Publications by authors named "Deepika Bhatla"
Article Synopsis
- Rheumatologic disease-associated hemophagocytic lymphohistiocytosis (HLH) is a rare and serious condition that may arise from underlying autoimmune diseases; real-world data on the use of the monoclonal antibody emapalumab for treatment is limited.
- A study called REAL-HLH reviewed medical records from 33 hospitals to evaluate the use and outcomes of emapalumab in patients with rheumatologic disease-associated HLH, finding that 14.3% of 105 patients studied had this subtype.
- Results showed that emapalumab treatment led to improvements in key laboratory parameters, significantly reduced glucocorticoid use, and had a high survival rate, indicating its potential effectiveness in managing
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening, hyperinflammatory syndrome. Emapalumab, a fully human monoclonal antibody that neutralizes the proinflammatory cytokine interferon gamma, is approved in the United States to treat primary HLH (pHLH) in patients with refractory, recurrent, or progressive disease, or intolerance with conventional HLH treatments. REAL-HLH, a retrospective study, conducted across 33 US hospitals, evaluated real-world treatment patterns and outcomes in patients treated with ≥1 dose of emapalumab between 20 November 2018 and 31 October 2021.
View Article and Find Full Text PDFBackground: Various processing methodologies are routinely used to reduce volume and red blood cell content of umbilical cord blood (UCB) units collected for hematopoietic stem cell transplantation. There is limited information regarding effects of UCB processing techniques on clinical outcomes.
Study Design And Methods: Retrospective data analysis compared laboratory and clinical outcomes following single-unit UCB transplantation performed between 1999 and 2015.
Article Synopsis
- Transplant-associated thrombotic microangiopathy (TA-TMA) is a serious complication that occurs in patients undergoing hematopoietic stem cell transplantation (HSCT), with a much higher incidence rate found in a single-center study compared to older studies that did not use systematic screening.
- In a multicenter study involving 614 pediatric patients, TA-TMA was found in 16% of cases diagnosed within a median of 22 days after transplant, affecting their health outcomes significantly.
- Results showed that patients with TA-TMA had higher rates of infections and longer hospital stays, leading to a lower overall survival rate (78%) compared to those without TA-TMA (93%), highlighting the importance of systematic screening for better patient management
Article Synopsis
- This study focuses on a newly identified autoinflammatory disease caused by mutations in a tRNA processing enzyme, particularly examining its clinical features, immune responses, and molecular mechanisms.
- In nine patients diagnosed with congenital sideroblastic anemia, significant genetic mutations were found, along with elevated inflammatory cytokines and notable cellular abnormalities.
- Treatment with a TNF inhibitor successfully reduced inflammation and improved patient outcomes, highlighting the importance of early molecular diagnosis for effective intervention.
Outcomes for Umbilical Cord Blood Transplantation in Severe Combined Immunodeficiency Disorders: Ten-Year Experience.
Pediatr Allergy Immunol Pulmonol
September 2017
The treatment of severe combined immunodeficiency (SCID) is immune reconstitution using hematopoietic stem cell (HSC) transplantation early in life. HLA-identical related donors are the preferred source of HSCs. Since sibling donors are available in <30% of patients, other sources of HSCs are considered-mismatched related donor, umbilical cord blood (UCB), and matched unrelated donor bone marrow.
View Article and Find Full Text PDFBackground: Umbilical cord blood (UCB) is an important source of hematopoietic stem cells for transplantation especially in minority populations with limited chances of finding a histocompatible volunteer donor in the registry. UCB has the advantages of early availability, successful outcomes despite some histocompatibility mismatch, and low incidence of chronic graft-versus-host disease. Public cord blood banks that disseminate UCB products for transplant depend on voluntary donation at participating hospitals and obstetrical providers for collection.
View Article and Find Full Text PDFEffect of cord blood processing on transplantation outcomes after single myeloablative umbilical cord blood transplantation.
Biol Blood Marrow Transplant
April 2015
Variations in cord blood manufacturing and administration are common, and the optimal practice is not known. We compared processing and banking practices at 16 public cord blood banks (CBB) in the United States and assessed transplantation outcomes on 530 single umbilical cord blood (UCB) myeloablative transplantations for hematologic malignancies facilitated by these banks. UCB banking practices were separated into 3 mutually exclusive groups based on whether processing was automated or manual, units were plasma and red blood cell reduced, or buffy coat production method or plasma reduced.
View Article and Find Full Text PDFThe relationship of race/ethnicity with outcomes of umbilical cord blood transplantation (UCBT) is not well known. We analyzed the association between race/ethnicity and outcomes of unrelated single UCBT for leukemia and myelodysplastic syndromes. Our retrospective cohort study consisted of 885 adults and children (612 whites, 145 blacks, and 128 Hispanics) who received unrelated single UCBT for leukemia and myelodysplastic syndromes between 1995 and 2006 and were reported to the Center for International Blood and Marrow Transplant Research.
View Article and Find Full Text PDFPediatric dosing of rituximab revisited: serum concentrations in opsoclonus-myoclonus syndrome.
J Pediatr Hematol Oncol
July 2010
Article Synopsis
- Rituximab was administered intravenously to 25 children with opsoclonus-myoclonus syndrome across four weeks, with varying levels of standard immunotherapy among groups.
- Serum levels of rituximab showed distinct increase and decrease patterns with each treatment, revealing significant differences in concentration between younger and older children after the treatment series.
- The findings suggest that serum IgM depletion is an important measure for determining appropriate rituximab dosing in pediatric patients and emphasize that dosing should be calculated based on body weight rather than body surface area.
Background: The variant polymorphism in the gene MDM2, SNP309, leads to increased level of mdm2 protein and subsequent downregulation of p53 tumor suppressor pathway. Presence of this single nucleotide polymorphism (SNP) has been associated with earlier tumorigenesis in patients with Li-Fraumeni syndrome, as well as decreased survival in patients with CLL. In addition, cells homozygous (G/G) for SNP 309 were found to have 10-fold increase resistance to topoisomerase II inhibitors in vitro.
View Article and Find Full Text PDFArticle Synopsis
- Cytosine arabinoside (ara-C) is a cancer drug that becomes non-toxic when converted by the enzyme cytidine deaminase (CDA), which is influenced by genetic variations.
- A common genetic variant (A79C) reduces the activity of CDA, leading to higher treatment-related mortality rates in children with acute myeloid leukaemia (AML) receiving certain therapies, specifically those involving ara-C.
- The study found that children with the lower activity genotype (CC) faced a higher risk of mortality after treatment compared to those with higher activity genotypes (AA and AC), especially with the IDA-FLAG treatment regimen.