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hemolytic anemia treatment

Jakub Ruszkowski, Zbigniew Heleniak, Alicja Dębska-Ślizień
Atypical hemolytic uremic syndrome (aHUS) is an extremely rare disease characterized by acute kidney injury, thrombocytopenia, and microangiopathic hemolytic anemia. Majority of patients have an underlying complement abnormality what makes aHUS possible to treat using eculizumab, antibody against the complement protein C5. Up to 2018, Polish adult patients were treated using glucocorticosteroids, plasma exchange or plasma infusion. Unfortunately, such protocols/ interventions were associated with poor prognosis; about 67% of adult patients with aHUS had been progressing to end-stage renal disease or had died within three years after diagnosis...
September 21, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
Shoichi Magawa, Hiroaki Tanaka, Fumi Furuhashi, Shintaro Maki, Masafumi Nii, Kuniaki Toriyabe, Tomoaki Ikeda
PURPOSE: Hepatitis due to herpes simplex virus (HSV) during pregnancy is rare, it is often not included in the differential disease. However, hepatitis leads to maternal death; hence, early diagnosis is necessary. Hepatic enzyme elevation in late pregnancy is often associated with obstetric-related diseases, such as acute gestational fatty liver and HELLP syndrome (hemolytic anemia, elevated liver enzymes, low platelet count). These pregnancy-related diseases often improve maternal condition after completion of pregnancy, but in patients with HSV hepatitis, invasive treatment, such as cesarean section, may lead to deterioration of maternal condition after completion of pregnancy...
September 20, 2018: Journal of Maternal-fetal & Neonatal Medicine
Yuji Tomori, Norio Motoda, Ryuji Ohashi, Takuya Sawaizumi, Mitsuhiko Nanno, Shinro Takai
RATIONALE: Preiser disease or avascular necrosis (AVN) of the scaphoid causes intolerable wrist pain and malalignment of the carpal bones. In previously reported cases, patients have had a history of steroid use for systemic illness such as autoimmune hemolytic anemia, systemic lupus erythematosus, or renal transplantation, or have had other risk factors, such as smoking, alcoholism, or infection. In particular, systemic glucocorticoid therapy has been most commonly associated with the disease...
September 2018: Medicine (Baltimore)
Candice Bulke, Clémentine Gondolf, Frédérique Grandhomme, Stéphane Allouche
Glycated hemoglobin A1c (HbA1c) reflects the mean blood glucose over the lifespan of red blood cells and has become a valuable tool both for diagnosis and monitoring of diabetes. Nevertheless, some factors may under-estimate the HbA1c value, compromising its application. The aim of this retrospective study was to evaluate the incidence rate of HbA1c lower than 4% and to identify some clinical and biological factors that can potentially reduce the HbA1c level. Between January 1st 2015 and October 1st 2017, we selected 17 patients with a HbA1c level lower than 4% that were measured in our laboratory of biochemistry at the university medical center of Caen...
October 1, 2018: Annales de Biologie Clinique
Alexandros Makis, Zoi Kanta, Dimitrios Kalogeropoulos, Nikoloaos Chaliasos
Autoimmune hemolytic anemia (AIHA) is a relatively uncommon hematological entity in children and sometimes is characterized by a severe course requiring more than one line course therapy. Treatment decisions depend on the severity and chronicity of the anemia and the characteristics of the autoantibodies. Immunosuppression with corticosteroids is the first-line treatment, especially in warm-reactive AIHA. Refractory cases are treated with immunosuppressive drugs, cytotoxic agents, androgens, or splenectomy, with various side effects and questionable efficacy...
2018: Case Reports in Hematology
Florin Zaharie, Mihai Stefan Muresan, Ciprian Tomuleasa, Gheorghe Popa, Cristina Blag, Roxana Zaharie, Claudiu Zdrehus, Mihnea Dragomir, Horia Bumbea, Alexandru Irimie, Delia Dima
Hereditary spherocytosis (HS) is a common inherited hemolytic anemia caused by a defective erythrocyte cellular membrane. Irrespective of type of surgery performed, several case reports comparing the two type of procedures, have not proven any significant difference between serum bilirubin, serum hemoglobin, red blood cells' and platelets' count, in the followup period at 6 and 12 months respectively, even if platelet count has maintained high for the first 6 months postoperatively, justifying an oral antiplatelet therapy for this time interval...
July 11, 2018: Annali Italiani di Chirurgia
Paul Knöbl
Thrombotic thrombocytopenic purpura (TTP) is a clearly defined entity of the thrombotic microangiopathies (TMA), a heterogeneous group of disorders characterized by microangiopathic hemolytic anemia with red cell fragmentation, thrombocytopenia and organ dysfunction due to disturbed microcirculation. TTP is characterized by a severe deficiency of ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), an enzyme responsible for physiological cleavage of von Willebrand factor (VWF)...
2018: Memo
Nadege Wesner, Louis Drevon, Alexis Guedon, Jean Baptiste Fraison, Salim Trad, Jean Emmanuel Kahn, Achille Aouba, Jerome Gillard, Matthieu Ponsoye, Thomas Hanslik, Clement Gourguechon, Eric Liozon, Kamel Laribi, Julien Rossignol, Olivier Hermine, Lionel Adès, Fabrice Carrat, Pierre Fenaux, Arsene Mekinian, Olivier Fain
OBJECTIVE: We report cases of myelodysplastic syndrome/myeloproliferative neoplasms (MDS/MPN) with trisomy 8 associated with inflammatory and autoimmune diseases (IADs). METHOD: Data for 21 patients with trisomy 8-MDS/MPN and IADs were analyzed and compared to 103 patients with trisomy 8-MDS/MPN without IADs. RESULTS: The median age of MDS/MPN patients with IADs was 67 [59-80]. The IADs were Behçet's-like disease in 11 (52%) patients, inflammatory arthritis in 4 (19%) and Sjögren's syndrome, autoimmune hemolytic anemia, aseptic abscess, periarteritis nodosa, Sweet's syndrome and unclassified vasculitis in one patient each...
September 15, 2018: European Journal of Haematology
T Klejtman, B Garel, P Senet, L Tribout, C Bachmeyer, A Barbaud, J-B Monfort
BACKGROUND: Digital necrosis is rarer than lower limb necrosis and constitutes a medical or surgical emergency. Etiological evaluation is required. Cold agglutinin disease is a cause of digital necrosis but diagnosis is difficult. PATIENTS AND METHODS: Herein we report the case of a 57-year-old man presenting recent paroxysmal acrosyndrome of the left hand subsequently complicated by digital necrosis following occupational exposure to cold in his work as a forklift driver...
September 6, 2018: Annales de Dermatologie et de Vénéréologie
Jaroslav Čermák, Martin Písačka
Autoimmune hemolytic anemia (AIHA) is caused by auto-antibodies directed against self red blood cell (RBC) surface antigens. A consequence may be an intravascular hemolysis mediated by activated complement or extravascular hemolysis caused by destruction of complex of RBC with autoantibody in spleen and liver. The basic classification subdivides AIHA in primary/idiopathic and secondary with known underlying disease. A classification according to the thermal range of antibody recognizes warm AIHA, cold aglutinin disease (CAD), mixed AIHA and paroxysmal cold hemoglobinuria...
2018: Vnitr̆ní Lékar̆ství
Hitomi Nakayama, Taku Kikuchi, Ryohei Abe, Keiichi Tozawa, Shintaro Watanuki, Takayuki Shimizu, Takayuki Mitsuhashi, Mitsuru Murata, Shinichiro Okamoto, Takehiko Mori
A 26-year-old man presented with fever, multiple lymphadenopathies, polyclonal hypergammaglobulinemia, and an elevated serum interleukin-6 (IL-6) level. Multicentric Castleman disease (MCD) was diagnosed by lymph node biopsy. Treatment with prednisolone (PSL) was initiated; however, its efficacy was limited. During PSL tapering, rapidly progressive anemia and thrombocytopenia developed concurrently with increased reticulocyte level, elevated serum LDH level, decreased haptoglobin level, and positive direct Coombs test...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Zhaohui Bai, Hongyu Li, Xiaozhong Guo, Yanqin Liu, Jiao Deng, Chunhui Wang, Yingying Li, Xingshun Qi
Both acute-on-chronic liver failure (ACLF) and autoimmune hemolytic anemia (AIHA) are common causes of jaundice. A co-occurrence of ACLF and AIHA is rare in clinical practice. This report describes a male elderly patient who developed persistently increased levels of total bilirubin and ascites after endoscopic retrograde cholangiopancreatography for the successful treatment of common bile duct stones. Eventually, he was diagnosed with ACLF and AIHA according to current diagnostic criteria. The patient was given conventional hepato-protective drugs, human albumin, and diuretics in combination with immune ozone without steroids, and he responded well...
August 2018: Intractable & Rare Diseases Research
Sylwia Jasinski, Chana L Glasser
While packed red blood cell (PRBC) transfusion therapy is a mainstay in the treatment of certain patients with sickle cell disease (SCD) and the standard of care for preoperative management, there are associated risks. Delayed hemolytic transfusion reaction (DHTR) is a risk of PRBC transfusion occurring 2 to 20 days from transfusion and typically presents with severe pain characteristic of vaso-occlusive crisis, fever, and hemolytic anemia. DHTRs are uncommon, occurring in only 4% to 11% of transfused patients with SCD, but may be catastrophic in nature with progression to multiorgan failure within hours...
August 31, 2018: Journal of Pediatric Hematology/oncology
Jennifer Hess, Leon Su, Frank Nizzi, Kristen Beebe, Kyrie Magee, Dana Salzberg, Jennifer Stahlecker, Holly K Miller, Roberta H Adams, Alexander Ngwube
BACKGROUND: After hematopoietic stem cell transplantation (HSCT) autoimmune hemolytic anemia (AIHA) is a known and fairly common complication. It is often refractory to conventional therapies including corticosteroids, intravenous immunoglobulin, splenectomy, and the more recently described use of monoclonal antibodies. The high morbidity associated with these severe persistent cases elucidates the gaps in alternative therapies available for treatment. STUDY DESIGN AND METHODS: We described the successful use of abatacept for severe refractory AIHA after HSCT in three patients...
September 2018: Transfusion
Lucy Neave, Marie Scully
Thrombotic microangiopathies (TMAs) are associated with microangiopathic hemolytic anemia and thrombocytopenia, resulting in microvascular thrombosis and end-organ damage. In pregnancy, this may be the result of pregnancy-related TMAs such as preeclampsia; hemolysis, elevated liver enzymes, and low platelets; or pregnancy-associated TMAs, specifically thrombotic thrombocytopenic purpura (TTP) or complement-mediated hemolytic uremic syndrome (CM HUS). TTP and CM HUS are rare disorders, and their diagnosis may be missed, no less because features at presentation may be misdiagnosed as a pregnancy-related TMA, such as hypertension, proteinuria, fetal growth restriction, or in utero fetal death...
August 18, 2018: Transfusion Medicine Reviews
Rodrigo A Sepúlveda, Rodrigo Tagle, Aquiles Jara
Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy, characterized by microangiopathic hemolytic anemia, thrombocytopenia and renal involvement. It causes end stage renal disease requiring dialysis in most affected patients. It mainly affects young adults (contrary to what was thought years ago). When aHUS is primary, the cause is a genetic mutation in the alternative complement pathway. Instead, secondary aHUS is caused by external factors that trigger the disease by themselves or in combination with a genetic vulnerability...
June 2018: Revista Médica de Chile
Toshimasa Shimizu, Naoki Iwamoto, Momoko Okamoto, Yushiro Endo, Sosuke Tsuji, Ayuko Takatani, Takashi Igawa, Masataka Umeda, Shoichi Fukui, Remi Sumiyoshi, Mineaki Kitamura, Tomohiro Koga, Shin-Ya Kawashiri, Kunihiro Ichinose, Mami Tamai, Hideki Nakamura, Tomoki Origuchi, Tomoya Nishino, Atsushi Kawakami
A 44-year-old Japanese man with a 14-year history of limited cutaneous systemic sclerosis (SSc) was admitted with a fever, hypertension, anemia, thrombocytopenia, and renal dysfunction. On admission, hypertension, hyperreninemia, acute renal dysfunction, hemolytic anemia, and thrombocytopenia led to the diagnosis of sclerotic renal crisis (SRC) complicated with thrombotic microangiopathy (TMA). The patient had also been infected with influenza B virus almost six days before admission. Following treatment with plasma exchange, an angiotensin-converting enzyme inhibitor, and an anti-virus agent, his general condition improved...
August 24, 2018: Internal Medicine
Neşe Yaralı, Özlem Arman Bilir, Arzu Yazal Erdem, Vildan Çulha, Abdurrahman Kara, Namık Özbek
BACKGROUND AND AIM: Autoimmune hemolytic anemia (AIHA) is characterized by autoimmune destruction of erythrocytes. In this retrospective study, the clinical, laboratory features and treatment responses of patients with primary AIHA were evaluated. MATERIAL AND METHODS: 21 consecutive patients diagnosed with primary AIHA in a children's hospital from 2008 to 2016 were included. Clinical, laboratory findings and treatment responses were analyzed. RESULTS: Twenty-one patients, aged 6 months-15 years, with direct antiglobulin test positive anemia were presented...
August 8, 2018: Transfusion and Apheresis Science
Han-Mou Tsai
Atypical hemolytic uremic syndrome commonly presents with the triad of microangiopathic hemolytic anemia, thrombocytopenia and renal function impairment without an antecedent hemorrhagic diarrhea. Less known are extra-renal complications due abnormal vascular permeability although these are a major cause of morbidity and mortality for the patients. Furthermore it is increasingly recognized that the disease may present with hypertension or renal function impairment with no or mild thrombocytopenia and microangiopathic hemolytic anemia...
August 23, 2018: American Journal of Medicine
Deng-Ho Yang, Meng-Yin Yang
Evans syndrome is a rare disorder with presentations of autoimmune hemolytic anemia and immune thrombocytopenia, in the absence of any underlying cause. Here, we reported a case with a history of Evans syndrome for seven years. A persistent scrotal ulcer with severe pain occurred for two weeks. He called at our emergency room because of a painful, necrolytic cutaneous ulcer over the scrotal region. A biopsy showed sterile dermal neutrophilia with lymphocytic vasculitis, and pyoderma gangrenosum was impressed...
August 22, 2018: Journal of Clinical Medicine
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