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ß-talassemi ve orak hücreli anemi (OHA) Çukurova’da önemli saðlýk problemleridir. Yýllardýr bu bölgede tarama programlarý sürdürülmektedir. 1992 yýlýndan beri Çukurova Üniversitesi’nde evlilik öncesi tarama ve prenatal taný programý yürütülmektedir. Tüm bunlara raðmen, tarama programlarý baþarýsýz olmuþtur. Bu çalýþmanýn amacý toplumun bu ciddi herediter hastalýklara duyarlýlýðýný arttýrmaktýr. Hedef kitle 8. sýnýf öðrencileridir. Biyoloji ve/veya fen kitaplarýndaki ders programlarý incelendikten sonra öðrencilere talassemi ve OHA konusunda görsel sunum yapýlmýþtýr. Yedi ilköðretim okulunda toplam 1221 öðrenci bilgilendirilmiþtir. Beþ okula sunum öncesi, iki okula sunum sonrasý anket yapýlmýþ ve iki grubun sonuçlarýnýn karþýlaþtýrýlmasý, verilen bilginin iyi alýndýðýný göstermiþtir. Doktorlarý, saðlýk personelini, sivil ve dini liderleri kapsayan geniþletilmiþ bir eðitim programý ve devlet desteði talassemi ve OHA’lý doðumlarý sýfýra indirebilir.

ß-thalassemia and sickle cell anemia (SCA) are major health problems in Çukurova. Screening studies have been carried on in this region for many years. The government started premarital screening programme and prenatal diagnosis has been done in Çukurova University Medical Faculty since 1992. In spite of all these, the prevention programme has not been successful. The purpose of this study was to start an education programme to increase the awareness of the people for these severe hereditary diseases. The target population was the 8th grade students. A visual presentation was given to students on Thalassemia and SCA after examining the curriculum in the Biology and/or the Science books. A total of 1221 students in seven Elementary Schools were informed. A questionnaire was given to five schools before the presentation and to two after the presentation. The comparison of the two group’s result showed that the information given was well received. Thus, an extensive education programme encompassing doctors, health personel, civil and religious leaders and the support of the government will result in nil SCA and Thalassemia births.

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Trombositopeni myelodisplastik sendromu (MDS)’nun ilk bulgusu olabilir ve refrakter trombositopeni (RT) adý altýnda Dünya Saðlýk Örgütü (WHO)’nün akut lösemi ve MDS sýnýflandýrmasýnda multilinaj displaziýe e.lik eden refrakter sitopeni (RCMD) grubuna dahil edilmektedir. MDS’li olgularýn %60-80’inde anormal sitogenetik bulgular olur. En sýk görülen sitogenetik anomaliler arasýnda monozomi 5, 5q-, monozomi 7, trizami 8, 20q delesýonu ve X ya da Y kromozom kaybý vardýr. Biz bu yazýda RT’li dokuz olgunun klinik özelliklerini bildirdik. yedi olguda sitogenetik anomali saptandý. Taný sýrasýnda normal karyotipe sahip iki olgudan bir tanesi AML’ye dönüþtü. Median 29 aylýk bir izleme sonunda, iki olgu hemorajiden kaybedilirken, bir olgu AML’ye dönüþerek kaybedildi. RT’li olgularýn özellikleri ve prognozlarýnýn daha geniþ serilerde deðerlendirilmesi gerekmektedir.

Thrombocytopenia may be the presenting cytopenia of myelodysplastic syndrome (MDS) and is named as refractory thrombocytopenia (RT) and categorized in the refractory cytopenia with multilineage dysplasia (RCMD) group according to the recent World Health Organization (WHO) classification of the acute leukemias and MDS. Abnormal cytogenetics can be found in 60% to 80% of patients with MDS. Most common cytogenetic abnormalities include monosomy 5, 5q-, monosomy 7, trisomy 8, deletion 20q and loss of X or Y chromosome. Here we report clinical features and outcomes of nine patients with RT. Cytogenetic abnormalities were detected in seven. Among two patients who have a normal karyotype at diagnosis, one of them transformed to acute myeloid leukemia (AML). During a median follow-up of 29 months, two patients died of hemorrhagia and one of AML. The features and prognosis of patients with RT needs to be determined by larger series.

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Bu yazýda Türk toplumunda Faktör V 1691 G-A ve protrombin 20210 G-A mutasyonlarýnýn birarada bulunma sýklýðý özetlenmekte ve bu birlikteliðin tromboz riskini arttýrdýðý vurgulanmaktadýr. Türk toplumu trombofili tarama programlarý için bu iki varyant eklenmelidir.

This report summarizes the coexistence of two mutations; Factor V 1691 G-A and prothrombin 20210 G-A in Turkish population and emphasises on the point that, this coexistence increases the risk of thrombosis in such patients. In thrombophilia screening programs, these two variants should be included, particularly in Turkish population.

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Sisplatin (CDDP) günlük klinik pratikte en çok kullanýlan anti-neoplastik ajanlardandýr. CDDP’nin en önemli toksisiteleri, nefrotoksisite ve ototoksisitedir. Sýçanlarda CDDP’ye baðlý, akut renal yetmezlikte serbest oksijen radikallerinin önemli rol oynadýðý bilinmektedir. α-tokoferol iyi bilinen antioksidan ajanlardandýr. Bu çalýþma α-tokoferolün sýçan böbreðinde CDDP’ye baðlý lipid peroksidasyonu önlemedeki rolünü araþtýrmak için yapýlmýþtýr. Erkek Wistar sýçanlarý 3 gruba bölünmüþtür. 1. kontrol (periton için salin), 2. CDDP (periton için10 mg/kg), 3. α-tokoferol (200 mg/kg) + CDDP, periton içi. Tedavinin üçüncü günü sýçan böbrek dokularý incelenmiþtir. CDDP verilen sýçanlarda malonildialdehid (MDA) düzeyleri yüksek bulunmuþtur (p< 0.05). CDDP artý α-tokoferol alan sýçanlarda ise kontrol grubuna göre fark saptanmamýþtýr. Bu sonuçlar α-tokoferolün CDDP’ye baðlý lipid peroksidasyonu önlemede kullanýlabileceðini göstermektedir.

Cisplatin (CDDP) is one of the most commonly used antineoplastic agents in current clinical practice. The major toxicities of CDDP are nonhaematological as nephrotoxicity and ototoxicity. Free oxygen radicals are known to play major role in CDDP-induced acute renal failure in rats. α-tocopherol is one of the well-known antioxidant agents. This study was designed to investigate the role of α-tocopherol pretreatment against CDDP-induced lipid peroxidation in rat kidney. Male Wistar rats were divided into three groups and treated as follows: control (saline intraperitoneally), CDDP (10 kg/kg, intraperitoneally), α-tocopherol (200 kg/kg, plus CDDP, intraperitoneally). Rats were sacrificed on third day of the treatment, and kidney tissues were obtained and analyzed. CDDP-treated rats showed high malondialdehyde (MDA) levels (p< 0.05). In the CDDP plus α-tocopherol group, renal MDA levels were not significantly different from the controls. These data suggest that α-tocopherol may be used to prevent CDDP-induced lipid peroxidation.

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Sekonder ya da tedaviye baðlý AML (t-AML) çeþitli kemoterapi rejimlerinin komplikasyonu olarak ortaya çýkar. Pediatrik yaþ grubunda osteosarkom tedavisine sekonder malignite nadirdir. Onbeþ yaþýnda bir kýz çocuðu hastanemize akut lösemi yakýnma ve bulgularý ile baþvurdu. Bir yýl öncesi sol femur osteosarkom tanýsý ile doksorubisin ve sisplatin alan hastaya kemik iliði incelemesi ve immünfenotiplendirme ile akut monoblastik lösemi tanýsý kondu. Sitogenetik anomali saptanmadý. Hastanýn babasý pelvik kondrosarkoma tanýsý almýþ olup, ailesel yatkýnlýk düþünüldü. Biz bu olguyu sitogenetik anomalisi olmayan nadir bir t-AML olgusu olarak deðerlendirdik ve nedeninin topoizomeraz II inhibitörleri içeren bir rejime platinum bileþikleri eklenmesi olduðuna inanmaktayýz.

Secondary or therapy-related acute myeloid leukemia (t-AML) occurs as a complication of various chemotherapy regimens. In pediatric age group, leukemia as a second malignancy after osteosarcoma treatment with doxorubicin and cisplatin is relatively rare. A 15-year-old girl was admitted to our hospital with signs and symptoms of leukemia. She had been treated one-year earlier with doxorubicin and cisplatin for osteosarcoma of the left femur. The patient was diagnosed with acute monoblastic leukemia on the basis of bone marrow examination and immunophenotype analysis. There were no cytogenetic abnormalities. The patient’s father had been diagnosed with pelvic chondrosarcoma, which suggested a familial susceptibility to cancer. We interpreted this as a rare case of t- AML with normal cytogenetic analysis, and believe the disease was induced by the addition of platinum compounds to a regimen including topoisomerase II inhibitors.

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Trombositopeni brusellozisin nadir görülen hematolojik komplikasyonlarýndan biridir. Bu yazýda, aðýr trombositopeni ile birlikte seyreden bir brusellozis olgusu sunuldu. Hasta, burun kanamasý, ekimoz, ateþ, trombositopeni (0.6 x 103/μL) ve anemi bulgularý ile hematoloji kliniðine kabul edildi. ilk tanýsý idiyopatik trombositopenik purpura olan hastaya kortikosteroid tedavisi baþlandý ve trombosit süspansiyonu verildi. Bu tedaviye raðmen klinik ve laboratuvar bulgularýnda düzelme olmadý. Kemik iliði aspirasyonunda önemli bir patoloji tespit edilmedi. Kan kültürlerinde yedinci günde Brucella abortus üremesi üzerine steroid tedavisi kesildi, rifampisin ve doksisiklin baþlandý. Antibiyotik tedavisinin ikinci haftasýnda hastanýn klinik ve laboratuvar bulgularý (trombosit sayýsý: 205 x 103/μL) düzeldi. Bu olgu, bruselloziste trombosit sayýsýnýn sýfýra yakýn deðerlere düþebileceðini göstermesi bakýmýndan önemlidir. Özellikle endemik bölgelerde, ateþ ve trombositopeni etyolojisinde burusellozis akýlda bulundurulmalýdýr.

Thrombocytopenia is one of the rare hematologic complication of brucellosis. Herein a case of brucellosis with severe thrombocytopenia was reported. The patient was admitted to hematology service with epistaxis, ecchymoses, fever, thrombocytopenia (0.6 x 103/μL) and anemia. His initial diagnosis was idiopathic thrombocytopenic purpura. Corticosteroid treatment was started and erythrocyte and platelet suspensions were transfused. Despite this treatment clinical symptoms and laboratory disorders were not improved. No significant pathology was detected in the examination of bone marrow aspiration. On the seventh day, Brucella abortus was yielded from his blood cultures. Steroid was stopped and rifampicin plus doxycycline started. His clinical symptoms were disappeared and laboratory findings improved (thrombocyte count: 205 x 103/μL) at the second week of the antibiotic treatment. This case was interesting showing us that thrombocyte count might decrease to very low levels in brucellosis. So that, especially in the endemic areas, brucellosis should be kept in mind for the etiology of fever and thrombocytopenia.

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Kaposi sarkomun ortaya çýkmasý iatrojenik veya altta yatan hastalýða baðlý immünyetmezlik sonucu olur. Bu yazýda, sistemik kemoterapi ile kýsmi klinik remisyon elde edilen non-Hodgkin lenfomalý bir olguda tedavi sonrasý geliflen iatrojenik Kaposi sarkoma sunulmaktadýr.

The development of Kaposi’s sarcoma (KS) has been associated with either iatrogenic or underlying disease related immunodeficiency. We report a case with iatrogenic Kaposi’s sarcoma developed after treatment of non- Hodgkin’s lymphoma (NHL) who was achieved partial clinical remission with systemic chemotherapy.

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