İlk bulgusu cilt tutulumu olan konjenital lösemi
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Date
2011-02-13
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Uludağ Üniversitesi
Abstract
Giriş: Konjenital lösemi, çocukluk çağı lösemilerinin %1’inden azını oluşturur. Prognozu kötüdür. En sık miyeloid form görülür. Olguların %25-30’unda lösemik cilt tutulumu bulunur. ‘Blueberry muffin’ denilen bu lezyonlar; mavi-mor renkli, sıklıkla çok sayıda ve yaygın nodüler lezyonlardır. Olgu Sunumu: Doğduğundan beri yaygın mavi-mor renkli nodüler lezyonları olan, sepsis düşünülerek yenidoğan ünitesinde 35 gün izlenen hasta, immün yetmezlik ön tanısı ile merkezimize yönlendirilmiştir. Fizik bakısında; soluk görünümde, tüm vücudunda yaygın, mavi-mor renkli cilt altı nodüler lezyonları ve hepatosplenomegali saptandı. Lökosit sayısı 363 000/mm3 idi. Periferik kanının morfolojik ve akım sitometrisi ile değerlendirilmesi ile Akut Monositer Lösemi (AML-M5) tanısı aldı. Tartışma: “Blueberry muffin” cilt lezyonları ile gelen hastalarda mutlaka konjenital lösemi ayırt edilmelidir.
Introduction: Congenital leukemia represents less than 1% of childhood leukemia. Its prognosis is poor. Myeloid form is the most common type, and leukemia cutis has been observed in 25-30% of the patients. These skin lesions are defined as ‘blueberry muffin’ type which are blue-violaceous and usually multiple and diffuse nodules. Case Report: She had diffuse blue-violaceous nodules since birth. She hospitalized due to sepsis for 35 days. She was referred to our center with the suspicion of immune deficiency. The initial physical findings were severe pallor, diffuse blue-violaceous subcutanose nodules and hepatosplenomegaly. The leucocyte count was found 363 000/mm3. Acute monositer leukemia (AML-M5) was determined with morphologic and flow cytometric evaluation of the peripheral blood. Conclusion: Congenital leukemia must be thought in differential diagnosis from other underlying disease presenting with blueberry muffin skin lesions.
Introduction: Congenital leukemia represents less than 1% of childhood leukemia. Its prognosis is poor. Myeloid form is the most common type, and leukemia cutis has been observed in 25-30% of the patients. These skin lesions are defined as ‘blueberry muffin’ type which are blue-violaceous and usually multiple and diffuse nodules. Case Report: She had diffuse blue-violaceous nodules since birth. She hospitalized due to sepsis for 35 days. She was referred to our center with the suspicion of immune deficiency. The initial physical findings were severe pallor, diffuse blue-violaceous subcutanose nodules and hepatosplenomegaly. The leucocyte count was found 363 000/mm3. Acute monositer leukemia (AML-M5) was determined with morphologic and flow cytometric evaluation of the peripheral blood. Conclusion: Congenital leukemia must be thought in differential diagnosis from other underlying disease presenting with blueberry muffin skin lesions.
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Keywords
Konjenital lösemi, Blueberry muffin lezyonu, Congenital leukemia, Blueberry muffin skin lesions
Citation
Evim, M. S. ve Demiral, M. (2012). "İlk bulgusu cilt tutulumu olan konjenital lösemi". Güncel Pediatri, 10(3), 103-106.