An 8-Year-Old Girl with Autoimmune Hepatitis Following Aplastic Anemia.

An 8-Year-Old Girl with Autoimmune Hepatitis Following Aplastic Anemia. J Nippon Med Sch. 2018;85(4):241-244 Authors: Nishigori A, Hatori T, Asano T Abstract We herein report a case of severe aplastic anemia diagnosed in an 8-year-old girl with a previous diagnosis of autoimmune hepatitis. We found significantly increased CD8+ and CD68+ cell numbers in her bone marrow, which can induce severe organ damage, refractory to immunosuppressive therapy. PMID: 30259895 [PubMed - in process]
Source: Journal of Nippon Medical School - Category: Universities & Medical Training Authors: Tags: J Nippon Med Sch Source Type: research

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Authors: Sawada K, Takai A, Yamada T, Araki O, Yamauchi Y, Eso Y, Takahashi K, Shindo T, Sakurai T, Ueda Y, Seno H Abstract Hepatitis-associated aplastic anemia (HAAA) is a variant of acquired aplastic anemia and characterized by bone marrow failure that follows the development of acute hepatitis. We herein report a rare case of HAAA with rapid progression of liver fibrosis due to repeated hepatitis. A pathological examination of liver specimens revealed liver fibrosis progression over a short period. Immunosuppressive therapy with cyclosporine effectively cured both the pancytopenia and hepatitis. Our case suggest...
Source: Internal Medicine - Category: Internal Medicine Tags: Intern Med Source Type: research
Chapin et  al reported the use of corticosteroid treatment for children with indeterminate pediatric acute liver failure (PALF) and children with aplastic anemia and acute hepatitis.1 This allows us to draw attention to our reported cohort of 38 children with a liver disease of indeterminate etiology, in mos t cases presenting with acute hepatitis (n = 14) or with PALF (n = 15).2 This condition was tentatively called “seronegative autoimmune hepatitis” because of the liver histology, the prompt response to immunosuppressive therapy used in typical autoimmune hepatitis3,4 (prednisone alo...
Source: The Journal of Pediatrics - Category: Pediatrics Authors: Tags: Letters to the Editor Source Type: research
We thank Maggiore et  al for their thoughtful commentary regarding our article discussing corticosteroid therapy for children with indeterminate acute hepatitis and acute liver failure. We have read with interest their article describing the spectrum of disorders seen in children with what they labeled “seronegative autoimmune hepatitis.”1 This descriptive term was used for convenience, and in their letter the authors state that the children with hepatitis, normal IgG levels, and blood cytopenia or aplastic anemia had features more consistent with immune activation and dysregulation.
Source: The Journal of Pediatrics - Category: Pediatrics Authors: Tags: Letter to the Editor Source Type: research
Acquired aplastic anemia (AAA) is a rare and potentially life threatening disorder. We retrospectively compared the outcomes of 29 children with AAA who received immunosuppressive therapy (IST) or underwent hematopoietic stem cell transplantation (HSCT). Median age at diagnosis was 9.0 years (range, 2 –18 years) and median follow-up period was 36 months (range, 3–108 months). Viral infection associated/post hepatitis AAA was in 6 patients (20.6%). According to the initial laboratory findings, 8 patients were classified as very severe AA (vSAA), 8 as severe AA (SAA), and 13 patients as transfu sion-dependent moderate AA (MAA).
Source: Transfusion and Apheresis Science - Category: Hematology Authors: Source Type: research
Abstract The two thrombopoietin receptor agonists (TPO-RAs), Eltrombopag and Romiplostim, were licensed in the United States for treatment of ITP in 2008 and progressively around the world in more than 100 countries. The six largest randomized controlled trials conducted in ITP have used one of these two agents. All studies have demonstrated a platelet response rate between 50-90%, depending upon the criteria used, with good safety and tolerability. As well, TPO-RAs were shown to be effective in reducing bleeding and need for concomitant and rescue medication. Many other explorations of mechanism of effect, prospe...
Source: Haematologica - Category: Hematology Authors: Tags: Haematologica Source Type: research
Qiancheng Deng1, Yangyang Luo1,2, Christopher Chang3, Haijing Wu1, Yan Ding4* and Rong Xiao1* 1Hunan Key Laboratory of Medical Epigenetics, Department of Dermatology, The Second Xiangya Hospital, Central South University, Changsha, China 2Department of Dermatology, Hunan Children's Hospital, Changsha, China 3Division of Rheumatology, Allergy and Clinical Immunology, University of California, Davis, Davis, CA, United States 4Department of Dermatology, Hainan Provincial Dermatology Disease Hospital, Haikou, China Autoimmune diseases are usually complex and multifactorial, characterized by aberrant produc...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Abstract Outcomes of allogeneic hematopoietic stem cell transplantation (HSCT) for hepatitis-associated aplastic anemia have not been fully evaluated. In the present study, the outcomes of 37 adult patients with hepatitis-associated aplastic anemia who underwent allogeneic HSCT were retrospectively analyzed using the registry database of Japan Society for Hematopoietic Cell Transplantation. The median age of the patients was 24 years (range, 16-61). The median period between diagnosis of hepatitis-associated aplastic anemia and HSCT was 6.0 months (range, 0.5-430.8). Stem cell sources were bone marrow (N...
Source: International Journal of Hematology - Category: Hematology Authors: Tags: Int J Hematol Source Type: research
We present 3 cases of hepatitis-associated aplastic anemia after hepatitis A virus infection. One of our cases is the first reported case of hepatitis-associated aplastic anemia after fulminant hepatitis A infection. Patient characteristics were consistent with older reports with regard to age and sex. All 3 patients were male individuals under the age of 20. In addition, all 3 patients had A+ blood group. Outcomes in our series were poor because of the unavailability of antithymocyte globulin and bone marrow transplantation.
Source: Journal of Pediatric Hematology Oncology - Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research
To examine the characteristics and outcomes of a multicenter patient cohort with indeterminate pediatric acute liver failure (IND-PALF) and with aplastic anemia with acute hepatitis treated with corticosteroids.
Source: The Journal of Pediatrics - Category: Pediatrics Authors: Tags: Original Articles Source Type: research
Conclusions: Pembrolizumab administered after ASCT in patients with R/R DLBCL is feasible with toxicity similar to its use in the R/R setting for other hematological malignancies. The high rate of neutropenia on this study, which is not a common AE of pembro in other settings, may be related to the burden of prior therapy or possibly to an accentuated toxicity of pembro in this specific patient population. The 18-month progression-free rate did not meet the protocol-specified primary objective, and therefore does not support a larger confirmatory study. Future studies in this setting should likely focus on specific subsets...
Source: Blood - Category: Hematology Authors: Tags: 723. Clinical Allogeneic and Autologous Transplantation: Late Complications and Approaches to Disease Recurrence: Therapy of Post-Transplantation Relapse Source Type: research
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