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hemolytic anemia treatment

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https://www.readbyqxmd.com/read/28330575/clinical-management-of-the-homozygous-%C3%AE-thalassemia-with-unusual-mandibular-manifestation-of-hematopoiesis
#1
J A Ruiz-Roca, R E Oñate-Sánchez, I Urrutia-Rodríguez, A Martínez-Izquierdo, D Mengual-Pujante, F J Rodríguez-Lozano
Alpha (α)-thalassemias are the most common genetic disorder of hemoglobin (Hb) synthesis, affecting up to 5% of the world's population. These congenital hemolytic anemias induce extramedullary hematopoiesis, including the liver, spleen, sinuses, and the diploic spaces of the skull. Oral health problems in patients with thalassemias are mostly related to a varied degree of facial deformities, malocclusions, and/or dental arch dimensions. We present a case with a 49-year-old man, diagnosed with homozygous α thalassemia that came to the Faculty of Dentistry at the University of Murcia for a dental treatment...
February 2017: J Stomatol Oral Maxillofac Surg
https://www.readbyqxmd.com/read/28324773/haptoglobin-is-frequently-low-in-patients-with-myelofibrosis-clinical-relevance
#2
Paolo Strati, Lucia Masarova, Prithviraj Bose, Naval Daver, Naveen Pemmaraju, Srdan Verstovsek
A recent study, showing the absence of paroxysmal nocturnal hemoglobinuria clones in myelofibrosis, has reopened the debate around the role of decreased haptoglobin in this disease. We present here a large prospective analysis of the clinical significance of low haptoglobin in 152 patients with myelofibrosis. Low haptoglobin (<32mg/dL) was observed in 50 patients (33%). Decreased haptoglobin did not associate with low hemoglobin levels, positive Coombs test or abnormal liver function tests, suggesting it is not result of autoimmune hemolytic anemia or liver cirrhosis...
March 8, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28286630/a-fatal-case-of-immune-hyperhemolysis-with-bone-marrow-necrosis-in-a-patient-with-sickle-cell-disease
#3
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/28277850/diagnosis-and-management-of-catastrophic-antiphospholipid-syndrome
#4
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/28277805/intrauterine-transfusion-and-non-invasive-treatment-options-for-hemolytic-disease-of-the-fetus-and-newborn-review-on-current-management-and-outcome
#5
Carolien Zwiers, Inge van Kamp, Dick Oepkes, Enrico Lopriore
Hemolytic disease of the fetus and newborn (HDFN) remains a serious pregnancy complication which can lead to severe fetal anemia, hydrops and perinatal death. Areas covered: This review focusses on the current prenatal management, treatment with intrauterine transfusion (IUT) and promising non-invasive treatment options for HDFN. Expert commentary: IUTs are the cornerstone in prenatal management of HDFN and have significantly improved perinatal outcome in the past decades. IUT is now a relatively safe procedure, however the risk of complications is still high when performed early in the second trimester...
March 20, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28273010/post-babesiosis-warm-autoimmune-hemolytic-anemia
#6
Ann E Woolley, Mary W Montgomery, William J Savage, Maureen O Achebe, Kathleen Dunford, Sarah Villeda, James H Maguire, Francisco M Marty
Background Babesiosis, a tickborne zoonotic disease caused by intraerythrocytic protozoa of the genus babesia, is characterized by nonimmune hemolytic anemia that resolves with antimicrobial treatment and clearance of parasitemia. The development of warm-antibody autoimmune hemolytic anemia (also known as warm autoimmune hemolytic anemia [WAHA]) in patients with babesiosis has not previously been well described. Methods After the observation of sporadic cases of WAHA that occurred after treatment of patients for babesiosis, we conducted a retrospective cohort study of all the patients with babesiosis who were cared for at our center from January 2009 through June 2016...
March 9, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28267090/a-rare-cause-of-neonatal-hemolytic-anemia-glutathione-synthetase-deficiency
#7
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/28239468/immunotherapy-associated-autoimmune-hemolytic-anemia
#8
Uqba Khan, Farman Ali, Muhammad Siddique Khurram, Awais Zaka, Tarik Hadid
BACKGROUND: Immunotherapy has been widely used in the treatment of several solid and hematologic malignancies. Checkpoint inhibitors have been the forefront of cancer immunotherapy in recent years. Cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) and programmed cell death 1 (PD-1) pathway are the prototypic checkpoint targets for immunotherapy. When combined, CTLA-4 and PD-1 checkpoint inhibitors work synergistically, but with increased probability of toxicity. The following case represents an unusual adverse effect of combined treatment with ipilimumab and nivolumab used for treatment of metastatic melanoma...
2017: Journal for Immunotherapy of Cancer
https://www.readbyqxmd.com/read/28225953/drug-reaction-with-eosinophilia-and-systemic-symptoms-dress-and-its-relation-with-autoimmunity-in-a-reference-center-in-mexico
#9
Juan Manuel Ruiz Matta, Silvia Méndez Flores, Judith Domínguez Cherit
BACKGROUND: Drug reaction with eosinophilia and systemic symptoms is a severe adverse drug reaction, with a reported mortality of 10%. Long-term outcomes involve organic failure and autoimmune diseases in some populations. OBJECTIVE: To evaluate the clinical prognosis of patients with drug reaction with eosinophilia and systemic symptoms. METHODS: We conducted a retrospective review at a referral hospital in Mexico City in a period of 22 years (1992-2013), looking up for records with diagnosis of DRESS according to RegiSCAR criteria...
January 2017: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/28210931/serotonin-syndrome-from-5-hydroxytryptophan-supplement-ingestion-in-a-9-month-old-labrador-retriever
#10
Hopkins Jennifer, Pardo Mariana, Bischoff Karyn
INTRODUCTION: 5-Hydroxytryptophan (5-HTP) supplements are available over the counter and labeled as sleeping aids and anxiolytics for human use. 5-HTP is a serotonin precursor and overdose can lead to serotonin syndrome. CASE REPORT: A 9-month-old female Labrador retriever was evaluated after ingestion of a 5-HTP supplement. Signs of agitation developed within 1 h of ingestion, and emesis was attempted by the owner with  3% hydrogen peroxide (H2O2) orally. On presentation, the dog was obtunded, bilaterally mydriatic and salivating...
February 16, 2017: Journal of Medical Toxicology: Official Journal of the American College of Medical Toxicology
https://www.readbyqxmd.com/read/28210839/thrombotic-microangiopathy-caused-by-methionine-synthase-deficiency-diagnosis-and-treatment-pitfalls
#11
Maria Helena Vaisbich, Andressa Braga, Maria Gabrielle, Clarissa Bueno, Flávia Piazzon, Fernando Kok
BACKGROUND: Inborn errors of cobalamin (Cbl) metabolism form a large group of rare diseases. One of these, Cbl deficiency type C (CblC), is a well-known cause of thrombotic microangiopathy (TMA), especially in infants. However, there has only been a single published case of TMA associated to Cbl deficiency type G (CblG), also known as methionine synthase deficiency (MSD). CASE DIAGNOSIS/TREATMENT: A 21-month-old boy presented with pallor and oral ulcers during episodes of upper respiratory infection (URI)...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28193740/development-of-delayed-hemolytic-anemia-after-treatment-with-oral-artemether-lumefantrine-in-two-patients-with-severe-falciparum-malaria
#12
Yasuhiro Tsuchido, Fukumi Nakamura-Uchiyama, Kasumi Toyoda, Moritoshi Iwagami, Kentaro Tochitani, Koh Shinohara, Naokuni Hishiya, Taku Ogawa, Kenji Uno, Kei Kasahara, Yukiteru Ouji, Shigeyuki Kano, Keiichi Mikasa, Tsunehiro Shimizu, Masahide Yoshikawa, Haruhiko Maruyama
Recently, reports of delayed hemolytic anemia after treatment with artemisinin and its derivatives have emerged. Here we report two cases of delayed hemolytic anemia in a patient with severe falciparum malaria after treatment with oral artemether-lumefantrine (AL). The first patient, a 20-year-old Japanese male student, was diagnosed with falciparum malaria and was administered AL. As having a high parasitemia rate (20.6%) was the only severe malaria criterion met in this case and his general condition was stable, we continued with AL treatment...
February 13, 2017: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/28182327/microangiopathic-hemolytic-anemia-due-to-adamts-13-loss-in-idiopathic-systemic-capillary-leak-syndrome-reply
#13
Daniel C Moreira, Jorge Di Paola
We sincerely appreciate Dr Thachil's letter, which provides further insight into the case we recently published, in which ADAMTS-13 was likely lost in third-space fluid in a patient with idiopathic systemic capillary leak syndrome (SCLS) and that this loss was proposed as a novel mechanism for microangiopathic hemolytic anemia (MAHA) [1]. As mentioned in the original publication, identifying high levels of ADAMTS-13 in the transudate fluid would have proven the proposed mechanism, but unfortunately a sample of the transudate fluid was not available...
February 9, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28133771/thrombotic-thrombocytopenic-purpura-misdiagnosed-as-autoimmune-cytopenia-causes-of-diagnostic-errors-and-consequence-on-outcome-experience-of-the-french-thrombotic-microangiopathies-reference-centre
#14
Maximilien Grall, Elie Azoulay, Lionel Galicier, François Provôt, Alain Wynckel, Pascale Poullin, Steven Grange, Jean-Michel Halimi, Alexandre Lautrette, Yahsou Delmas, Claire Presne, Mohamed Hamidou, Stéphane Girault, Frédéric Pène, Pierre Perez, Tarik Kanouni, Amélie Seguin, Christiane Mousson, Dominique Chauveau, Mario Ojeda-Uribe, Virginie Barbay, Agnès Veyradier, Paul Coppo, Ygal Benhamou
Thrombotic thrombocytopenic purpura (TTP) has a devastating prognosis without adapted management. Sources of misdiagnosis need to be identified to avoid delayed treatment. We studied 84 patients with a final diagnosis of severe (<10%) acquired ADAMTS13 deficiency-associated TTP from our National database that included 423 patients, who had an initial misdiagnosis (20% of all TTP). Main diagnostic errors were attributed to autoimmune thrombocytopenia, associated (51%) or not (37%) with autoimmune hemolytic anemia...
April 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28128521/itpa-gene-variation-and-ribavirin-induced-anemia-in-patients-with-genotype-2-chronic-hepatitis-c-treated-with-sofosbuvir-plus-ribavirin
#15
Miyako Murakawa, Yasuhiro Asahina, Hiroko Nagata, Mina Nakagawa, Sei Kakinuma, Sayuri Nitta, Fukiko Kawai-Kitahata, Satoshi Otani, Shun Kaneko, Masato Miyoshi, Tomoyuki Tsunoda, Yu Asano, Ayako Sato, Yasuhiro Itsui, Seishin Azuma, Toshihiko Nouchi, Yohei Furumoto, Tooru Asano, Yoshimichi Chuganji, Shuji Tohda, Mamoru Watanabe
AIM: Sofosbuvir (SOF) and ribavirin (RBV) combination therapy produces a sustained response in many patients with genotype 2 chronic hepatitis C. However, RBV-induced anemia is a troublesome side effect that may limit this treatment. Genetic variation leading to inosine triphosphatase (ITPA) deficiency is known to protect against RBV-induced hemolytic anemia. This study aimed to evaluate the relationships between the efficacy and safety of SOF/RBV treatment and ITPA gene variants. METHODS: Ninety patients with genotype 2 chronic hepatitis C treated with SOF/RBV were studied...
January 27, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28123330/the-plethora-clinical-manifestations-and-treatment-options-of-autoimmunity-in-patients-with-primary-immunodeficiency
#16
Hatice Ezgi Barış, Ayça Kıykım, Ercan Nain, Ahmet Oğuzhan Özen, Elif Karakoç-Aydıner, Safa Barış
AIM: Although the association between primary immunodeficiency and autoimmunity is already well-known, it has once again become a topic of debate with the discovery of newly-defined immunodeficiencies. Thus, investigation of the mechanisms of development of autoimmunity in primary immunodefficiency and new target-specific therapeutic options has come to the fore. In this study, we aimed to examine the clinical findings of autoimmunity, autoimmunity varieties, and treatment responses in patients who were genetically diagnosed as having primary immunodeficiency...
December 2016: Türk Pediatri Arşivi
https://www.readbyqxmd.com/read/28121924/autoimmune-diseases-and-hiv-infection-a-cross-sectional-study
#17
Emilie Virot, Antoine Duclos, Leopold Adelaide, Patrick Miailhes, Arnaud Hot, Tristan Ferry, Pascal Seve
To describe the clinical manifestations, treatments, prognosis, and prevalence of autoimmune diseases (ADs) in human immunodeficiency virus (HIV)-infected patients.All HIV-infected patients managed in the Infectious Diseases Department of the Lyon University Hospitals, France, between January 2003 and December 2013 and presenting an AD were retrospectively included.Thirty-six ADs were found among 5186 HIV-infected patients which represents a prevalence of 0.69% including immune thrombocytopenic purpura (n = 15), inflammatory myositis (IM) (n = 4), sarcoidosis (n = 4), Guillain-Barré syndrome (GBS) (n = 4), myasthenia gravis (n = 2), Graves' disease (n = 2), and 1 case of each following conditions: systemic lupus erythematosus, rheumatoid arthritis, autoimmune hepatitis, Hashimoto thyroiditis and autoimmune hemolytic anemia...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28104125/efficacy-of-eculizumab-therapy-for-atypical-hemolytic-uremic-syndrome-recurrence-and-antibody-mediated-rejection-progress-after-renal-transplantation-with-preformed-donor-specific-antibodies-case-report
#18
T Yamamoto, Y Watarai, K Futamura, M Okada, M Tsujita, T Hiramitsu, N Goto, S Narumi, A Takeda, T Kobayashi
Atypical hemolytic uremic syndrome (aHUS) develops as the result of unregulated complement progression and precipitates de novo thrombotic microangiopathy. Plasma therapy is used to control the progression of the complement cascade, but that therapy is not effective in all patients and is accompanied by risk of infection and/or allergy. Eculizumab has been reported as an efficient therapy for aHUS. We report the case of a 35-year old woman who underwent effective eculizumab therapy for aHUS recurrence and antibody-mediated rejection (AMR) progress after renal transplantation with preformed donor-specific antibodies (DSA)...
January 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28101432/atypical-hemolytic-uremic-syndrome-in-first-trimester-pregnancy-successfully-treated-with-eculizumab
#19
Gabriela Andries, Michael Karass, Srikanth Yandrapalli, Katherine Linder, Delong Liu, John Nelson, Rahul Pawar, Savneek Chugh
BACKGROUND: Atypical hemolytic uremic syndrome is a rare disorder which is known to cause acute thrombotic microangiopathy during pregnancy with poor maternal and fetal outcomes. Atypical hemolytic uremic syndrome is caused mostly by dysregulation of alternative complement pathway secondary to genetic mutations. Most of the cases reported have been in the post-partum period. We report a rare case of a patient who presents with thrombotic microangiopathy in the first trimester of her eleventh pregnancy and was successfully treated with eculizumab...
2017: Experimental Hematology & Oncology
https://www.readbyqxmd.com/read/28078984/rasburicase-induced-methemoglobinemia-in-a-patient-with-glucose-6-phosphate-dehydrogenase-deficiency
#20
Maliha Khan, Shilpa Paul, Saad Farooq, Thein Hlaing Oo, Priya Ramshesh, Nitin Jain
BACKGROUND: Rasburicase is commonly used in patients with hematologic malignancies for tumor lysis syndrome prophylaxis and management. Methemoglobinemia is a serious rare adverse effect of rasburicase, more common in patients with G6PD deficiency. Prompt diagnosis and appropriate management of this condition can make the difference between successful recovery and significant morbidity. Here we discuss the link of rasburicase with methemoglobinemia and the pathophysiology behind increased incidence of this side effect in G6PD deficient patients...
January 11, 2017: Current Drug Safety
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