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Clinical considerations of acute leukemia or transient myeloprolifo- rative disorder in Down syndrome.

Journal of the Korean Pediatric Society 1991;34(1):74-82.
Published online January 31, 1991.
Clinical considerations of acute leukemia or transient myeloprolifo- rative disorder in Down syndrome.
Eun Sil Dong, Sung Hee Jang, Hong Hoe Koo, Hye Lim Jung, Hee Young Shin, Hyo Seop Ahn
Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
Down 증후군에 동반된 급성 백혈병 및 일과성 골수증식장애에 대한 임상적 고찰
동은실, 장성희, 구홍회, 정혜림, 신희영, 안효섭
서울대학교 의과대학 소아과학교실
Received: 30 August 1990   • Accepted: 12 October 1990
Children with Down syndrome have an increased incidence of acute leukemia. Infants with Down syndrome are also at risk of developing a transient myeloproliferative disorder indistinguishable from acute nonlymphocytic leukemia (ANLL) except by its eventual clinical recovery. We observed 11 patients with acute leukemia or transient myeloproliferative disorder in Down syndrome who had admitted to the Departmetn of Pediatrics, Seoul National University Children’s Hospital, from January 72 to March ’90. There were 6 cases of acute leukemias and 5 cases of transient myelo- proliferative disorder (TMD). Seven were males and four were females. The age of onset revealed that 9 of 11 patients were under 2 years. Manifested symptoms were lethargy, pallor, purpura, respiratory difficulty, jaundice, poor oral intake, fever and abdominal distension. Hepatomegaly was seen in all cases, and splenomegaly was associated especially in acute leukemia. Lymph node enlargement was not seen in transient myeloproliferative disorder. Six of 11 cases had congenital cardiac anomalies which were 4 cases of atrial septal defect, 1 case of patent ductus arteriosus and 1 case of tricuspid regurgitation. Four of 11 cases were expired due to infection. Three cases of 5 transient myeloproliferative disorders had spontaneous remission and one of them might be transformed into acute leukemia. We concluded as follows. If there are abnormal hematologic findings in children with Down syndrome, we should examine carefully and chromosomal analysis and bone marrow examination are necessary for distinction between an acute leukemia and a transient myeloproliferative disorder. We suspect a transient myeloproliferative disorder when the age of onset is young and there is no lymph node enlargement and proportion of blasts in peripheral blood is low. Patients with transient myeloproliferative disorder in Down syndrome have spontaneous remission without treatment. If transient myeloproliferative disorder is susupected, clinical observation without chemo-therapy is very important. Because increased mortality attributed to infection, prophylactic antibiotic therapy and thorough infection control should be considered during induction chemotherapy in patients with Down syndrome and leukemia.
Key Words: Down syndrome, Acute leukemia, Transient myeloproliferative disorder

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