A Case of Congenital Leukemia with Nasal Hemorrhage and Jaundice

Zahra Khalili Matinzadeh, MD; Zohreh Kavehmanesh*, MD; Susan Amirsalari, MD; Shahla Afsharpeyman, MD Department of Pediatrics, Baqiyatallah Medical Sciences University, Tehran, IR Iran Received: Mar 01, 2009; Accepted: Mar 15, 2010 Congenital leukemia (CL) is a very rare malignancy which occurs at estimated rate of 1 per 5 million births, and accounts for less than 1% of all childhood leukemias[1,2]. Only approximately 200 patients with congenital leukemia have been reported in the literature[3]. CL has no pathognomonic findings. The proposed diagnostic criteria for CL include: I) presentation in the first 4 weeks of life; II) proliferation of immature myeloid, lymphoid or erythroid cells; III) infiltration of these cells into nonhaematopoietict issues; IV) absence of other diseases which may explain this proliferation[5]. Most CL cases have been reported as Acute Myeloid Leukemia (AML). The acute myelomonocytic (FAB M4) and acute monocytic (FAB M5) subtypes are by far the most frequent AML respectively[4]. Herein we report a case of CL with skin lesions on his abdomen who was hospitalized because of jaundice and nasal hemorrhage. After an uneventful full-term pregnancy, a boy was born with a birth weight of 3600 gr. No maternal ante partum illness was reported. At the age of 13 days he was readmitted to the hospital because of jaundice, nasal hemorrhage and respiratory distress. Physical examination at this time revealed hepatosplenomegaly with the liver and spleen palpable 5 and 3 cm below the costal margin respectively. Multiple bluish macropapules (blue berry muffin lesion) had been scattered over his abdomen since his birth. Except for the stated signs and tachypnea, other physical examinations were normal. Laboratory tests showed: white blood cell (WBC) count 59,000 cell/mm3, Prothrombin Time (PT) >50 seconds, Partial Thromboplastin Time (PTT) >120 seconds, and total serum bilirubin 17 mg/dL with direct bilirubin not exceeding more than O.5mg/dL. AlT and AST levels were 44 and 51 IU respectively. Blood cultures were negative and serology testing was not indicative of congenital infection. Lumbar puncture did not reveal meningeal involvement. Chest X-ray showed diffuse infiltration. Cerebral ultrasound was normal. The patient was hospitalized with a primary diagnosis of sepsis, and antibiotics were initiated besides supportive therapy. Gradually all blood cells decreased: WBC count 1400 cell/mm3, hemoglobin 7 g/dl and platelet 8000/mm3. Therefore packed cells, platelet, fresh frozen plasma (FFP) and granulocyte colonystimulating factor (G-CSF) transfusions were given. Despite treatment, his general condition did not improve and echymotic plaques began to expand over all the body. Bone marrow aspiration was occupied with >80% monoblast cells. A diagnosis of AML, French-American-British (FAB) type M5, was made based on monoblast cells (Fig. 1).