Hematopoietic Stem Cell Transplantation in Primary Immunodeficiency Patients in the Black Sea Region of Turkey

Yıldıran, Alişan; Celiksoy, Mehmet Halil; Borte, Stephan; Güner, Şükrü Nail; Elli, Murat; Fışgın, Tunç; Ozyurek, Emel; Sancak, Recep; Oğur, Gönül

doi:10.4274/tjh.2016.0477

Cited by 2 publications

(2 citation statements)

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“…Besides, VOD, cGvHD, and autoimmune diseases were seen in 13.7%, 8.6%, and 5.1% respectively, and treated successfully except for one patient who died because of a severe interstitial lung disease due to chronic GvHD. These ratios were similar to those of reported children studies 7,8,15‐17 …”

Section: Discussionsupporting

confidence: 90%

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Primary immunodeficiencies: HSCT experiences of a single center in Turkey

Çipe

Aksoy

Aydoğdu

et al. 2021

Pediatric Transplantation

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Background Primary immunodeficiency diseases (PID) are characterized by the occurrence of frequent infections and are caused by many genetic defects. Hematopoietic stem cell transplantation (HSCT) is the only curative treatment option for the majority of PID. As a Pediatric Hematology‐Oncology‐Immunology Transplantation Unit, we wanted to present our HSCT experience regarding treatment of primary immunodeficiency diseases. Methods 58 patients were included in the study between January 2014 and June 2019. We searched 9/10 or 10/10 matched‐related donor (MRD) firstly, in the absence of fully matched‐related donor. We screened matched unrelated donor (MUD) from donor banks. MRD was used in 24 (41.3%) patients, MUD in 20 (34.4%) patients, and haploidentical donors in 14 (24.1%) patients. Demographic data, HSCT characteristics, and outcome were evaluated. While 16 patients had severe combined immunodeficiency (SCID), the remaining was non‐SCID. Results Of the 58 patients, 38 were male and 20 were female. Median age at transplantation was 12 months (range: 2.5–172 months). Combined immunodeficiencies consisted 67.2% of patients. Mean follow‐up time was 27 months (6 months–5 years). Median neutrophil, lymphocyte, and thrombocyte engraftment days were similar in comparison of both donor type and stem cell source. The most common complication was acute GvHD in 15 (25.8%) patients. In total, five patients (31%) belonging to the SCID group and 10 patients (23.8%) belonging to the non‐SCID group died. Our total mortality rate was 15 (25.8%) in all patients. Conclusions We would like to present our HSCT experiences as a pediatric immunology transplantation center. Existing severe infections before transplantation period, BCGitis, and CMV are important issues of transplantation in Turkey. However, the follow‐up time is shorter than some studies, our results regarding complications and survival are similar to previous reports.

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Section: Discussionsupporting

confidence: 90%

“…These ratios were similar to those of reported children studies. transplantation was 50% of SCID patients which was slightly higher than other data collected in Turkey, 8,17 but it was of a value near to other reported studies. 18,19 After transplantation, BCGitis can also be accepted as the first sign of lymphocyte engraftment.…”

Section: Discussioncontrasting

confidence: 47%