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Madurella mycetomatis infection following allogenic stem cell transplantation for aplastic anemia  [cached]
Sanjeev Kumar Sharma,Anjan Mukherjee,Avinash Kumar Singh,Tuika Seth
Mediterranean Journal of Hematology and Infectious Diseases , 2012, DOI: 10.4084/mjhid.2012.
Abstract: Madurella mycetomatis is the most common fungal agent causing eumycetoma. The commonest clinical presentation of the infection is the appearance of multiple sinuses with discharge of grains, which are the colonies of the fungus. It is an emerging fungal infection among transplant recipients and has not been reported following stem cell transplantation. We report here a case of aplastic anemia who developed madura foot, caused by Madurella mycetomatis, following allogenic peripheral blood stem cell transplantation. The diagnosis was made by the examination of discharged black grains under microscope which reavaled presence of septate hyphae and the culture of the discharged granules grew Madurella mycetomatis. The patient was treated with voriconazole followed by excision of the lesion, which resulted in complete recovery. Considering the increasing number of patients undergoing stem cell transplantation for various hematological diseases, the implications of this fungal infection should be recognized as delay in treatment may be life-threatening.
APLASTIC ANEMIA AND VIRAL HEPATITIS
Laura Cudillo
Mediterranean Journal of Hematology and Infectious Diseases , 2009, DOI: 10.4084/mjhid.2011.
Abstract: Acquired aplastic anemia(aAA) is a severe and rare disease, characterized by hematopoietic bone marrow failure and peripheral cytopenia. The pathophysiology is immune mediated in most cases, activated T1 lymphocytes have been identified as effector cells . The disease can be successfully treated with combined immunosuppressive therapy or allogeneic hematopoietic stem cell transplantation. Hepatitis-associated aplastic anemia (HAA) is a syndrome of bone marrow failure following the development of acute seronegative hepatitis. HAA syndrome most often affects young males who presented severe pancytopenia two to three months after an episode of acute hepatitis. The clinical course of hepatitis is more frequently benign but a fulminant severe course is also described. The bone marrow failure can be explosive and severe and it is usually fatal if untreated, no correlations have been observed between severity of hepatitis and AA. In none of the studies a specific virus could be identified and most cases are seronegative for known hepatitis viruses. The clinical characteristics and response to immunotherapy indicate a central role for immune-mediated mechanism in the pathogenesis of HAA. The initial target organ of the immune response is the liver as suggested by the time interval between hepatitis and the onset of bone marrow failure. Liver histology is characterized by T cell infiltrating the parenchyma as reported in acute hepatitis. Recently in HAA it has been demonstrated intrahepatic and blood lymphocytes with T cell repertoire similar to that of confirmed viral acute hepatitis. The expanded T cell clones return to a normal distribution after response to immunosuppressive treatment, suggesting the antigen or T cell clearance. Therapeutic options are the same as acquired aplastic anemia.
Aplastic anemia presenting as hemophagocytic lymphohistiocytosis
Tiraje Celkan
Turkish Journal of Hematology , 2010,
Abstract: Two unusual cases of hemophagocytic lymphohistiocytosis (HLH) complicating aplastic anemia (AA) are described. Each patient had a history of preexisting acute hepatitis of unknown cause at the time of HLH diagnosis and infection-associated secondary HLH. They developed high fever and pancytopenia. Hemophagocytes were seen in the bone marrow. With steroid (in combination with etoposide and CyA in 1 patient), high fever disappeared and the patients’ liver function gradually recovered. As severe pancytopenia persisted, bone marrow became acellular and AA was diagnosed. Since HLH is known to be able to cause an aplastic bone marrow if untreated for a prolonged time, it is therefore in line that hepatitis-associated AA may also be associated with HLH. Aplastic anemia-associated HLH has been reported rarely, and problems in the diagnostic procedure are discussed.
Role of myeloperoxidase index in differentiation of megaloblastic and aplastic anemia  [cached]
Ziaei Jamal Eivazi,Dastgiri Saeed
Indian Journal of Medical Sciences , 2004,
Abstract: BACKGROUND: Elevated neutrophil myeloperoxidase may have a role in the diagnosis of megaloblastic erythropoiesis. AIMS: To study the differentiating role of myeloperoxidase index in megaloblastic and aplastic anemia. SETTINGS AND DESIGN: The myeloperoxidase index (MPXI) was studied in 96 patients with megaloblastic and aplastic anemia diagnosed on bone marrow aspiration and biopsy examinations. METHODS AND MATERIALS: MPXI was measured with Technicon H1 (Bayer) automated analyzer. Nonparametric Mann-Whitney statistical test was used to compare the MPXI values between groups. RESULTS: The mean MPXI in megaloblastics and aplastic anemia was 18.3 and 1.8 (p<0.001) respectively. MPXI >20 denoted megaloblastic and MPXI <-11.6 denoted aplastic anemia. CONCLUSION: MPXI measurement may assist differentiation of megaloblastic from aplastic anemia, while MPXI >20 rules out aplastic and MPXI <-11.6 rules out megaloblastic anemia.
Aplastic anemia: immunosuppressive therapy in 2010  [cached]
Antonio M. Risitano,Fabiana Perna
Pediatric Reports , 2011, DOI: 10.4081/pr.2011.s2.e7
Abstract: Acquired aplastic anemia (AA) is the typical bone marrow failure syndrome characterized by an empty bone marrow; an immune-mediated pathophysiology has been demonstrated by experimental works as well as by clinical observations. Immunusuppressive therapy (IST) is a key treatment strategy for aplastic anemia; since 20 years the standard IST for AA patients has been anti-thymocyte globuline (ATG) plus cyclosporine A (CyA), which results in response rates ranging between 50% and 70%, and even higher overall survival. However, primary and secondary failures after IST remain frequent, and to date all attempts aiming to overcome this problem have been unfruitful. Here we review the state of the art of IST for AA in 2010, focusing on possible strategies to improve current treatments. We also discuss very recent data which question the equality of different ATG preparations, leading to a possible reconsideration of the current standards of care for AA patients.
Pregnancy-associated Aplastic Anemia: Case Report
Ahmet KALE
Trakya Universitesi Tip Fakultesi Dergisi , 2010,
Abstract: Aplastic anemia in pregnancy is a rare condition with high maternal morbidity and mortality rates. Intensive hematological support during antenatal course remains the mainstay of the therapy. A successful obstetric outcome can be best accomplished with the clinical collaboration of the obstetrician and the hematologist. We present a patient with moderately severe aplastic anemia who was managed with intensive hematological support given repeatedly once in a month during pregnancy. She delivered a healthy infant by cesarean section.
Thymoma followed by aplastic anemia: two different responses to immunosuppressive therapy
Castro, Murilo Antunes de;Castro, Mariana Antunes de;Arantes, Adriano de Moraes;Roberti, Maria do Rosário Ferraz;
Revista Brasileira de Hematologia e Hemoterapia , 2011, DOI: 10.5581/1516-8484.20110125
Abstract: aplastic anemia is an uncommon complication of thymoma and is extremely infrequent after the surgical removal of a thymic tumor. aplastic anemia is a result of marrow failure and is characterized by peripheral pancytopenia and severely depressed marrow cellularity; it may be an autoimmune manifestation of thymoma. as thymoma-associated hematological dyscrasias, which include pure red cell aplasia, aplastic anemia and myasthenia gravis, are supposed to be of immunologic origin, two cases of very severe aplastic anemia following the resection of lymphocytic thymomas treated with immunosuppression are herein presented.
Coloncancer in a patient with underlying aplastic anemia: A clinical challenge  [cached]
Hilda Wong,Pierre Chan,Thomas Yau
World Journal of Clinical Oncology , 2012, DOI: 10.5306/wjco.v3.i2.29
Abstract: The association of gastrointestinal malignancy with aplastic anemia has rarely been reported in the literature. Although it is not clear whether there is any direct relationship between aplastic anemia and gastrointestinal cancers, a retrospective analysis did suggest the notion that patients with aplastic anemia might have a higher incidence of colorectal cancer. Here, we report the diagnostic and therapeutic challenges in managing a patient with aplastic anemia and advanced colorectal cancer. Early diagnosis is challenging due to overlapping symptomatology and clinical features, increased risk of diagnostic procedures, and confounding complications arising from aplastic anemia and its treatment. A high index of suspicion and multidisciplinary input are essential.
Current management of severe acquired aplastic anemia
Phillip Scheinberg
Einstein (S?o Paulo) , 2011,
Abstract: Overall survival in severe aplastic anemia has markedlyimproved in the past four decades due to advances in stem celltransplantation, immunosuppressive therapies and supportive care.Horse anti-thymocyte globulin plus cyclosporine is the standardimmunosuppressive regimen in severe aplastic anemia, and oftenemployed as initial therapy as most are not candidates for a matchedrelated stem cell transplantation. With this regimen, hematologicresponse can be achieved in 60 to 70% of cases, but relapse isobserved in 30 to 40% of responders and clonal evolution in 10 to 15%of patients. Efforts to improve outcomes beyond horse anti-thymocyteglobulin plus cyclosporine have been disappointing, with no significant improvement in the critical parameter of hematologic response, which strongly correlates with long-term survival in severe aplastic anemia. Furthermore, rates of relapse and clonal evolution have also not improved with the development of three drug regimens or with more lymphocytotoxic therapies. Therefore, horse anti-thymocyteglobulin plus cyclosporine remains the standard immunosuppressionof choice as first therapy in severe aplastic anemia. Interestingly,survival has markedly improved over the years in large part due tobetter anti-infective therapy and more successful salvage therapieswith immunosuppression and stem cell transplantation. In this reviewgeneral aspects of diagnosis and management are discussed.
An Unusual Case of Aplastic Anemia Caused by Temozolomide
Gazi Comez,Alper Sevinc,Ozlem Nuray Sever,Taner Babacan,Ibrahim Sar ,Celalettin Camci
Case Reports in Medicine , 2010, DOI: 10.1155/2010/975039
Abstract: Radiotherapy and concomitant/adjuvant therapy with temozolomide are a common treatment regimen for children and adults with high-grade glioma. Although temozolomide is generally safe, it can rarely cause life-threatening complications. Here we report a case of a 31-year-old female patient who underwent surgical resection followed by radiotherapy plus concomitant temozolomide. She developed pancytopenia after adjuvant treatment with temozolomide. A bone marrow aspiration and biopsy showed hypocellularity with very few erythroid and myeloid cells, consistent with aplastic anemia. In the English literature, aplastic anemia due to temozolomide is extremely rare.
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