ABSTRACT
PURPOSE: To describe the experience of performing ovarian tissue cryopreservation (OTC) before hematopoietic stem cell transplantation (HSCT), among girls/women with severe sickle cell disease (SCD)(SS or S/ß0-thalassemia) who are, besides the usual surgical risk, at risk of SCD-related complications during the fertility preservation procedure for improving their counseling and management. METHODS: This retrospective study included 75 patients (girls/women) with SCD who have had OTC before myeloablative conditioning regimen (MAC) for HSCT. Characteristics of patients and data on OTC, ovarian status follow-up, and results of ovarian tissue transplantation (OTT) were collected in medical records. RESULTS: At OTC, the median (IQR 25-75; range) age of the patients was 9.6 (6.9-14.1; 3.6-28.3) years, 56/75 were prepubertal, and no SCD or surgery-related complications occurred. The median follow-up post-HSCT was > 9 years. At the last follow-up, among prepubertal patients at HSCT, 26/56 were ≥ 15 years old and presented with a premature ovarian insufficiency (POI), except 2, including the patient who had received an OTT to induce puberty. Eight were 13-15 years old and presented for POI. The remaining 22 patients were under 13. Among the 19 patients who were menarche at HSCT, 2 died 6 months post-HSCT and we do not have ovarian function follow-up for the other 2 patients. All the remaining patients (n = 15) had POI. Five patients had OTT. All had a return of ovarian function. One patient gave birth to a healthy baby. CONCLUSION: OTC is a safe fertility preservation technique and could be offered before MAC independent of the patient's age.
Subject(s)
Anemia, Sickle Cell , Cryopreservation , Fertility Preservation , Hematopoietic Stem Cell Transplantation , Ovary , Primary Ovarian Insufficiency , Humans , Female , Fertility Preservation/methods , Hematopoietic Stem Cell Transplantation/methods , Hematopoietic Stem Cell Transplantation/adverse effects , Cryopreservation/methods , Anemia, Sickle Cell/therapy , Ovary/transplantation , Child , Adolescent , Adult , Follow-Up Studies , Young Adult , Child, Preschool , Retrospective Studies , Transplantation Conditioning/methods , Transplantation Conditioning/adverse effects , PregnancySubject(s)
Deferasirox , Iron Chelating Agents , Registries , Thalassemia , Humans , Deferasirox/therapeutic use , Deferasirox/administration & dosage , Thalassemia/therapy , Male , Female , Iron Chelating Agents/therapeutic use , Iron Chelating Agents/administration & dosage , Adolescent , France/epidemiology , Child , Blood Transfusion , Puberty/drug effects , Administration, Oral , Triazoles/administration & dosage , Triazoles/therapeutic use , Chelation TherapySubject(s)
Anemia, Sickle Cell , Disease Progression , Humans , Anemia, Sickle Cell/complications , Adolescent , Male , Female , Young Adult , Infant, Newborn , Adult , Cohort Studies , Risk Factors , Chronic DiseaseABSTRACT
The impact of hydroxyurea (HU) on the ovarian reserve of female patients with sickle cell disease (SCD) remains poorly elucidated. Only direct histological analysis of ovarian follicle density can effectively evaluate HU's effect on ovarian reserve. By analyzing digitized slides of ovarian tissue from girls and young women with SCD who underwent ovarian tissue cryopreservation (OTC) before hematological stem cell transplantation (HSCT), we meticulously counted follicles and categorized them based on their growth stage. We then calculated the densities of different follicle types and assessed their correlation with patient characteristics, clinical manifestations, and treatments extracted from medical records. Seventy-six SCD patients participated in the study, with a median age at OTC of 10.2 years (interquartile range [IQR] 7.5, 14.6), and 50 (65.8%) were prepubertal. Of these, 35 patients (46.1%) had received HU, with a median daily dosage of 23.0 mg/kg (IQR 20.0, 25.0) and median exposure time of 44 months (IQR 24.0, 54.0). Primordial follicle density was comparable between the HU and non-HU groups (5.8 follicles/mm2 [IQR 1.0, 13.3] versus 4.2 follicles/mm2 [IQR 1.1, 14.4], respectively; P = .95). However, in the HU group, after adjusting for age, the density of growing follicles was marginally lower compared to the non-HU group (P = .09). Notably, other parameters such as vaso-occlusive crisis did not affect follicular density. In conclusion, exposure to hydroxyurea did not demonstrate a reduction in ovarian reserve in girls/women with SCD. Therefore, fertility preservation measures before initiating HU treatment do not seem necessary.