Talasemi Hastalarında Fertilite ve Gebelik Sonuçları: Türk Deneyimi

Objective: in recent years, the rates of marriage and pregnancy are increasing in patients with thalassemia major. the aim of the present study was to investigate the fertility rate of thalassemic patients and the course of pregnancies in terms of mother and infant health. Materials and Methods: in this observational study patients with major hemoglobinopathy were evaluated regarding marital status, the need for assisted reproductive techniques, fertility rate, iron status, and pregnancy complications. Results: Seventeen female patients gave birth to 21 healthy infants. About one-third of the patients needed assisted reproductive techniques. Thalassemia major patients showed increased serum ferritin levels from 1203±1206 µg/L at baseline to 1880±1174 µg/L at the end of pregnancy. All babies are still alive and healthy. Conclusion: Pregnancy in patients with thalassemia can be safe for the mother and newborn with close monitoring and a multidisciplinary approach.Amaç: Son yıllarda, talasemi majör olgularının evlilik ve gebelik oranları giderek artmaktadır. Bu çalışmanın amacı, talasemi hastalarının fertilite oranlarının araştırılması ve anne ve bebek sağlığı açısından gebelik sonuçlarının değerlendirilmesidir. Gereç ve Yöntemler: Bu gözlemsel çalışmada; majör hemoglobinopatili hastalar; evlenme ve çocuk sahibi olma oranları, yardımcı üreme tekniklerine gereksinimleri, demir statüleri ve gebelik komplikasyonları açısından değerlendirilmiştir. Bulgular: on yedi talasemik kadın hasta, toplam 21 sağlıklı bebek doğurmuştur. Hastaların üçte biri bebek sahibi olabilmek için yardımcı üreme tekniğine ihtiyaç duymuştur. Talasemi majör olgularının serum ferritin değerleri hamileliğin başında ortalama 1203±1206 ug/L saptanmış olup, hamileliğin sonunda 1880±1174 ug/L seviyesine yükselmiştir. Tüm bebekler halen hayatta ve sağlıklıdırlar. Sonuç: Talasemi olgularında hamilelik süreci yakın takip ve multidisipliner yaklaşım ile beraber güvenli olarak geçirilebilecektir

Retrospective Evaluation of Childhood Cutaneous Mastocytosis Cases

Aim: Mastocytosis is a rare disease characterized by clonal mast cell proliferation in one or more organs. It can lead to different clinical manifestations and has no definitive treatment. in this study, we aimed to evaluate the clinical and laboratory characteristics of our patients diagnosed with mastocytosis in our clinic. Materials and Methods: Thirteen patients that were followed up with the diagnosis of mastocytosis at the Pediatric Hematology Clinic, in the Ege University Faculty of Medicine between November 1999 and April 2016 were retrospectively analyzed. Results: Seven of patients were female (53.8%) and six were male (42.6%). the mean age at diagnosis was 20 (3-68) months. At the time of diagnosis, complete blood count and peripheral smear were found to be compatible with the anemia of iron deficiency in three patients. Other parameters were normal. Mean tryptase level was detected as 5.9 (3.6-16.6) ng/mL, and only one tryptase level was found as slightly increased. the median level of total IgE was 91.1 (4.47-362) IU/mL. Mast cell proliferation was not detected in bone marrow aspiration and biopsy material of any patients. All of the cases were evaluated as cutaneous type mastocytosis. Conclusion: the possibility of mastocytosis in systemic form in childhood is very rare and bone marrow examination may be necessary in selected cases

Febril Nötropenili Çocuklarda Bakteriyemiyi Öngörmede İnterlökin-6, İnterlökin-8, İnterlökin-10'un Tanısal Doğruluğu

Despite improvements in diagnosis and treatment, infections are still a major cause of morbidity and mortality in children with febrile neutropenia. In the majority of febrile episodes, the source of infection cannot be defined. In this study, we aimed to identify the earlier predictors of bacteremia/fungemia and a useful cytokine to identify the source of infection and to discriminate the patients with culture-confirmed bacterial/fungal infection. The most sensitive cytokine was interleukin (IL)-10 and the most specific was IL-8 in predicting culture-confirmed cases. IL-8 had greater sensitivity and specificity in determination of gram-negative bacterial infections with a higher negative predictive value; therefore, IL-8 can be used particularly to rule out gram-negative bacterial infections. IL-6, IL- 8, and IL-10 circulating levels were shown to be higher in cases of infection. Further studies are needed to recommend a routine practice for predicting culture-confirmed bacterial infections.Tanı ve tedavideki gelişmelere rağmen, febril nötropenili çocuklarda enfeksiyonlar morbidite ve mortalitenin önemli bir nedenidir. Febril nötropeni epizodlarının çoğunluğunda enfeksiyon odağı belirlenememektedir. Bu çalışmada, enfeksiyon odağını ve kültürle kanıtlanmış bakteriyel ve fungal enfeksiyonlarını belirlemede daha erken belirteçler bulunması amaçlanmıştır. Kültür ile kanıtlanmış enfeksiyonları belirlemede en hassas sitokin interlökin (IL)-10 ve en özgül sitokin IL-8'dir. Gram-negatif bakteriyel enfeksiyonları belirlemede en hassas ve özgül sitokin IL-8'dir ve IL-8'in negatif prediktif değerinin yüksek olması nedeniyle, özellikle gram-negatif bakteriyel enfeksiyonları dışlamada kullanılabilir. IL-6, IL-8, IL-10'un kan düzeyleri enfeksiyon durumunda daha yüksek saptanmıştır. Sitokinlerin kültür ile kanıtlanmış enfeksiyonları belirlemede rutin kullanımını önermek için gelecekte yapılacak çalışmalara ihtiyaç vardır

Hypertension and Life-Threatening Bleeding in Children with Relapsed Acute Myeloblastic Leukemia Treated with FLT3 Inhibitors

Experiences with new multikinase inhibitors are limited, especially in children. In this report we summarize our experience with 2 patients with relapsed acute myeloblastic leukemia (AML), one with FMS-like tyrosine kinase-3-internal tandem duplication mutation and the other with a single base mutation (D835Y). Both patients received sorafenib, one for 19 days and the other for 42 days, with clofarabine-including chemotherapy. One additionally received sunitinib for a total of 20 days. Both patients developed severe pancytopenia, hypertension, life-threatening bleedings from the gastrointestinal system, and, finally, intrapulmonary hemorrhage. Although both reached severe aplasia of the bone marrow without blastic infiltration, death occurred with neutropenic sepsis

Rare severe mycotic infections in children receiving empirical caspofungin treatment for febrile neutropenia

Empirical antifungal therapy is most often given to patients with leukemia. However breakthrough fungal infections under antifungal therapy are not uncommon. Four children, with hematologic malignant disease developed mycotic breakthrough infections while on empirical caspofungin treatment for a median of 14 (range 11–19) days. Trichosporon asahii was detected in the blood culture of two patients and Geotrichum capitatum in the other two (one patient also had positive cerebrospinal fluid culture). Because the patients’ clinical situation worsened, voriconazole was empirically added for two patients three and five days before the agent was detected. The first sterile blood culture was obtained 3–7 days of voriconazole treatment. All patients reached clear cultures but one patient died. One patient with central nervous system infection with G. capitatum had severe neurological sequelae. Very severe fungal infections can occur during empirical caspofungin therapy. Therefore, patients should be followed closely. Keywords: Geotrichum capitatum, Trichosporon asahii, Invazive fungal infection, Febrile neutropeni