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Aplastic Anaemia

Krista Vaht, Magnus Göransson, Kristina Carlson, Cecilia Isaksson, Stig Lenhoff, Anna Sandstedt, Bertil Uggla, Jacek Winiarski, Per Ljungman, Mats Brune, Per-Ola Andersson
OBJECTIVES: Antithymocyte globulin (ATG)-based immunosuppression remains a cornerstone in aplastic anaemia (AA) treatment. However, most ATG studies are not population-based and knowledge about real-world results concerning response and outcome could offer important information for treating physicians. METHODS: We have recently performed a nationwide retrospective cohort study on all AA patients diagnosed in Sweden in 2000-2011 and now present treatment and outcome data on patients receiving first-line ATG...
March 12, 2018: European Journal of Haematology
Chien-Yuan Chen, Wang-Huei Sheng, Feng-Ming Tien, Po-Chu Lee, Shang-Yi Huang, Jih-Luh Tang, Woei Tsay, Hwei-Fang Tien, Po-Ren Hsueh
BACKGROUND/PURPOSE: This study was aimed to investigate clinical characteristics and treatment outcomes of pulmonary invasive fungal infection (IFI) among patients with hematological malignancy. METHODS: All patients with hematological malignancy who were treated at a medical centre from 2008 to 2013 were evaluated. Pulmonary IFI was classified according to the European Organization for Research and Treatment of Cancer 2008 consensus. RESULTS: During the study period, 236 (11...
January 31, 2018: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
Shefali K Sharma, Prasanth Balasubramanian, Bishan Radotra, Manphool Singhal
Mucormycosis, a rare opportunistic infection seen in immunocompromised hosts, is caused by fungi of Mucorales family. It may be confined to the organs, such as rhinocerebral and pulmonary mucormycosis, or may cause disseminated infection. A 14-year-old boy presented to our clinic with fever and left upper quadrant abdominal pain, and on evaluation was found to have pancytopaenia, and imaging revealed ill-defined splenic collection with thrombus in the splenic vein. He was started on empirical intravenous antibiotics, followed by antifungals empirically as he did not show any improvement clinically...
February 8, 2018: BMJ Case Reports
Sergio Fasullo, Stefania Davì, Gioacchino Cosenza, Francesca Di Franco, Nicola La Manna, Alfonso Giubilato, Graziella Vetrano, Giorgio Maringhini
This case report describes agranulocytosis immediately after oral administration of dabigatran in a 68 years old man with atrial fibrillation (AF). Dabigatran is an oral, reversible and competitive thrombin inhibitor that has shown promising results. In patients with atrial fibrillation of RE-LY study (Randomized Evaluation of Long-Term Anticoagulant Therapy), dabigatran administered at a dose of 150 mg, as compared with warfarin, was associated with lower rates of stroke and systemic embolism but similar rates of major hemorrhage...
February 5, 2018: Journal of Thrombosis and Thrombolysis
Mrinalini Kotru, Rahul Sharma, Suman Kumar Pramanik, Abhishek Purohit, Gurmeet Singh, Avinash Kumar Singh, Deepti Muterja, Pravas Mishra, Tulika Seth, Seema Tyagi, Manoranjan Mahapatra, Hara Prasad Pati, Renu Saxena
BACKGROUND & OBJECTIVES: Diagnosis of paroxysmal nocturnal haemoglobinuria (PNH), a rare haematopoietic stem cell disorder, is challenging in patients with bone marrow failure (BMF) syndrome like aplastic anaemia (AA). This study was conducted with the aim to test the efficacy of the newly recommended markers viz. anti-CD16 and CD66b antibody over the existing anti-CD55 and CD59 antibody for PNH diagnosis in India. METHODS: This study was conducted on 193 suspected cases of PNH by flow cytometry using lyse wash technique to stain the granulocytes with CD16/CD66b and CD55/CD59...
September 2017: Indian Journal of Medical Research
R Valli, L Vinti, A Frattini, M Fabbri, G Montalbano, C Olivieri, A Minelli, F Locatelli, F Pasquali, E Maserati
Background: The majority of the cases of bone marrow failure syndromes/aplastic anaemias (BMFS/AA) are non-hereditary and considered idiopathic (80-85%). The peripheral blood picture is variable, with anaemia, neutropenia and/or thrombocytopenia, and the patients with idiopathic BMFS/AA may have a risk of transformation into a myelodysplastic syndrome (MDS) and/or an acute myeloid leukaemia (AML), as ascertained for all inherited BMFS. We already reported four patients with different forms of BMFS/AA with chromosome anomalies as primary etiologic event: the chromosome changes exerted an effect on specific genes, namely RUNX1, MPL, and FLI1, leading to the disease...
2018: Molecular Cytogenetics
L-P Xu, Z-L Xu, F-R Wang, X-D Mo, T-T Han, W Han, Y-H Chen, Y-Y Zhang, J-Z Wang, Y Wang, C-H Yan, Y-Q Sun, F-F Tang, X-H Zhang, X-J Huang
We conducted a retrospective analysis to evaluate outcomes of haploidentical transplantation in adult severe aplastic anaemia (SAA) patients. Fifty-one adults received haploidentical transplantation between May 2011 and December 2016. Patients were administered busulfan (Bu), cyclophosphamide (Cy) and anti-thymoglobulin (ATG) as conditioning regimens, followed by bone marrow and peripheral blood transplantation. The patients' median age was 25 years. Forty-nine patients survived for more than 28 days and all achieved donor myeloid engraftment...
January 15, 2018: Bone Marrow Transplantation
Atreyee Dutta, Rajib De, Tuphan Kanti Dolai, Pritha Pal, Shanoli Ghosh, Pradip Kumar Mitra, Ajanta Halder
OBJECTIVES: Fanconi anaemia (FA) is a rare inherited bone marrow failure and autosomal recessive blood disorder. FA patients have a higher risk of cancer, including acute myeloid leukaemia and squamous cell carcinoma. Maximum, but not all, affected individuals have one or more somatic abnormalities, including skin, skeletal, genitourinary, gastrointestinal, cardiac and neurological anomalies, etc. Positive stress cytogenetics has immense implications for the treatment and management of FA...
January 7, 2018: Hematology (Amsterdam, Netherlands)
Yu-Yan Hwang, Harinder Gill, Thomas S Y Chan, Garret M K Leung, Carol Y M Cheung, Yok-Lam Kwong
OBJECTIVE: The thrombopoietin mimetic eltrombopag has been used in clinical trials for the frontline and salvage treatment of aplastic anaemia (AA). Eltrombopag was investigated in AA patients on a non-trial all-comer basis. METHODS: Consecutive newly diagnosed and relapsed/refractory AA patients were treated with eltrombopag. RESULTS: In a 4.5-year period, 20 consecutive AA patients (newly diagnosed, N = 10; relapsed/refractory, N = 10) at a median age of 47 (22-84) years were treated with eltrombopag...
January 5, 2018: Hematology (Amsterdam, Netherlands)
Cristina Gonçalves, Sandra Ferreira, Susana Nobre, Isabel Gonçalves
Prognosis of hepatitis-associated aplastic anaemia (HAAA) was improved with haematopoietic stem cell transplantation (HSCT) and immunosuppression, but the long-term outcome remains undefined. Case 1: a girl aged 3 years with acute liver failure (ALF) submitted to orthotopic liver transplantation (OLT) subsequently developed aplastic anaemia and HSCT from a compatible sibling was performed. Post-HSCT, the patient developed post-transplant lymphoproliferative disorder and rituximab was administered with good response...
December 13, 2017: BMJ Case Reports
Ruhi Kumar, Sridhar Chivukula, Goutham Reddy Katukuri, U K Chandrasekhar, K N Shivashankar
Antiepileptic Drugs (AEDs) are commonly associated with haematological disorders, including anaemia, thrombocytopenia, neutropenia and even bone marrow failure. Fatal disorders like aplastic anaemia are uncommon. On exploring through the literature, older AEDs are more associated with haematological alterations than newer AEDs, and careful monitoring is warranted especially with phenytoin, carbamazepine and valproate. The exact cause of these alterations is not established, though immune mechanisms and pharmacology of individual drugs are the proposed mechanisms, a further research along this path is underway...
September 2017: Journal of Clinical and Diagnostic Research: JCDR
Martin Hoenigl, Juergen Prattes, Peter Neumeister, Albert Wölfler, Robert Krause
Recent years have seen important advances in the diagnosis of invasive pulmonary aspergillosis (IPA), complemented by the introduction of new therapies. Despite this, IPA remains a major cause of infection-related mortality in patients with haematological diseases. There are two main reasons for this. First, diagnosis of IPA remains a challenge, since risk factors and the clinical, radiological and mycological presentations vary not only by fungal disease stage, but also by patient group (eg neutropenic vs non-neutropenic patients)...
March 2018: Mycoses
Biju George, Nisham Pn, Anup J Devasia, Uday Kulkarni, Anu Korula, Kavitha M Lakshmi, Aby Abraham, Alok Srivastava, Vikram Mathews
High-dose cyclophosphamide (PTCY) after allogeneic hematopoietic cell transplantation (HSCT) has been shown to be effective in preventing graft-versus-host disease (GVHD) after HLA-matched bone marrow transplantation. We performed a phase II study of PTCY given at 50 mg/kg i.v. on days 3 and 4 as the sole GVHD prophylaxis after HSCT for severe aplastic anemia (SAA) in patients receiving granulocyte colony-stimulating factor-mobilized peripheral blood stem cell (PBSC) grafts from HLA-matched related donors after conditioning with fludarabine, CY, and single-dose total body irradiation...
March 2018: Biology of Blood and Marrow Transplantation
Nicholas Hendren, Joseph Moore, Sandra Hofmann, Siayareh Rambally
A previously healthy 44-year-old woman presented with 3 days of worsening petechial rash, epistaxis and fatigue. Admission labs revealed pancytopenia, low reticulocyte index and elevated liver enzymes. Bone marrow biopsy demonstrated a profoundly hypocellular bone marrow without dysplasia and additional testing demonstrated an acute hepatitis B infection. In the context of an acute hepatitis B infection, elevated liver enzymes and aplastic anaemia, our patient was diagnosed with severe hepatitis-associated aplastic anaemia due to an acute hepatitis B infection...
October 3, 2017: BMJ Case Reports
Sundaramurthi Sudharsanan, Chellappa Vijayakumar, Kumar Manish, T P Elamurugan, Ali S Manwar
Gas gangrene is one of the most serious infections of traumatic and surgical wounds. The importance lies in the fact that, if not managed at the right time, the condition is fatal. Though perianal cellulitis and gangrene are commonly reported in immunocompromised patients, we report the case of a patient with gas gangrene involving only the anal canal extending to the rectum, a rare presentation to be reported in literature.  An 18-year-old lady, a patient of aplastic anaemia - immunodeficiency, developed gas gangrene of the anal canal possibly due to faecal contamination of anal fissures...
July 13, 2017: Curēus
Qian Niu, Qiang Zhou, Yumei Liu, Hong Jiang
Acquired aplastic anaemia (AA) is caused by T-cells migrating to and attacking bone marrow (BM) in response to chemokines (e.g., CXCR4). We investigated CXCR4 expressions on circulating T-cell subsets, plasma IL-17A concentrations, and their correlations with AA manifestations. We enrolled 71 patients with acquired AA (36 severe AA cases [SAA] and 35 non-severe AA cases [NSAA]) and 42 healthy volunteers. We used flow cytometry and ELISA to measure circulating CD4(+) and CD8(+) T-cells, their CXCR4 expressions, and plasma IL-17A concentrations...
August 22, 2017: Scientific Reports
Hee S Park, Si N Park, Kyongok Im, Sung-Min Kim, Jung-Ah Kim, Sang M Hwang, Dong S Lee
We investigated the frequencies of cytogenetic aberrations and somatic mutations of prognostic relevance in 393 patients with aplastic anaemia (AA). Clonality was determined by G-banding/fluorescence in situ hybridization (FISH) (n = 245), and targeted capture sequencing was performed for 88 haematopoiesis-related genes (n = 70). The telomere length (TL) of bone marrow nucleated cells was measured at the single cell level by FISH (n = 135). Eighteen (4·6%) patients showed disease progression, and monosomy 7 (50·0%) was the most predominant cytogenetic evolution at disease transformation...
August 2017: British Journal of Haematology
Shahzad Sarwar, Monazza Chaudhry, Natasha Ali
Paroxysmal nocturnal haemoglobinuria (PNH) is a rare, acquired, life-threatening haematological disorder. It is characterised by complement induced haemolytic anaemia, thrombosis and impaired bone marrow function. Thrombosis most commonly occurs in the hepatic, portal, superior mesenteric and cerebral veins. A22-year female, previously diagnosed with severe aplastic anaemia treated with anti-lymphocyte globulin (ALG) and cyclosporine, had become transfusion independent for more than 10 years. She presented with abdominal pain and vomiting, initially diagnosed with portal and superior mesenteric vein thrombosis...
November 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Atsushi Narita, Hideki Muramatsu, Yusuke Okuno, Yuko Sekiya, Kyogo Suzuki, Motoharu Hamada, Shinsuke Kataoka, Daisuke Ichikawa, Rieko Taniguchi, Norihiro Murakami, Daiei Kojima, Eri Nishikawa, Nozomu Kawashima, Nobuhiro Nishio, Asahito Hama, Yoshiyuki Takahashi, Seiji Kojima
The clinical significance of paroxysmal nocturnal haemoglobinuria (PNH) in children with aplastic anaemia (AA) remains unclear. We retrospectively studied 57 children with AA between 1992 and 2010. During the follow-up, five patients developed clinical PNH, in whom somatic PIGA mutations were detected by targeted sequencing. The 10-year probability of clinical PNH development was 10·2% (95% confidence interval, 3·6-20·7%). Furthermore, the detection of minor PNH clones by flow cytometry at AA diagnosis was a risk factor for the subsequent development of clinical PNH...
September 2017: British Journal of Haematology
Hai Cheng, Jiang Cao, Wei Chen, Kun-Ming Qi, Zhi-Ling Yan, Kai-Lin Xu
OBJECTIVE: To evaluate the clinical significance of thrombopoietin (TPO) level in diagnosis of pateints with aplastic anaemia (AA). METHODS: The TPO levels in sera from 54 AA patients and 119 healthy controls were examined. A total of 92 samples were collected from AA patients including 43 samples harvested at diagnosis, 23 samples in the cytopenic period after treatment, and 26 samples of patients in partial (n=10) or complete remission (n=16) following immunosuppressive treatment...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
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