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Öğe Epstein?Barr virus?related lymphoproliferative disorders in T?cell repleted haploidentical transplantation with post?transplant cyclophosphamide(Springer Link, 2021) Uygun, Vedat; Özsan, Nazan; Daloğlu, Hayriye; Öztürkmen, Seda; Yalçın, Koray; Karasu, Gülsün; Yeşilipek, AkifEBV-associated lymphoproliferative disorders (LPDs) are common in hematopoietic stem cell transplantation (HSCT) with T-cell-depleted grafts, but are extremely rare in HSCT patients with T-cell-replete grafts with post-transplant cyclophosphamide (PTCy). Here we present the cases of two pediatric patients who developed EBV-related LPD after T-cell-replete haplo-HSCT with PTCy. One of these is the first reported case of EBV-positive mucocutaneous ulcer (EBVMCU) developing after PTCy. EBV-related diseases are rare in T-cell-replete haplo-HSCT patients with PTCy. However, in patients with risk factors, it is reasonable to screen for EBV viremia for LPD.Öğe Hematopoetic stem cell transplantation in CD40 ligand deficiency(SPRINGERNATURE, 2020) Uygun, Vedat; Uygun, Dilara Fatma Kocacık; Daloğlu, Hayriye; Öztürkmen, Seda; Kılıç, Suar Çakı; Bingöl, Aysen; Yalçın, Koray; Çelen, Safiye Suna; Hazar, Volkan; Tezcan Karasu, Gülsun; Yeşilipek, Akif[No Abstract Available]Öğe Hematopoietic stem cell transplantation in CD40 ligand deficiency: a single-center experience(Wiley, 2020) Uygun, Dilara Fatma Kocacık; Uygun, Vedat; Tezcan Karasu, Gülsun; Daloğlu, Hayriye; Öztürkmen, Seda Irmak; Çelmeli, Fatih; Torun, Selda Hançerli; Özen, Ahmet; Barış, Safa; Aydıner, Elif Karakoç; Yalçın, Koray; Kılıç, Suar Çaki; Hazar, Volkan; Bingöl, Aysen; Yeşilipek, AkifDeficiency of the CD40L, expressed on the surface of T lymphocytes, is caused by mutations in the glycoproteinCD40L (CD154)gene. Resulting defective humoral and cellular responses cause a clinical presentation that includes recurrent sinopulmonary bacterial infections, opportunistic infections, sclerosing cholangitis, neutropenia, and autoimmune manifestations. HSCT represents the only curative treatment modality. However, the therapeutic decision to use HSCT proves challenging in many cases, mainly due to the lack of a phenotype-genotype correlation. We retrospectively reviewed patients with CD40L deficiency who were transplanted in Antalya and Goztepe MedicalPark Pediatric HSCT units from 2014 to 2019 and followed by Akdeniz University School of Medicine Department of Pediatric Immunology. The records of eight male cases, including one set of twins, were evaluated retrospectively. As two transplants each were performed on the twins, a total of ten transplants were evaluated. Conditioning regimens were predominantly based on myeloablative protocols, except for the twins, who received a non-myeloablative regimen for their first transplantation. Median neutrophil and platelet engraftment days were 13 (range 10-19) and 14 (range 10-42) days, respectively. In seven of ten transplants, a CMV reactivation was developed without morbidity. None of the patients developed GVHD, except for one mild case of acute GVHD. All patients survived, and the median follow-up was 852 days. Our data show that HSCT for patients with CD40 ligand deficiency is a potentially effective treatment for long-term disease control.Öğe Hematopoietic stem cell transplantation in serine/threonine kinase 4 (stk4) deficiency: report of two cases and literature review(WILEY, 2022) Uygun, Vedat; Keleş, Sevgi; Daloğlu, Hayriye; Öztürkmen, Seda; Yalçın, Koray; Tezcan Karasu, Gülsün; Yeşilipek, AkifBackground Serine/threonine kinase 4 (STK4) deficiency is a combined immunodeficiency (CID) characterized by early onset recurrent bacterial, viral, and fungal infections. Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative therapy for CID; however, little is known about the necessity and benefits of HSCT in patients with STK4 deficiency. Methods We report two siblings with STK4 deficiency transplanted from two unrelated donors with the same conditioning regimen. Results In the conditioning regimen, rituximab was given on Day -11 (375 mg/m(2)), and sirolimus was added on the same day. Busulfan was administered at a myeloablative dose (3.2 mg/kg; Days -7 to -4) with 150 mg/m(2) of fludarabine (Days -7 to -3). They were transplanted with peripheral blood stem cells, and graft-versus-host disease (GVHD) prophylaxis was administered with 10 mg/m(2) methotrexate on Days 1, 3, and 6. In addition, mycophenolate mofetil (MMF) was started on Day 1 with ongoing use of sirolimus. We did not encounter veno-occlusive disease (VOD), high-grade acute GVHD, or significant organ toxicity in either patient. Both patients were well at the end of the first year after HSCT with complete donor chimerism. Conclusions Serine/threonine kinase 4 deficiency is a disease with high mortality post-HSCT; therefore, the conditioning regimen and GVHD prophylaxis strategies are important considerations in these patients. In our opinion, the conditioning regimen, which includes rituximab and busulfan and fludarabine (BU-FLU), GVHD prophylaxis with sirolimus and MMF, and short-term methotrexate, offers favorable outcomes and is well tolerated in our STK4-deficient patients.Öğe Pearson syndrome in a child transplanted for diamond-blackfan anemia(Sociedad Argentina de Pediatria, 2021) Uygun, Vedat; Daloğlu, Hayriye; Öztürkmen, Seda; Karasu, Gülsün; Yeşilipek, AkifPearson syndrome (PS), shares a number of overlapping features with Diamond-Blackfan anemia (DBA), including early onset of severe anemia, making differential diagnosis important. Differential diagnosis of DBA and PS is critical, since those with DBA may respond to treatment with steroids, may undergo remission, or may benefit from hematopoietic stem cell transplantation (HSCT). However, patients with PS have a different prognosis, with a very high risk of developing acidosis, metabolic problems, and pancreatic dysfunction, and a shorter life expectancy than those with DBA. Here we present a patient who underwent HSCT for DBA but was subsequently diagnosed with PS after developing some complications.Öğe Pearson syndrome in a patient transplanted for diamond-blackfan anemia(SPRINGERNATURE, 2020) Tezcan Karasu, Gülsün; Uygun, Vedat; Daloğlu, Hayriye; Öztürkmen, Seda; Yeşilipek, Akif[No Abstract Available]Öğe Pre-transplantation vitamin D deficiency increases acute graft-versus-host disease after hematopoietic stem cell transplantation in thalassemia major patients(WILEY, 2022) Daloğlu, Hayriye; Uygun, Vedat; Öztürkmen, Seda; Yalçın, Koray; Karasu, Gülsü; Yeşilipek, AkifBackground: Although there are many studies on the role of vitamin D deficiency (VDD) in hematopoetic stem cell transplantation (HSCT), outcomes have often reported conflicting results because of the heterogeneity of the patients in the studies. Methods: We investigated the association between VDD prior to HSCT and outcomes after HSCT in a relatively homogenous group of patients with thalassemia major (TM) who received identical treatment for TM before transplantation, and the same conditioning regimen and GVHD prophylaxis during and after transplantation. All patients, including the patients with normal vitamin D3 levels received 400 to 800 IU per day of vitamin D for the first 6 months after HSCT. Results: Pre-HSCT VDD increased the frequency of aGVHD after transplantation, particularly in HSCTs performed with PBSC for the stem cell source. Pre-transplant low vitamin D3 levels had no association with transplant outcomes such as engraftment, viral infections, alloimmunization, chronic GvHD, total days of hospitalization, and success in terms of transfusion independence. Conclusions: Low vitamin D3 levels before HSCT carry a significant risk for aGVHD. All patients with TM should be screened for VDD before HSCT, and every effort should be made to supplement vitamin D before the transplant in VDD patientsÖğe Ruxolitinib salvage therapy is effective for steroid-refractory graft-versus-host disease in children: a single-center experience(Wiley, 2020) Uygun, Vedat; Tezcan Karasu, Gülsün; Daloğlu, Hayriye; Öztürkmen, Seda; Kılıç, Suar Çakı; Yalçın, Koray; Çelen, Safiye Suna; Hazar, Volkan; Yeşilipek, AkifBackground Despite the increasing performance of allogeneic hematopoietic cell transplantation over the last decades, graft-versus-host disease (GVHD) remains the main cause of morbidity and mortality. The efficacy of ruxolitinib against GVHD has been demonstrated in adult studies; however, very few studies have been conducted in children. Procedure This study aimed to evaluate the efficacy of ruxolitinib in 29 children with steroid-refractory acute or chronic GVHD. Twenty-five (87%) patients received at least three different immune modulator agents, including methylprednisolone, before initiating ruxolitinib. Results All grade 2 acute GVHD patients completely responded to ruxolitinib treatment; 82% of high-grade (3-4) acute GVHD patients and 80% of chronic GVHD (moderate-severe) patients had at least a partial response. Of seven patients with bronchiolitis obliterans, five had a partial response after ruxolitinib. Of 29 patients, 22 were administered steroids at any time in the first month of acute GVHD or the first three months of chronic GVHD during ruxolitinib usage, which was significantly tapered by the end of the observation period. Conclusion Steroid-refractory acute and chronic pediatric GVHD patients treated with ruxolitinib had a high overall response rate, with the additional benefit of steroid sparing.Öğe Talasemi Majör Hastalarında Hematopoetik Kök Hücre Nakli Uzun Dönem Sağkalım Sonuçları(2023) Daloğlu, Hayriye; Uygun, Vedat; Öztürkmen, Seda; Yalçın, Koray; Çelen, Suna; Karasu, Gülsün; Yeşilipek, AkifAmaç: Günümüzde talasemi majör (TM) hastalarında en önemli sorun transfüzyonların neden olduğu demir yükü ve bunun doğurduğu komplikasyonlardır. Bu durumun günümüzde kabul edilen tek küratif tedavi seçeneği hematopoetik kök hücre nakli (HKHN)’dir. Çalışmamızda, TM nedeniyle akraba ve akraba dışı donörden merkezimizde HKHN uygulanmış hastalarımızın uzun dönem sağkalım sonuçları ve kronik graft versus host hastalığı (kGVHH) değerlendirilmiştir. Hastalar ve Yöntem: TM tanısı ile HKHN uygulanmış 378 hastanın klinik verileri retrospektif olarak incelendi. Tüm hastalara busulfan içeren myeloablatif hazırlama rejimi ve ATG uygulandı. Nakil öncesi ve sonrası nakil ilişkili veriler değerlendirildi ve tüm sağkalım (OS), talasemisiz sağkalım (TS), talasemi ve kGVHH´siz sağkalım (TGS) belirlendi. Bulgular: Ortanca 79 ay izlenen hastaların ikisinde primer greft rejeksiyonu, 22´sinde sekonder greft rejeksiyonu gelişti. Son kontrol tarihlerine göre, hastaların %92.9´unun ya tam kimerik ya da transfüzyon ihtiyacı olmadan stabil miksed kimerizm ile izlendiği saptandı. HKHN sonrası 15 hasta ölürken, sağ kalan 363 hastanın 25’inde kGVHH bulguları gelişti ancak 17´si izlemde düzeldi. Yedi yıllık OS, TS ve TGS sırası ile %95.9, %89.5 ve %87.1 olarak saptandı. Sonuç: Talasemi majör hastalarında, tam uyumlu donör varlığında nakil sonrası yaşam kalitesi de göz önünde bulundurularak organ hasarı gelişmeden, mümkün olan en erken dönemde HKHN uygulanmalıdır.Öğe Timing of initiation of calcineurin inhibitors in pediatric haploidentical transplantation with post-transplantation cyclophosphamide: Effects on survival, relapse, and cytokine release syndrome(Karger, 2022) Uygun, Vedat; Karasu, Gülsüm; Yalçın, Koray; Öztürkmen, Seda; Daloğlu, Hayriye; Çelen, Safiye Suna; Hazar, Volkan; Yeşilipek, AkifBackground: The use of unmanipulated haploidentical hematopoietic stem cell transplantations (haplo-HSCT) with post-transplant cyclophosphamide (PTCY) in children has emerged as an acceptable alternative to the patients without a matched donor. However, the timing of calcineurin inhibitors (CNIs) used in combination with PTCY is increasingly becoming a topic of controversy. Method: We evaluated 49 children with acute leukemia who underwent unmanipulated haplo-HSCT with PTCY according to the initiation day of CNIs (pre- or post-cyclophosphamide [CY]). Results: There were no significant differences in the overall survival analysis between the 2 groups. The cumulative incidence of relapse at 2 years was 21.2% in the pre-CY group and 38.9% in the post-CY group (p = 0.33). Cytokine release syndrome (CRS) was observed more frequently in the post-CY group (p = 0.04). The overall survival and event-free survival at 2 years in patients with and without CRS in the pre-CY group were 42.9% versus 87.5% (p = 0.04) and 38.1% versus 87.5% (p = 0.04), respectively. Conclusion: Our study shows that the argument for starting CNI administration after CY is tenuous, and the rationale for not starting CNIs before CY needs to be reconsidered.Öğe Use of low cell dose for unmanipulated donor lymphocyte for management of cytomegalovirus infection: a single-center experience(2020) Uygun, Vedat; Tezcan Karasu, Gülsün; Daloğlu, Hayriye; Öztürkmen, Seda; Yalçın, Koray; Çelen, Safiye Suna; Yeşilipek, AkifAlthough advancements have been made in monitoring and preventing viral infections in HSCT patients, CMV reactivation still remains a critical post-transplant complication. Adoptive cell therapy is an alternative to pharmacotherapy of CMV infection in refractory patients. We retrospectively reviewed CMV infection cases after allogeneic HSCT who received U-DLI as treatment. In total, five pediatric patients between the ages of 0.5-16 years that received U-DLI for a post-HSCT CMV infection were evaluated. The dose of CD3+ lymphocytes administered in DLI was 5 × 104 /kg, except in one patient transplanted from his sibling. One patient, who was transplanted from an unrelated donor, received U-DLI from his haploidentical mother. CMV titers dramatically reduced after U-DLI. If the availability of CMV-specific CTL is an issue, we propose that one should consider using the U-DLI therapy with low cell dose from a seropositive donor. In case the stem cell donor is seronegative and a seropositive donor is unavailable, using the U-DLI therapy from seropositive, haploidentical donors is a promising way of treatment. More studies need to be conducted to further confirm the safety and efficacy of this treatment procedure.
