All issues > Volume 34(1); 1991

Original Article

J Korean Pediatr Soc. 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 Dong¹, Sung Hee Jang¹, Hong Hoe Koo¹, Hye Lim Jung¹, Hee Young Shin¹, Hyo Seop Ahn¹

¹Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea

Received: August 30, 1990;  Accepted: October 12, 1990.

Abstract

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.

Keywords :Down syndrome;Acute leukemia;Transient myeloproliferative disorder