Difficulties in diagnosing thalassemia minor

Here we outline a practical approach to the detection of thalassemias in some clinical settings. Any patient with a low mean corpuscular volume (MCV) with or without anemia or a neonatal screening result indicating possible presence of thalassemia minor needs evaluation by a hematologist. But sometimes it is difficult to diagnose a patient whose hemoglobin electrophoresis is normal. We review the various types of the thalassemia syndromes, where exact diagnosis could be difficult. (Turk Arch Ped 2009; 44 Suppl: 24-6

Basic transfusion management in children

Blood transfusion is an important part of the treatment of children cared by general pediatricians, hematologists, oncologists and surgeons. Transfusion carry many risks including transfusion related infections and adverse reactions. Physicians prescribing blood and blood components should have a basic understanding of the right indications for transfusion, correct storage conditions and management of adverse reactions. The purpose of this article is to cover basic transfusion strategies and "appropriate use" of blood and blood components. (Turk Arch Ped 2009; 44 Suppl: 19-23

Toxic epidermal necrolysis after the use of high-dose cytosine arabinoside

We report a fatal case of toxic epidermal necrolysis (TEN) resulting from a high dose of cytosine arabinoside (ARA-C), A 13-year-old girl with acute lymphocytic leukemia was treated according to the protocol of the BFM Group (BFM-95, HRG), On the fifth day after administration of a high dose of ARA-C (2 g/m(2) intravenously every 12 hours), she developed bullous lesions on the hands and soles that disseminated, evolving to necrosis, sepsis, and death on the 22nd day, ARA-C is frequently associated with dermatologic toxicity, but this is only the second case of toxic epidermal necrolysis described in connection with this drug

Glanzmann thrombasthenia: Cerrahpasa Medical Faculty experience

Aim: Glazmann thrombasthenia is a rare autosomal recessive disease characterized by a defect in platelet aggregation. Here we report the management of children with Glazmann thrombasthenia followed up at Cerrahpasa Medical Faculty Pediatric Hematology Department

Childhood mastocytosis: results of a single center

AIM: We aimed to retrospectively evaluate histopathological, demographic and clinical findings of children with mastocytosis diagnosed with mastocytosis in our clinic. MATERIAL AND METHODS: The files of 21 patients diagnosed with mastocytosis between 2000 and 2014 in our clinic were retrospectively analyzed. RESULTS: All patients had cutaneous mastocytosis, 19 patients had urticaria pigmentosa and 2 patients had mastocytoma. The male-female ratio was: 1/1.6. The median age for onset of disease was 12.1 months and the disease occured in the newborn period in 3 patients. While all patients had eruption, 10 patients had pruritis, 1 patient had a bullous formation, 1 patient had abdominal pain and 1 patient had attacks of redness throughout the body and a sense of burning in the chest. Two patients had a positive familial history. The diagnosis was confirmed with skin biopsy in all patients. The median follow up time of the patients were 5 years. The patients were treated with H1, H2 antihistaminics, local moisturizing creams and topical corticosteroid drugs. The lesions resolved completely in 4 patients who reached to puberty and 7 patients had marked improvement in a 5.5 year-follow-up period. Ten patients had stabile lesions in a 3.6 year-follow-up period. CONCLUSIONS: Most cases of childhood mastocytosis are observed in the form of cutaneous mastocytosis. The prognosis is good; the disease limits itself and is prone to regress in the adolescent period