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4. An International Survey of Allogeneic Hematopoietic Cell Transplantation for X-Linked Agammaglobulinemia

Author

Nishimura, Akira, Uppuluri, Ramya, Raj, Revathi, Swaminathan, Venkateswaran Vellaichamy, Cheng, Yifei, Abu-Arja, Rolla F., Fu, Bin, Laberko, Alexandra, Albert, Michael H., Hauck, Fabian, Bucciol, Giorgia, Bigley, Venetia, Elcombe, Suzanne, Kharya, Gaurav, Pronk, Cornelis Jan H, Wehr, Claudia, Neven, Bénédicte, Warnatz, Klaus, Meyts, Isabelle, Morio, Tomohiro, Gennery, Andrew R., and Kanegane, Hirokazu

Published
2023
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7. Treatment free remission in pediatric chronic myeloid leukemia

Author

Meena Satishkumar, Vellaichamy Swaminathan Venkateswaran, Ganesan Kavitha, Duraisamy Suresh, Chakraborty Sohini, Uppuluri Ramya, and Raj Revathi

Subjects
CML, Treatment free remission, Imatinib, Children, Long-term outcomes, Pediatrics, RJ1-570
Abstract

Background: The aim was to evaluate the impact of discontinuation of imatinib in children in chronic phase of chronic myeloid leukemia (CML) and assess treatment free remission (TFR). Patients and methods: We performed a retrospective study in children diagnosed with CML chronic phase up to 18 years of age, who were on imatinib for 5 years and had consistent BCR:ABL1 negative/undetectable transcripts through RT-PCR. Complete blood counts were monitored monthly and BCR:ABL1 transcripts by RT-PCR monitored 3 months. Results: Among the 14 patients who continued to remain PCR negative, we attempted discontinuing imatinib in eleven children as per the above-mentioned criteria. Eight (72%) continued to be in remission and did not require imatinib to be restarted. Among the remaining three patients, one child was noted to have increasing basophilia in the differential counts and was hence restarted on imatinib. Two other patients demonstrated recurrence of BCR:ABL1 transcript copy numbers six months after stopping TKI, following which imatinib was restarted. All three patients had documented major molecular response within three months of therapy. The median duration of TFR was 18 months in our cohort (range: 3 months–5 years). Among patients who continued to be off imatinib, TKI withdrawal symptoms were documented in 4 (36%) patients. Conclusions: Treatment free remission was achieved in 8/11 (72%) children after discontinuation of imatinib, and re-introduction of imatinib resulted in major molecular response in those with recurrence. Monitoring of BCR:ABL1 transcripts through RT PCR is essential.

Published
2023
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10. Outcomes of COVID-19 in children with cancer – Report from the Indian Pediatric Oncology Group (InPOG) COVID-19 registry in India

Author

Raj, Revathi, Uppuluri, Ramya, Parambil, Badira, Dhariwal, Nidhi, Totadri, Sidharth, Mathew, Leni Grace, Radhakrishnan, Venkatraman, Charan, Abhishek, Yadav, Satya Prakash, Mahajan, Amita, Mudaliar, Sangeeta, Sharma, Ratna, Manglani, Mamta, Kharya, Gaurav, Verma, Nishant, Arora, Ramandeep, Ghara, Niharendu, KS, Reghu, Radhakrishnan, Nita, Iyer, Nisha, Mehta, Priti, Singh, Amitabh, Patel, Shivani, John, Joseph, Bhat, Sunil, Badiger, Shobha, Siddaiahgari, Sirisharani, Kakkar, Shruti, Scott, Julius, Jayaraman, Dhaarani, Srinivasan, Arathi, Krishnan, Yamini, Sonamani, Ngangbam, Misra, Ruchira, Bafna, Vibha, Seth, Rachna, Kutty, Purvi Kadakia, Kanakia, Swati, Rao, Vasudha, Prakash, Anand, Bharadwaj, Vandana, and Chinnasamy, Girish

Published
2022
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13. Clinical, immunological and genomic characteristics of children with X-linked agammaglobulinemia from Kerala, South India

Author

Madathil Govindaraj, Geeta, Jain, Abhinav, Edavazhippurath, Athulya, Bhoyar, Rahul C., Dhanasooraj, Dhananjayan, Mishra, Anushree, Gupta, Vishu, Nair, Mohandas, Shiny, P.M., Uppuluri, Ramya, Kumar, Anoop, Kashyap, Atul, Ajith Kumar, V.T., Shankaran, Gireesh, Senthivel, Vigneshwar, Imran, Mohamed, Kumar Divakar, Mohit, Sawant, Sneha, Dalvi, Aparna, Madkaikar, Manisha, Raj, Revathi, Sivasubbu, Sridhar, and Scaria, Vinod

Published
2022
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20. Clinical, immunological, and molecular findings in two patients with MHC class I deficiency and post‐transplant outcome.

Author

Ramalingam, Thulasi Raman, Vaidhyanathan, Lakshman, NK, Harsha Rasheed, Uppuluri, Ramya, and Raj, Revathi

Subjects
KILLER cells, CYTOTOXIC T cells, SEVERE combined immunodeficiency, GRANULOMATOSIS with polyangiitis, WHEEZE
Abstract

This article explores the clinical, immunological, and molecular aspects of MHC class I deficiency, a rare immunodeficiency disorder characterized by the absence or reduced expression of MHC class I molecules. These molecules play a crucial role in presenting peptides from intracellular pathogens to CD8+ T cells, which eliminate infected cells. The article presents two cases of children with MHC class I deficiency who underwent hematopoietic stem cell transplantation (HSCT) and discusses their outcomes after the transplant. MHC class I deficiency can lead to susceptibility to infections and malignancies, and its symptoms can vary from mild to severe. The role of transplantation in treating this condition is not well understood, as immune reconstitution is limited due to poor thymic function. Further research is necessary to gain a better understanding of MHC class I deficiency and its treatment options. [Extracted from the article]

Published
2024
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24. X-Linked Lymphoproliferative Syndrome: A Spectrum of Clinical and Immunological Profile and Novel Pathogenic Variants from Chandigarh, India

Author

Jindal, Ankur Kumar, primary, Mondal, Sanjib, additional, Sil, Archan, additional, Rawat, Amit, additional, Chawla, Sanchi, additional, Tyagi, Rahul, additional, Sudhakar, Murugan, additional, Banday, Aaqib Zaffar, additional, Suri, Deepti, additional, Vignesh, Pandiarajan, additional, Dhaliwal, Manpreet, additional, Sharma, Saniya, additional, Rikhi, Rashmi, additional, Saka, Ruchi, additional, Sharma, Rajni, additional, Chatterjee, Debajyoti, additional, Sreedharanunni, Sreejesh, additional, Uppuluri, Ramya, additional, Raj, Revathi, additional, and Singh, Surjit, additional

Published
2024
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25. Diving deep for the needle in the haystack: An outbreak investigation of Burkholderia cenocepacia bacteremia

Author

Ghafur, Abdul, primary, Balaguru, Praveen, additional, Ramanan, S. G., additional, Rajendranath, Rejiv, additional, Raj, Revathi, additional, Zehran, Mohammed, additional, Raja, T., additional, Easow, Jose M., additional, Srinivasan, Sankar, additional, Devaprasad, Dedeepiya, additional, Sukumar, Thilakavathy, additional, Nilgar, Praveen, additional, Sakthivel, K., additional, Nirumal, Rakkesh M., additional, Chandra, K., additional, Lakshmi, Sree V., additional, Sethuraman, Nandini, additional, Devi Bakthavatchalam, Yamuna, additional, and Veeraraghavan, Balaji, additional

Published
2024
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28. Impact of Conditioning Regimen and Graft-versus-Host Disease Prophylaxis on The Outcome of Haploidentical Peripheral Blood Stem Cell Transplantation for High-Risk Severe Aplastic Anemia in Children and Young Adults: A Report from the Pediatric Severe Aplastic Anemia Consortium of India

Author

Kharya, Gaurav, Jaiswal, Sarita R., Bhat, Sunil, Raj, Revathi, Yadav, Satya P., Dua, Vikas, Sen, Santanu, Bakane, Atish, Badiger, Shobha, Uppuluri, Ramya, Rastogi, Neha, Sachdev, Mansi, Sharma, Bharti, Saifullah, Ashraf, and Chakrabarti, Suparno

Published
2023
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30. Outcome of allogeneic stem cell transplant for Fanconi anemia in India

Author

Yadav, Satya Prakash, primary, Raj, Revathi, additional, Uppuluri, Ramya, additional, Choudhary, Dharma, additional, Doval, Divya, additional, Dua, Vikas, additional, Bhat, Sunil, additional, Kharya, Gaurav, additional, Patil, Rajesh, additional, Bansal, Shweta, additional, M, Deendayalan, additional, Mehdi, Intezar, additional, Mathews, Vikram, additional, Abraham, Aby, additional, and George, Biju, additional

Published
2023
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45. Cytomegalovirus Reactivation as a Risk Factor for All-Cause Mortality in Children Undergoing Hematopoietic Stem Cell Transplantation: Experience Over Two Decades from a Tertiary Referral Center in India

Author

Chakraborty, Sohini, primary, Swaminathan, Venkateswaran Vellaichamy, additional, Ganesan, Kavitha, additional, Duraisamy, Suresh, additional, Meena, Satishkumar, additional, Jayakumar, Indira, additional, Krishna, Vidya, additional, Uppuluri, Ramya, additional, and Raj, Revathi, additional

Published
2023
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47. Outcome of allogeneic stem cell transplant for Fanconi anemia in India.

Author

Yadav, Satya Prakash, Raj, Revathi, Uppuluri, Ramya, Choudhary, Dharma, Doval, Divya, Dua, Vikas, Bhat, Sunil, Kharya, Gaurav, Patil, Rajesh, Bansal, Shweta, M, Deendayalan, Mehdi, Intezar, Mathews, Vikram, Abraham, Aby, and George, Biju

Subjects
*FANCONI'S anemia, *STEM cell transplantation, *HEMATOPOIETIC stem cell transplantation, *BONE marrow transplantation
Abstract

This letter to the editor discusses the outcome of allogeneic stem cell transplant (HSCT) for Fanconi Anemia (FA) in India. FA is a rare genetic disease that can lead to bone marrow failure, myelodysplastic syndrome (MDS), acute myeloid leukemia (AML), and head and neck malignancies. The study analyzed data from 190 patients who underwent HSCT for FA in India from 1999 to 2019. The results showed that the overall survival rate was 64%, and the event-free survival rate was 62.5%. The study also found that matched related donor transplants had the highest survival rate, and chronic graft-versus-host disease (GVHD) was associated with a higher risk of second malignancy. The authors suggest considering T-cell depletion and the use of bone marrow grafts to reduce the risk of chronic GVHD and associated malignancies. [Extracted from the article]

Published
2024
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48. A rare cause of rectovaginal fistula in early infancy: It is in the genes!

Author

Swaminathan, Aravind, Sathiyasekaran, Malathi, Padankatti, Swathi, Padankatti, Rajiv, Arulprakash, S., and Raj, Revathi

Subjects
BCG vaccines -- Genetic aspects, Genetic research -- Genetic aspects, BCG -- Genetic aspects, Genes -- Genetic aspects, Fistula -- Diagnosis -- Genetic aspects -- Care and treatment, Tazobactam, Colitis -- Diagnosis -- Care and treatment -- Genetic aspects, Hematopoietic stem cells -- Transplantation, Interleukins, Gastrointestinal diseases -- Diagnosis -- Care and treatment -- Genetic aspects, Health
Abstract

Byline: Aravind. Swaminathan, Malathi. Sathiyasekaran, Swathi. Padankatti, Rajiv. Padankatti, S. Arulprakash, Revathi. Raj Acquired Rectovaginal Fistula (RVF) is rare in infants. Interleukin10/ Interleukin 10 receptor deficiencies are monogenic disorders presenting [...]

Published
2021

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