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Hemolytic anemia coombs negative

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https://www.readbyqxmd.com/read/29504353/a-case-of-thrombosis-due-to-paroxysmal-nocturnal-haemoglobinuria-presenting-at-an-early-age
#1
Qurrat Ul Ain, Huzaifa Saleem, Sarwat Iqbal, Rabia Ghayas
Paroxysmal Nocturnal Haemoglobinuria (PNH) is an acquired, rare life-threatening disorder characterised by compliment mediated hemolytic anemia, thrombosis and impaired bone marrow function. It occasionally presents in childhood or adolescence. This is a case of a 14-year old female presented with complaints of shortness of breath, palpitation and abdominal pain whose laboratory test results were consistent with Coomb's test negative haemolytic anaemia. Contrast enhanced Computed Tomography Scan (CT scan) of abdomen revealed splanchnic circulation thrombosis as well as partially occluding thrombus in the inferior vena cava...
January 2018: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/29460700/the-lupus-patient-with-positive-rheumatoid-factor
#2
A Fedrigo, T A F G Dos Santos, R Nisihara, T Skare
Background Patients with systemic lupus erythematosus (SLE) may form clusters with clinical manifestations and autoantibodies. Objective The objective of this report is to study whether SLE patients with positive rheumatoid factor (RF) have a special clinical and/or serological profile. Methods A retrospective study of 467 SLE patients seen at a single rheumatology unit was conducted. Epidemiological data (age, gender, age at disease onset, ethnic background and tobacco use), clinical data (malar rash, photosensitivity, oral ulcers, discoid lesions, serositis, glomerulonephritis, convulsions, psychosis, hemolytic anemia, leukopenia, lymphocytopenia, arthritis and hypothyroidism) and serological profile (anti-dsDNA, anti-Ro/SS-A, anti-La/SS-B, anti-RNP, anti-Sm, IgG aCL, IgM aCL, lupus anticoagulant, direct Coombs and RF) were collected...
July 2018: Lupus
https://www.readbyqxmd.com/read/29428971/emergence-of-anti-mitochondrial-m2-antibody-in-patient-with-angioimmunoblastic-t-cell-lymphoma
#3
Shun-Ichi Wakabayashi, Takefumi Kimura, Naoki Tanaka, Satoru Joshita, Kazuhito Kawata, Takeji Umemura, Yuki Hiroshima, Hiromitsu Mori, Hikaru Kobayashi, Shuichi Wada, Eiji Tanaka
A 68-year-old woman was referred to our hospital due to fever and rash on the neck and extremities. Laboratory findings revealed hepatic dysfunction and positivity for anti-mitochondrial M2 antibody (AMA-M2). Hepatosplenomegaly and systemic lymphadenopathy were detected by enhanced computed tomography. One week after her first visit, hypoxemia, ascites, and Coomb test-positive autoimmune hemolytic anemia had newly appeared in addition to worsened fever, hepatosplenomegaly, and lymphadenopathy. Results of axillary lymph node, skin, and bone-marrow biopsies led to the diagnosis of angioimmunoblastic T-cell lymphoma (AITL), for which CEPP therapy (cyclophosphamide, etoposide, procarbazine, and prednisolone) was initiated...
February 10, 2018: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29348906/an-ank1-ivs3-2a-c-mutation-causes-exon-4-skipping-in-two-patients-from-a-chinese-family-with-hereditary-spherocytosis
#4
Xiong Wang, Liyan Mao, Na Shen, Jing Peng, Yaowu Zhu, Qun Hu, Yanjun Lu
Hereditary spherocytosis (HS) is a congenital hemolytic anemia that affects the cell membrane of red blood cells and is characterized by the presence of spherical-shaped erythrocytes in the peripheral blood film. The clinical manifestation of HS ranges from asymptomatic to severe cases that require transfusion during early childhood. HS is caused by mutations in red blood cell membrane protein encoding genes, including ANK1, EPB42, SLC4A1, SPTA1, and SPTB. Mutations of the ANK1 gene account for 75% of all HS cases, and these particular mutations are typically inherited in an autosomal dominant manner...
December 22, 2017: Oncotarget
https://www.readbyqxmd.com/read/29325287/-the-diagnostic-value-and-limits-of-diagnostic-parameters-for-wilson-s-disease
#5
X Yang
Wilson disease (WD) is a rare and treatable genetic disorder. This paper describes the new advances and author's long-term experiences in the diagnosis of WD. The characteristics in clinical and routine tests are: the age of presentation can be quite broad, the WD could not be excluded based on age only; the patients usually have mild digestive symptoms but obvious chronic liver disease signs; liver function tests may reveal normal or a mild elevation in bilirubin, ALT and AST, but quite abnormal in serum albumin and prothrombin time in most patients; Coombs-negative hemolytic anemia, normal or markedly subnormal serum alkaline phosphatase (typically < 40 IU/L) are useful for the diagnosis of fulminant WD...
December 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29130008/a-rare-association-of-autoimmune-hemolytic-anemia-with-gastric-adenocarcinoma
#6
Kavita Agrawal, Flores Alfonso
An 80-year-old male presented with dyspnea on exertion for at least two months. He also complained of progressive dysphagia and weight loss of 35 pounds over the last eight months. Initial blood tests showed hemoglobin of 6.1 g/dl, reticulocytes count of 19.7%, total bilirubin of 3.2 mg/dl, lactate dehydrogenase of 600 U/L, and haptoglobin of less than 8 mg/dl, and direct Coombs test was positive for warm immunoglobulin G. The impression was autoimmune hemolytic anemia (AIHA). The evaluation of dysphagia with esophagogastroduodenoscopy revealed a single irregular 4 cm malignant appearing ulcerated mass at the incisura angularis of the stomach...
2017: Case Reports in Oncological Medicine
https://www.readbyqxmd.com/read/28762061/direct-antiglobulin-coombs-test-in-systemic-lupus-erythematosus-patients
#7
Thelma Skare, Leandro Picelli, Thiago Alberto G Dos Santos, Renato Nisihara
The objective of the study is to study the positivity of Coombs test or direct antiglobulin test (DAT) in systemic lupus erythematosus (SLE) patients and its relationship with disease's clinical and serological profile. Retrospective study of 373 SLE patients seen at single Rheumatology Unit. Epidemiological data (age, gender, age at disease onset, auto declared ethnic background and tobacco use), clinical (malar rash, photosensitivity, oral ulcers, discoid lesions, serositis, glomerulonephritis, convulsions, psychosis, hemolytic anemia, leukopenia, lymphocytopenia and arthritis), and serological profile (anti ds DNA, anti Ro/SS-A; anti La/SS-B, Anti RNP, Anti Sm, aCl (anticardiolipin) IgG, aCl Ig M, LA or lupus anticoagulant, rheumatoid factor and direct Coombs) were collected...
September 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28620416/antiphospholipid-syndrome-multiple-manifestations-in-a-single-patient-a-high-suspicion-is-still-needed
#8
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/28588179/childhood-wilson-disease-bangladesh-perspective
#9
M Rukunuzzaman, A B Karim, M Nurullah, F Sultana, M W Mazumder, M A Rahman, S B Billah, F Begum, M Oliullah
Wilson's disease (WD) is an autosomal recessive disorder affecting copper metabolism causing copper induced damage to various organs. In children liver is commonly involved. Central nervous system, eyes, RBC, kidneys, brain and bones may also be affected. Aim of the study is to evaluate clinical & laboratory profile of Wilson's disease in children. This cross sectional descriptive study was conducted at the department of Paediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh, from January 2011 to December, 2013...
April 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28484163/progress-in-diagnosis-and-treatment-of-autoimmune-hemolytic-disorders
#10
Toyomi Kamesaki
Discovery of the Coombs' test was an epoch-making event in the history of managing autoimmune hemolytic anemia (AIHA). The Coombs' test allows immune-related hemolytic anemia to be distinguished from nonimmune acquired hemolytic anemia, but also creates a complicated category; Coombs-negative AIHA. To resolve this problem, several trials have been conducted to detect immunoglobulin (Ig) G molecules on erythrocytes (RBC-IgG) that the Coombs' test cannot detect. In Japan, RBC-IgG can be quantitated using a radioimmunoassay, but this procedure is time-consuming and expensive...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28028749/oral-cyclophosphamide-was-effective-for-coombs-negative-autoimmune-hemolytic-anemia-in-cd16-cd56-chronic-lymphoproliferative-disorder-of-nk-cells
#11
Nodoka Sekiguchi, Sayaka Nishina, Toru Kawakami, Hitoshi Sakai, Noriko Senoo, Yasushi Senoo, Toshiro Ito, Hiroshi Saito, Hideyuki Nakazawa, Tomonobu Koizumi, Fumihiro Ishida
An 84-year-old woman was referred to our hospital presenting anemia. Her hemoglobin level was 5.8 g/dL, and white blood cell count was 9400/μL, consisting of 82% lymphocytes. Given the lymphocyte phenotype (CD2+, CD3-, CD16+, and CD56-) and negative whole blood EBV viral load, we made a diagnosis of chronic lymphoproliferative disorder of NK cells (CLPD-NK). We suspected hemolytic anemia because of the high levels of reticulocytes in the peripheral blood and the low haptoglobin value. Although the direct Coombs test was negative and there was no cold agglutination, we examined her red-blood-cell-bound IgG (RBC-IgG), which was elevated...
June 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/27869428/-hemolytic-anemia-as-the-first-clinical-presentation-of-wilson-disease-a-pediatric-case
#12
José A Henao C, Kathia Valverde Muñoz, María L Ávila A
Wilson disease is an autosomal recessive disorder of the copper's hepatic metabolism; it results in toxicity due to accumulation of the mineral. The hemolytic anemia is present in 17% at some point of the disease, although it is a rare initial clinical presentation. CASE REPORT: 11 years old boy who presented with negative Coombs hemolytic anemia and elevation of liver enzymes. The possibility of Wilson disease was considered, which was confirmed with the finding of a Kayser-Fleischer ring in the eye exam...
December 1, 2016: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/27642476/-hemolytic-uremic-syndrome-hus-in-children-at-the-university-hospital-center-in-dakar-about-four-cases
#13
Aliou Thiongane, Aliou Abdoulaye Ndongo, Idrissa Demba Ba, Djibril Boiro, Papa Moctar Faye, Younoussa Keita, Aïssatou Ba, Djeynaba Fafa Cissé, Idrissa Basse, Lamine Thiam, Indou Déme Ly, Babacar Niang, Abou Ba, Amadou Lamine Fall, Saliou Diouf, Ousmane Ndiaye, Mamadou Ba, Mamadou Sarr
Hemolytic-uremic syndrome (HUS) is a common cause of organic acute renal failure (ARF) in children. It is a progressive complication of acute gastroenteritis (AGE), especially caused by Escherichia coli in children. This study aimed to describe the clinical, therapeutic and evolutionary aspects of this affection in four children. We collected four cases of HUS. The average age was 10,5 months (5-15mois), exclusively boys. Clinical examination revealed a hemolytic anemia (pallor and jaundice), oligoanuria and edematous syndrome (2 cases), arterial hypertension (1 patient), AGE associated with severe dehydration and hypovolemic shock (2 patients), consciousness disorders...
2016: Pan African Medical Journal
https://www.readbyqxmd.com/read/27460033/c3-mesangial-proliferative-glomerulonephritis-initially-presenting-with-atypical-hemolytic-uremic-syndrome-a-case-report
#14
Can Huzmeli, Ferhan Candan, Ayse Seker, Esin Yildiz, Hatice Terzi, Mansur Kayatas
BACKGROUND: Hemolytic uremic syndrome is characterized by acute renal failure, thrombocytopenia, and Coombs-negative hemolytic anemia. In C3 mesangial proliferative glomerulonephritis, an increase in mesangial cell proliferation without thickening in the glomerular capillary wall can be seen under light microscopy, but the definitive diagnosis is made with the immunohistologic demonstration of isolated C3 deposits in the mesangium. C3 glomerulonephritis may be detected in childhood; however, in this case report we describe the first case of isolated C3 glomerulonephritis together with atypical hemolytic uremic syndrome in an adult patient...
July 27, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27429816/histiocytic-sarcoma-associated-with-coombs-negative-acute-hemolytic-anemia-a-rare-presentation
#15
Sandeep Batra, Stephen C Martin, Mehdi Nassiri, Amna Qureshi, Troy A Markel
Histiocytic sarcoma (HS) rarely involves extranodal sites, such as the spleen. We report a unique pediatric case of massive splenomegaly and refractory Coombs negative hemolytic anemia (CNHA) secondary to HS. The CNHA resolved completely after an emergent splenectomy. Next generation sequencing (NGS) revealed novel ASXL1, PTPN11, KIT, and TP53 mutations, unmasking a clonal heterogeneity within the same neoplasm.
2016: Case Reports in Oncological Medicine
https://www.readbyqxmd.com/read/27408382/rare-occurrence-of-3-h-hypercalcemia-hemolytic-anemia-and-hodgkin-s-lymphoma
#16
Ankur Jain, Pankaj Malhotra, Gaurav Prakash, Subhash Varma, Narender Kumar, Asim Das
Clinicians in hematology practice commonly encounter anemia, hypercalcemia and renal failure, which when present in combination evoke a diagnostic workup for multiple myeloma. We report a 71-years old lady who presented to our hematology clinic with fever and easy fatiguability of 3 months duration and on investigations was found to have anemia and hypercalcemia. Direct Coomb's test characterized the anemia as complement mediated (anti-C3d) hemolysis. Biochemical investigations revealed normal 25(OH) Vitamin D3 and suppressed Parathormone levels and a negative workup for plasma cell dyscrasias, sarcoidosis and autoimmune disorders...
June 2016: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/27323535/-microangiopathic-hemolytic-anemia-as-presenting-symptom-of-gastric-adenocarcinoma
#17
Najib Dally, Marat Kashlikov, Raymond Farah
Metastatic adenocarcinoma presenting as microangiopathic hemolytic anemia (MAHA) is a rare finding. We report the case of a young patient who presented with MAHA as the initial symptom of metastatic gastric adenocarcinoma. Cancer-related microangiopathic hemolytic anemia is a paraneoplastic syndrome characterized by Coombs-negative hemolytic anemia with schistocytes and thrombocytopenia. A high index of suspicion and prompt diagnosis can prevent ulterior deterioration, especially when chemotherapy is the optimal treatment in these cases, which can stop the hemolysis...
April 2016: Harefuah
https://www.readbyqxmd.com/read/27249898/wilson-s-disease-in-thai-children-between-2000-and-2012-at-king-chulalongkorn-memorial-hospital
#18
Palittiya Sintusek, Voranush Chongsrisawat, Yong Poovorawan
OBJECTIVE: Wilson's disease (WD) is a rare autosomal recessive disorder characterized by copper accumulation. Clinical presentations are extraordinarily diverse, and currently no single diagnostic test can confirm WD with high accuracy. A complete understanding of the presentations and improved diagnostic methods are important for disease management. The authors' aimed to examine disease characteristics, management, and treatment outcome of WD in children, especially when genetic analysis and liver copper measurements were limited...
February 2016: Journal of the Medical Association of Thailand, Chotmaihet Thangphaet
https://www.readbyqxmd.com/read/27161726/po-38-young-women-with-breast-cancer-and-inferior-vena-cava-thrombosis-which-is-the-best-therapeutic-option-and-for-how-long
#19
A B Vilaseca, C L Capmany, F Sabsay
INTRODUCTION: Thromboembolic disease (TED) is frequent, and, thromboembolic events are the second cause of death in active cancer patients Today we have knowledge of a lot of risk and predictor factors of thrombosis in cancer, although some mechanisms underlying this increased thromboembolic risk, still remains unclear. AIM: Knowing that cancer is today curable, we want to remark that not only old people but also young should be investigated about their personal burden of thromboembolic disease to improve prognosis, and at the same time remark the need to establish different therapeutic strategies in each stage of the disease to prevent or treat TED...
April 2016: Thrombosis Research
https://www.readbyqxmd.com/read/27049736/clostridium-perfringens-infection-in-a-febrile-patient-with-severe-hemolytic-anemia
#20
Masamitsu Hashiba, Atsutoshi Tomino, Nobuyoshi Takenaka, Tomonori Hattori, Hideki Kano, Masanobu Tsuda, Naoshi Takeyama
BACKGROUND: Clostridium perfringens (C. perfringens) can cause various infections, including gas gangrene, crepitant cellulitis, and fasciitis. While C. perfringens sepsis is uncommon, it is often rapidly fatal because the alpha toxin of this bacterium induces massive intravascular hemolysis by disrupting red blood cell membranes. CASE REPORT: We present the case of a male patient with diabetes who developed a fatal liver abscess with massive intravascular hemolysis and septic shock caused by toxigenic C...
April 6, 2016: American Journal of Case Reports
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