang, Rosi Bissinger, Syed M Qadri, Florian Lang
In analogy to apoptosis of nucleated cells, erythrocytes may enter eryptosis characterized by cell shrinkage and cell membrane scrambling. Eryptotic erythrocytes are rapidly cleared from circulating blood and may adhere to the vascular wall. Stimulation of eryptosis thus impairs microcirculation and leads to anemia as soon as the loss of erythrocytes cannot be fully compensated by enhanced erythropoiesis. Signaling stimulating eryptosis includes increase of cytosolic Ca(2+) -activity, ceramide, caspases, calpain, p38-kinase, protein-kinase C, Janus-activated kinase 3, casein-kinase 1α, and cyclin-dependent kinase 4...
May 23, 2017: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/28535517/coupled-plasma-filtration-adsorption-application-for-liver-and-thyroid-toxins
#17
Gabriele Donati, Irene Capelli, Anna Laura Croci Chiocchini, Nicolò Natali, Anna Scrivo, Gaetano La Manna
Coupled plasma filtration and adsorption (CPFA) is a detoxification system that combines a plasma adsorption circuit and a continuous renal replacement therapy circuit. Its main application is for sepsis and septic shock with or without acute renal failure. Several recent studies have suggested that CPFA can reduce the mortality when the volume of plasma absorbed on the styrenic resin is at least >0.18 L/kg/day. At present, new applications for CPFA are under investigation, also in patients without significant kidney failure...
2017: Contributions to Nephrology
https://www.readbyqxmd.com/read/28533306/bendamustine-plus-rituximab-for-chronic-cold-agglutinin-disease-results-of-a-nordic-prospective-multicenter-trial
#18
Sigbjørn Berentsen, Ulla Randen, Markku Oksman, Henrik Birgens, Tor Henrik Anderson Tvedt, Jakob Dalgaard, Eivind Galteland, Einar Haukås, Robert Brudevold, Jon Hjalmar Sørbø, Inger Anne Næss, Agnieszka Malecka, Geir E Tjønnfjord
Primary chronic cold agglutinin disease (CAD) is a well-defined clinicopathologic entity in which a bone marrow clonal B-cell lymphoproliferation results in autoimmune hemolytic anemia and cold-induced circulatory symptoms. Rituximab monotherapy or fludarabine-rituximab in combination are documented treatment options. In a prospective, non-randomized multicenter trial, 45 eligible patients received rituximab 375 mg/m(2) day 1 and bendamustine 90 mg/m(2) day 1 and 2 for 4 cycles at 28 days interval. Thirty-two patients (71%) responded; 18 (40%) achieved complete response (CR) and 14 (31%) partial response (PR)...
May 22, 2017: Blood
https://www.readbyqxmd.com/read/28529915/hemolytic-uremic-syndrome-in-adults-a-case-report
#19
Fabiel Gerardo Pérez-Cruz, Patricia Villa-Díaz, María Consuelo Pintado-Delgado, María Loreto Fernández Rodríguez, Ana Blasco-Martínez, María Pérez-Fernández
Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by platelet aggregation and mechanical damage to erythrocytes, clinically characterized by microangiopatic haemolytic anemia, thrombocytopenia and organ injury. We are reporting a case of a woman patient with severe hemolytic uremic syndrome associated to infectious diarrhoea caused by Shiga toxin-producing pathogen, who were admitted to our intensive care unit. The patient described developed as organ injury, neurological failure and acute renal failure, with need of haemodialysis technique...
May 4, 2017: World Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28516093/iatrogenic-supravalvular-aortic-stenosis
#20
Paolo Bosco, Antonella Ferrara, Samer A M Nashef
We describe a case of hemolytic anemia and proximal anastomotic site stenosis following emergency repair of a Type A aortic dissection. This rare complication led to a reoperation to correct the iatrogenic aortic stenosis and cure the consequent hemolysis. A "sandwich" technique (with two Teflon strips on the outside and inside of the aortic wall) was used in the initial repair to reinforce the suture line and prevent bleeding from the aortic anastomoses. At the time of reoperation, the inner Teflon strip at the proximal aortic anastomosis was found to have inverted into the aortic lumen, as suggested by the preoperative magnetic resonance imaging...
October 2016: Aorta (Stamford, Conn.)
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