- Ominous comorbidities: Small ventricular septal defect and warm autoimmune hemolytic anemia. [Journal Article]
- APAnn Pediatr Cardiol 2018 Jan-Apr; 11(1):114-116
- Haploidentical HSCT for hemoglobinopathies: improved outcomes with TCRαβ+/CD19+-depleted grafts. [Journal Article]
- BABlood Adv 2018 Feb 13; 2(3):263-270
- We examined outcomes of haploidentical hematopoietic cell transplantation (haplo-HCT) using T-cell receptor αβ+(TCRαβ+)/CD19+-depleted grafts (TCR group, 14 patients) in children with hemoglobinopath...
We examined outcomes of haploidentical hematopoietic cell transplantation (haplo-HCT) using T-cell receptor αβ+(TCRαβ+)/CD19+-depleted grafts (TCR group, 14 patients) in children with hemoglobinopathies. Patients received a preparative regimen consisting of busulfan, thiotepa, cyclophosphamide, and antithymocyte globulin preceded by fludarabine, hydroxyurea, and azathioprine. The median follow-up among surviving patients was 3.9 years. The 5-year probabilities of overall survival (OS) and disease-free survival (DFS) were 84% and 69%, respectively. The incidence of graft failure was 14%. We compared outcomes to a historical group of 40 patients with hemoglobinopathies who received CD34+-selected grafts (CD34 group). The median follow-up of surviving patients for the CD34 group was 7.5 years. The 5-year probabilities of OS and DFS were 78% and 39%, respectively. The CD34 group had a significantly higher incidence of graft failure (45%) than the TCR group (14%) (P= .048). The incidences of grades 2 to 4 acute graft-versus-host disease (GVHD) in the TCR and CD34 groups were 28% and 29%, respectively, and 21% and 10% (P= .1), respectively, for extensive chronic GVHD. Viral reactivation was common in both groups. The overall incidence of posttransplant lymphoproliferative disorders for the entire group was 16%. Among all patients, 5 developed autoimmune hemolytic anemia or thrombocytopenia, with the overall cumulative incidence of 11%. The 2 groups showed suboptimal CD4+recovery within the first 6 months of transplantation with no significant difference between groups. These data demonstrate that TCRαβ+/CD19+-depleted grafts are associated with a reduced incidence of graft failure, but delayed immune reconstitution and associated morbidity and mortality remain a significant challenge.
- Emergence of anti-mitochondrial M2 antibody in patient with angioimmunoblastic T-cell lymphoma. [Journal Article]
- CJClin J Gastroenterol 2018 Feb 10
- 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 ...
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. Her serum levels of hepatobiliary enzymes normalized and AMA-M2 became negative after treatment. The unexpected positivity for AMA-M2 might have been caused by AITL cell-activated intrahepatic immune cells or the tumor cells themselves inflicting bile duct injury that mimicked primary biliary cholangitis. Alternatively, cross reactivity due to the overproduction of immunoglobulins may have caused this phenomenon. The present case may shed light on of the mechanisms of liver dysfunction accompanying AITL.
- IVIg for apparently autoimmune small-fiber polyneuropathy: first analysis of efficacy and safety. [Journal Article]
- TATher Adv Neurol Disord 2018; 11:1756285617744484
- CONCLUSIONS: These results provide Class IV, real-world, proof-of-concept evidence suggesting that IVIg is safe and effective for rigorously selected SFPN patients with apparent autoimmune causality. They provide rationale for prospective trials, inform trial design and indirectly support the discovery of small-fiber-targeting autoimmune/inflammatory illnesses.
- De Novo Allergy and Immune-Mediated Disorders Following Solid-Organ Transplantation-Prevalence, Natural History, and Risk Factors. [Journal Article]
- JPedJ Pediatr 2018 Jan 22
- CONCLUSIONS: Allergy and autoimmunity are common in pediatric liver, heart, and multivisceral transplant recipients and pose a significant health burden. Further studies are required to clarify the mechanisms behind this post-transplant immune dysregulation.
- Should Giant Cell Hepatitis With Autoimmune Hemolytic Anemia Be Considered a Pediatric Autoimmune Liver Disease? [Journal Article]
- JPJ Pediatr Gastroenterol Nutr 2018 Feb 01
- [Evans syndrome in infants]. [Journal Article]
- BMBol Med Hosp Infant Mex 2017 Mar - Apr; 74(2):141-146
- CONCLUSIONS: For the management of children with thrombocytopenia, the pediatrician must analyze for other cell lineage disorders. In the cases that we report here, we found the presence of autoimmune hemolytic anemia and monocytosis. Therefore, infectious and immunological studies must be included. The first-line treatment of choice are steroids, and intravenous immunoglobulin can be considered if severe immune thrombocytopenia is associated, as observed in these cases.
- [A monocentric retrospective study of low-dose rituximab in the treatment of 12 cases refractory or relapsed idiopathic autoimmune hemolytic anemia patients]. [Journal Article]
- ZXZhonghua Xue Ye Xue Za Zhi 2017 Dec 14; 38(12):1075-1077
- Simultaneous early-onset severe autoimmune hemolytic anemia and albuminuria during alemtuzumab treatment for multiple sclerosis. [Journal Article]
- MSMult Scler 2018 Jan 01; :1352458517743093
- CONCLUSIONS: Our report adds to literature on AIHA and nephropathy after alemtuzumab treatment and suggests to add Coombs' tests to the screening panel required for alemtuzumab treatment.
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- Life-threatening autoimmune warm hemolytic anemia following treatment for multiple sclerosis with alemtuzumab. [Journal Article]
- MSMult Scler 2018 Jan 01; :1352458517729766
- CONCLUSIONS: We report the third case of autoimmune hemolytic anemia following treatment with alemtuzumab in a young MS patient. Due to the severity of this adverse event, neurologists using this treatment should be alert.