Özet:

Introduction: Congenital leukemia is defined as the manifestation of leukemia that occurs in the first 4 weeks of life. Estimated incidence varies between 1–5 cases per million live births. Which is the main cause of death of all malignancies in newborns. Acute myeloid leukemia (AML) (56–64%) is more common than acute lymphoblastic leukemia (ALL) (21–38%). The predominant subtypes of AML in neonates are myelomonocytic, monocytic and megakaryotic leukemia (M4, M5, and M7). Leukemia cutis is a proliferation of leukemic cells in the skin that occurs in 25-64% of patients with neonatal acute leukemia. Case presentation: A baby girl was born with characteristic and widespread skin lesions on the face in the form of erythematous patches partially violaceus/purplish in colour, multiple, well-defined, irregular edges, varying in shape and size, petechiae on the face and body that do not disappear with pressure and there is oedema on the nose and labia majora. Laboratory results showed hyperleukocytosis and thrombocytopenia. From the results of the examination of the peripheral blood smear, it showed normochromic anisopoikilocytosis, macrocytic, burr cell (+), normoblastemia, extremely increased leukocyte count, myeloblasts (+), monoblasts (+) and from bone marrow smears, it was found that there was an increase in erythropoiesis and granulopoiesis activity, myeloblasts 45 %, monoblast 15%. We diagnosed patients with congenital leukemia with AML-M4 and leukemia cutis. Conclusion: Congenital leukemia with AML-M4 is rare in neonates. It has a typical manifestation that there is hyperleukocytosis, leukemiacutis and there is proliferation of myeloblasts and monoblasts. Congenital leukemia has a poor prognosis, namely 23% (1975-2000) and an increase of 44.2% (2001-2016).

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