Congenital Leukemia Initially Presenting with Leukemia Cutis

Yıl 2012, Cilt: 10 Sayı: 3, 103 - 106, 01.12.2012

Melike Sezgin Evim , Meliha Demiral

https://doi.org/10.4274/Jcp.10.06

Öz

Introduction: Congenital leukemia represents less than 1% of childhood leukemia. Itsprognosis is poor. Myeloid form is the most common type, and leukemia cutis hasbeen observed in 25-30% of the patients. These skin lesions are defined as ‘blueberrymuffin’ type which are blue-violaceous and usually multiple and diffuse nodules. Case Report: She had diffuse blue-violaceous nodules since birth. She hospitalized dueto sepsis for 35 days. She was referred to our center with the suspicion of immunedeficiency. The initial physical findings were severe pallor, diffuse blue-violaceoussubcutanose nodules and hepatosplenomegaly. The leucocyte count was found 363 000/mm3. Acute monositer leukemia AML-M5 was determined with morphologicand flow cytometric evaluation of the peripheral blood. Conclusion: Congenital leukemia must be thought in differential diagnosis from otherunderlying disease presenting with blueberry muffin skin lesions

Anahtar Kelimeler

Congenital leukemia, blueberry muffin skin lesions

İlk Bulgusu Cilt Tutulumu Olan Konjenital Lösemi

Öz

Giriş: Konjenital lösemi, çocukluk çağı lösemilerinin %1’inden azını oluşturur.Prognozu kötüdür. En sık miyeloid form görülür. Olguların %25-30’unda lösemik cilttutulumu bulunur. ‘Blueberry muffin’ denilen bu lezyonlar; mavi-mor renkli, sıklıkla çoksayıda ve yaygın nodüler lezyonlardır. Olgu Sunumu: Doğduğundan beri yaygın mavi-mor renkli nodüler lezyonları olan, sepsisdüşünülerek yenidoğan ünitesinde 35 gün izlenen hasta, immün yetmezlik ön tanısı ilemerkezimize yönlendirilmiştir. Fizik bakısında; soluk görünümde, tüm vücudunda yaygın,mavi-mor renkli cilt altı nodüler lezyonları ve hepatosplenomegali saptandı. Lökositsayısı 363 000/mm3değerlendirilmesi ile Akut Monositer Lösemi AML-M5 tanısı aldı.Tartışma: “Blueberry muffin” cilt lezyonları ile gelen hastalarda mutlaka konjenitallösemi ayırt edilmelidir

Anahtar Kelimeler

Konjenital lösemi, blueberry muffin lezyonu

Kaynakça

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