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    Autoimmune hemolytic anemia in children, 20 years experience of single center
    (2020) Albayrak, Canan; Karakurt, Neslihan; Albayrak, Davut
    Aim: Autoimmune hemolytic anemia (AIHA) is a rare disease in pediatrics, whose mortality rate was reported to be as high as 10%. AIHA can be primary or secondary to other diseases, Availability of new immunsupressive drugs like mycofenolate mofetil (MMF), has provided the opportunity to reduce long term steroid administration and mortality. In this study we aimed to represent AIHA patients of 20 years, from single centre and focus on the causes, treatment and outcomes. The secondary object was to represent outcomes of patients who received MMF.Material and Methods: This study was designed as a retrospective study. Patients aged three months to 18 years old with hemoglobin level less than 10 g/dl and positive DAT with signs of hemolysis were included in the study.Results: Twenty five AIHA patients (F/ M: 14/ 11) aged 6.2± 4.6 years old were followed- up for a mean period of 5.3± 4.8 years. Primary AIHA was detected in 12 (48%) patients. Immune deficiency/ autoimmune lymphoproliferative syndrome was the prominent etiological factor in secondary AIHA. The other underlying diseases were systemic lupus erythematosus, malignancy, autoimmune hepatitis and infection.Eleven patients received MMF with a mean duration of 2.6± 1.6 years. Two of them had primary AIHA, the others had secondary disease. During the follow- up time, eight patients (75%) achieved remission with MMF. None of MMF users developed side effect. One but all patients with AIHA achieved remission. No death related to AIHA was recorde': recorded Conclusion: Understanding the biology of the disease and making accurate diagnosis is important to avoid harmful treatment and to consider targeted therapy. After the failure of first line therapy with steroids or as a steroid- sparing agent, MMF seems to be an effective second-line maintanance immunosuppressive drug without significant side effects.
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    Avascular necrosis of the femoral head in sickle cell disease
    (2017) Karakurt, Neslihan; Deveci, Mehmet Ali
    Sickle cell disease (SCD) is a life threatening hemoglobinopathy effecting various systems. SCD is an important health issue in Turkey, especially in the southern areas. The patients suffer from complications resulting from vaso-occlusion and hemolysis. Twenty- two percent of children and 49% of adults with Hb SS are reported to have avascular necrosis (AVN). The SCD panel 2014 recommends to evaluate all children with SCD and hip pain for AVN by history, physical examination, radiography and magnetic resonance imaging. Long term daily oral treatment with hydroxyurea has been shown to reduce complications in SCD. Hydroxyurea does not only increase the level of fetal hemoblobin but also it causes a reduction in the number of circulating leukocytes, increases red blood cell size. Here in two SCD patients who developed AVN in femoral head are reported and it is aimed to raise awareness to this morbidity and the importance of regular use and dosage escalation of hydroxyurea therapy.
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    Trombocytopenia related to colchicine: Report of two cases
    (2017) Karakurt, Neslihan; Candir, Mehmet Onur; Kosan Culha, Vildan; Yarali, Nese; Cakar, Nilgun; Ozbek, Namik
    Familial Mediterranean fever (FMF) is the most frequent autoinflammatory disease which can be well controlled with lifelong use of colchicine. Daily colchicine use has been shown to reduce the frequency, severity, and duration of attacks. Colchicine also has been found to be effective in decreasing the prevalence of amyloidosis. Therapeutic oral dose of colchicine (0.5-2.0 mg/ day) may cause cramping, abdominal pain, hyperperistalsis, diarrhea and vomiting. Besides, colchicine may rarely cause bone marrow failure, agranulocytosis and/ or thrombocytopenia as well. Here in we report two pediatric cases of FMF who developed trombocytopenia after colchicine medication and call attention to colchicine related trombocytopenia.