Clinicopathological features of transient myeloproliferative syndrome and congenital leukaemia

The objectives of the study were to determine the spectrum of the clinical and pathological findings, the management and prognosis of patients of transient myeloproliferative syndrome [TMS] and congenital leukaemia. Case series. The study was conducted over a period of 8 years, from January 2000 to December 2007, at the Children's Hospital and the Institute of Child Health, Lahore. Suspected patients presenting with fever, pallor, bruises and hepatosplenomegaly and diagnosed as either transient myeloproliferative disorder or congenital leukaemia were studied. The complete blood count, reticulocyte count, leukocyte alkaline phosphatase score, liver function tests, karyotyping studies and bone marrow aspiration biopsy were performed in all of those patients. Management and outcome was noted. Results were described as frequency percentages. Out of 10,000 patients presenting during this period, 24 patients were diagnosed as either of transient myeloproliferative syndrome or congenital leukaemia. Fifteen of these were diagnosed as patients of TMS and 9 as patients of congenital leukaemia. Down syndrome [DS] was diagnosed in 75% of these patients. TMS patients were put on supportive treatment and recovered spontaneously. One DS patient with congenital leukaemia went into spontaneous remission and 2 of DS patients with congenital leukaemia responded to chemotherapy while rest of them either died or lost to follow-up. TMS and congenital leukaemia were not very uncommon in the studied population. Majority had Down syndrome. It is important to differentiate their clinical and pathological presentations for proper management. TMS may resolve with supportive treatment while congenital leukaemia is a fatal condition requiring chemotherapy

Congenital leukaemia

This is a case series of three infants who presented with pallor, bruises, and rashes in first month of their life with non specific symptomatology and were diagnosed to have congenital leukaemia. All were of acute myeloblastic leukaemia [AML] M5-FAB type

Pediatric myelodysplastic syndrome [MDS] - case series at the childrens hospital and the institute of child health, Lahore

A study was planned to determine the pattren of FAB type distribution of myelodysplastric syndrome in childhood haematological malignancies. It was a cross sectional study carried over a twenty-month period in Haematology and Transfusion Medicine Department of Children Hospital and Institute of Child Health. All patients of age suspected of haematological malignancy were included in the study. All patients underwent a complete blood count, reticulocyte count, peripheral blood film from neonate 15 years examination, bone marrow aspiration and trephine biopsy. During a period of 20 months [from Sep 2000 to May 2002] 195 cases of haematological malignancies were diagnosed. Out of these 9 [4.6%] patients were diagnosed as cases of Paediatric MDS. There were 110 cases of acute lymphoplastic leukemia [ALL], 63 cases of acute myeloid leukemia [AML] and 13 cases of chronic myeloid leukemia [CML]. Out of the 9 MDS cases five were of MDS Type III - Refractory Anemia with Excess of Blasts-[RAEB] and four were of MDS Type I Refractory Anemia [RA]. It can be conducted that paediatric MDS represents 4.6% of all childhood haematological malignancies with nearly equal distribution of REAB and RA