OBJECTIVE: To determine the clinicohaematological features, treatment and outcome of @entity1 diagnosed with @entity2150 at a single institution. STUDY DESIGN: Observational study. PLACE AND DURATION OF STUDY: The Aga Khan University Hospital, Karachi, from January 1999 till December 2008. METHODOLOGY: Medical records of @entity1 aged less than 15 years of age diagnosed with @entity2150 were reviewed. Clinicohaematological features, treatment and its response to therapy and outcome were recorded. Results were described in percentages. RESULTS: Ninety @entity1 were diagnosed to have @entity2150 ( @entity2150 ); 65 were male during the study period. Age ranged from 1 to 15 years. Fever in 65 @entity1 (72.2%), pallor in 53 (58.8%), @entity548 in 49 (54.4%) and @entity1415 in 31(34.4%) were the most common and frequent presenting features. @entity11807 was found in 15 (16.6%) and acquired idiopathic in 75 (83.4%) of @entity1 . Very severe @entity2150 (VSAA) was seen in 29 (32.2%), 26 (28.9%) had severe @entity2150 and 17 (18.9%) had moderate @entity2150 . Eight @entity1 (8.9%) underwent haematopoietic stem cell transplantation (HSCT), 12 (13.3%) received immunosuppressive therapy (IST) and 70 @entity1 (77.7%) received other and supportive therapy. Five (62.5%) @entity1 showed complete response to HSCT and 3 (37.5%) failed to engraft. IST showed complete response in 3 (25%), partial response in 5 (41.6%) and no response in 4 (33.3%). Twenty two @entity1 (24.4%) expired either due to @entity281 in 16 (72.7%, fungal in 6, bacterial in 10) and @entity932 in 6 (27.3%) cases. CONCLUSION: Majority of cases with @entity2150 were acquired and idiopathic in etiology. VSAA and @entity5475 were frequent. Response to HSCT and IST was sub-optimal.

XXXX : clinicohaematological features, treatment and outcome analysis.

@entity1 :: ('9606', 'Species') :: ['children', 'patients'] @entity932 :: ('MESH:D020300', 'Disease') :: ['intracranial haemorrhage'] @entity548 :: ('MESH:D006470', 'Disease') :: ['skin bleeding'] @entity1415 :: ('MESH:D004844', 'Disease') :: ['epistaxis'] @entity2150 :: ('MESH:D000741', 'Disease') :: ['aplastic anemia', 'AA'] @entity281 :: ('MESH:D007239', 'Disease') :: ['infection'] @entity5475 :: ('6287', 'Gene') :: ['SAA'] @entity11807 :: ('MESH:D005199', 'Disease') :: ["Congenital (Fanconi's) anemia"]
@entity2150 :: (MESH:D000741,Disease) :: ['Aplastic anemia']
