Diffuse Cutaneous Mastocytosis: A Current Understanding of a Rare Disease.

Mastocytosis is a heterogeneous disease characterized by the expansion and accumulation of neoplastic mast cells in various tissues. Diffuse cutaneous mastocytosis (DCM) is a rare and most severe form of cutaneous mastocytosis, which typically occurs in childhood. There have been reports of a familial DCM with specific gene mutations, indicating both sporadic and hereditary factors involved in its pathogenesis. DCM is associated with severe MC mediator-related symptoms and an increased risk of anaphylaxis. The diagnosis is based on the appearance of skin lesions, which typically show generalized thickening, erythroderma, blistering dermographism, and a positive Darier's sign. Recognition, particularly in infants, is challenging due to DCMs resemblance to other bullous skin disorders. Therefore, in unclear cases, a skin biopsy is crucial. Treatment focuses on symptom management, mainly including antihistamines and mast cell stabilizers. In extremely severe cases, systemic steroids, tyrosine kinase inhibitors, phototherapy, or omalizumab may be considered. Patients should be equipped with an adrenaline autoinjector. Herein, we conducted a comprehensive review of literature data on DCM since 1962, which could help to better understand both the management and prognosis of DCM, which depends on the severity of skin lesions, intensity of mediator-related symptoms, presence of anaphylaxis, and treatment response.

Pregnancy After Hematopoietic Cell Transplant in Severe Aplastic Anemia: Report of 2 Cases.

BACKGROUND: Hematopoietic stem cell transplants (HSCT) treat malignant and nonmalignant diseases. Aplastic anemia (AA) is a rare condition associated with ineffective hematopoiesis. The first-line treatment for AA is an allogenic hemopoietic stem cell transplant (allo-HSCT). After allo-HSCT, most patients become infertile. METHODS: This study presents 2 case reports of women who become pregnant after allo-HSCT in the treatment of severe AA. In both women, conditioning was performed using the fludarabine, cyclophosphamide, and antithyroglobulin antibodies protocol. RESULTS: Case 1, a 27-year-old woman, underwent allo-HSCT at the age of 19. She received cyclosporine immunosuppression. The transplant was without complications. The woman's menstrual resumption was observed after 2 months. Eight years post-transplantation, the woman had her first pregnancy. Fetal growth restriction was diagnosed, and she was qualified for labor induction after the 37th week of gestation. She gave birth to a baby boy in good general condition. Case 2 is a 28-year-old woman with allo-HSCT at aged 25. The procedure was performed during a period of active fungal infection. Immunosuppression with cyclosporine and methotrexate was administered. During the transplant procedure, she developed acute kidney injury and liver failure. Her menstrual cycle returned 1 month after the transplant. Three years after the transplant, the woman was pregnant with twins. After 37 weeks of gestation, the woman was qualified for Cesarean delivery. Both babies, a boy and a girl, were in good general condition. CONCLUSION: Preservation of fertility after allo-HSCT is feasible, particularly in those with AA treated with conditioning regimens without total body irradiation with lower doses of alkylating agents.

Uterine Artery Pulsatility Index in Pregnant Kidney or Liver Recipients and Its Impact on Perinatal Outcome.

BACKGROUND: The uterine artery pulsatility index (UtA PI) is associated with blood flow to the placenta. Its increased values imply impaired placentation. This study aimed to evaluate UtA PI measurements in first-trimester ultrasound in pregnancies after kidney (KTx) or liver transplantation (LTx) and its relationship with perinatal outcome. MATERIALS AND METHODS: A retrospective analysis of 72 pregnancies in female kidney (35) or liver (37) transplant recipients between 2017 and 2023 was performed. Data concerning UtA PI were available for 17 kidney and 19 liver recipients. Statistical analysis of variables between KTx and LTx groups and the correlation with perinatal outcomes was performed using Student's t test and Pearson's correlation with P < .05 considered statistically significant. RESULTS: The mean UtA PI results were similar, and there were no statistical differences between the group of pregnant kidney and liver recipients with mean values of 1.46 (SD 0.44] and 1.73 (SD 0.51] respectively (P = .10). The mean neonate birth weight was lower in KTx group (2158 g ([SD 723 g]) compared with the LTx group (2780 g [SD 754g]; P =.02). In the KTx and LTx groups, mean UtA PI was in negative correlation with Apgar score in the first minute (P = .04, P = .01 respectively). CONCLUSIONS: Uterine artery Doppler is useful in predicting perinatal outcomes in the general population and organ recipient pregnancies, even in the early stages of pregnancy, as we observed the correlation between UtA PI and Apgar score. Pregnant kidney recipients remain at higher risk for complications and more unpredictable outcomes than liver recipients.

Level of Knowledge of Post-Transplant Women About Breastfeeding During Immunosuppression.

BACKGROUND: The number of women treated with immunosuppressants is increasing. Often, these women are of childbearing age. Consequently, they must face the decision of whether to breastfeed when they do have a child. Although available studies recommend breastfeeding during immunosuppression, patients appear to need more knowledge to enable the decision-making process. This study aimed to investigate the knowledge of women after transplantation about breastfeeding during immunosuppression and their source of information. MATERIAL AND METHODS: We performed a cross-sectional study from February 1 through August 31, 2022, with 45 female graft recipients (28 post-kidney and 17 post-liver transplantation) of childbearing age (15-49 years). The women were polled during their routine outpatient appointments and then divided into 2 groups: parous women (group 1, n = 26) and nulliparous women (group 2, n = 19). RESULTS: Most of the patients (84%) were administered tacrolimus-based regimens. Thirty-seven women voiced concerns about the possible harm to their babies through immunosuppressants in their breast milk (82%). The average score for knowledge of the benefits of breastfeeding was 51%; 58% in group 1, and 41% in group 2. Among parous women, 5 breastfed on immunosuppression, 15 did not, and the remainder did not take immunosuppression during breastfeeding. The decision regarding breastfeeding was influenced mainly by counseling from gynecologists (75%) and transplantologists (56%). CONCLUSION: Women's knowledge about the benefits of breastfeeding and the possibility of it during immunosuppression is not satisfactory.

Pregnancies in Women After Liver Transplant due to Wilson's Disease-Case Series.

Wilson's disease is a rare autosomal recessive disorder. Due to a defect in membrane copper transporter, copper is not excreted in the bile and accumulates in the tissues. The only treatment for acute liver failure in Wilson's disease is a liver transplant. AIM: Assessment of the course of pregnancies and comparison of obstetric outcomes in female liver transplant recipients in the course of Wilson's disease. METHODOLOGY: Retrospective analysis of data of women, who were pregnant and gave birth in the years: 2017 to 2023. Evaluation of their liver function used pharmacotherapy and obstetric outcomes. RESULTS: We recorded 11 pregnancies in liver transplantation recipients due to Wilson's disease. Ten single pregnancies and 1 twin (DCDA) were observed. In all pregnancies, graft functions and immunosuppressive drug concentrations were monitored. Three women suffered from epilepsy, one was diagnosed with psychiatric disorder. Two were diagnosed with cholestasis, and another 2 with gestational diabetes. Two of them were treated for pregnancy-induced hypertension and 2 developed preeclampsia. Deterioration of liver function parameters in pregnancy was observed in 2 cases. In total, 8 full-term babies were born and 4 late-preterm, including twins at 35 weeks of gestation. Seven pregnancies were delivered by caesarean section and 4 delivered vaginally. No complications in early postpartum period have been reported. CONCLUSIONS: Women with Wilson's disease treated with organ transplantation have a chance of successful pregnancies and deliveries.

Pregnancy in Liver and Kidney Female Recipient: A Case Report.

Deterioration of kidney function after orthotopic liver transplantation is a common complication that may occur after perioperative acute kidney injury (AKI) and preexisting or developing chronic kidney disease (CKD). AKI is described in the early postoperative period in more than half of recipients, whereas the main cause of CKD is pharmacotherapy. When end-stage renal failure occurs, patients may be qualified for additional transplantations. We present a rare case of a 27-year-old woman who, as a teenager, underwent 2 liver transplantations due to Wilson's disease. Surgeries were complicated by systemic infection and multiple organ failure. The kidneys did not regain their function, and therefore, after 6 months of dialysis, the organ was transplanted. Three organ transplantations were performed. Due to the patient's willingness and good graft functions, the patient started trying to conceive. Three months before successful conception, immunosuppressive therapy was changed to tacrolimus and azathioprine. Pregnancy was complicated by pregnancy-induced hypertension, and its course was closely monitored. Organ functions and immunosuppressive therapy were regularly assessed. Due to the pre-eclampsia developed in the 35th week of gestation, a Cesarean delivery was performed, and she gave birth to a daughter weighing 2350 g (Apgar 7-7-8). The patient decided to breastfeed. There were no obstetric complications or graft function deterioration in the early postpartum period. Mother and daughter left home after 7 days of hospitalization. The presented clinical situation proves that multiorgan transplantation recipients can have a successful pregnancy without impairing graft functions. Therefore, the pregnancy requires adequate preparation and increased care.

Family Planning and Assessment of the Frequency of Exposure to Drugs Contraindicated in Pregnancies After Kidney or Liver Transplantation: A Retrospective Cross-Sectional Study.

BACKGROUND: A successful organ transplant restores gonadal function in the first months after surgery, which leads to the normalization of menstrual cycles and increases the chance of pregnancy. Recipients of organ transplants should effectively prevent pregnancy for a minimum of 1 year and optimally up to 2 years after surgery. This study aimed to evaluate the incidence of unplanned pregnancies in female organ transplant recipients METHODS: A cross-sectional, single-center survey study of 46 pregnant organ recipients who were hospitalized at the Department of Obstetrics and Gynaecology. RESULTS: In the post-transplant period, we recorded 46 patients, including 27 kidney recipients (59%) and 19 liver recipients (41%). Forty-nine respondents reported 66 pregnancies, of which 52 ended in live births (79%). Twenty of the pregnancies were not planned. In that group, 16 pregnancies ended in labor, 2 in miscarriage, and 2 in termination. In 10 of the unplanned pregnancies, the women were treated with potentially teratogenic drugs in the first trimester. The duration of the pregnancy was shorter in the group of women who had not planned their pregnancies and had conceived during potentially teratogenic therapy (30.66 ± 3.61 weeks) than in women who had planned their pregnancies (34.95 ± 4 weeks, P < .0215). CONCLUSION: Women after organ transplantation are at high risk for pregnancy complications. Therefore, conception planning is an important element of post-transplant care, especially because the percentage of unplanned pregnancies in this group remains high despite the use of potentially teratogenic drugs.