A study of bone marrow failure syndrome in children.
Indian J Med Sci
;
2008 Jan; 62(1): 13-8
Article
in English
| IMSEAR
| ID: sea-66066
ABSTRACT
Background:
Bone marrow failure syndrome (BMFS), or aplastic anemia, includes peripheral blood single cytopenias, as well as pancytopenia due to inability of the marrow to effectively produce blood cells.Aim:
To study the clinico-hematological profile and etiological factors of bone marrow failure syndrome in children. Setting andDesign:
This prospective study was carried out in the Department of Pediatrics of a university teaching hospital over 36 months. Materials andMethods:
Children with pancytopenia (Hb 9 /L, platelet count < 100 x 10 9 /L) and bone marrow cellularity < 25% were included in the study. History of exposure to drugs, socioeconomic status, ethnicity and occupation of father were noted. Bone marrow aspiration; trephine biopsy; Ham test; viral studies for hepatitis A, B and C; and cytogenetic investigations were carried out. StatisticalAnalysis:
Relative risk was estimated by odds ratio (OR) with 95% confidence interval (CI) in matched cases and controls.Results:
Of the 53 children studied, 6 (11.3%) were diagnosed as Fanconi anemia. Two cases had features of myelodysplastic syndrome. Forty-five children were labeled as acquired aplastic anemia, of whom one had evidence of hepatitis B infection and two patients (5.8%) had paroxysmal nocturnal hemoglobinuria. Aplastic anemia was more common in children from family with lower socioeconomic status; in Muslims; and where the father's occupation was weaving, dyeing and painting. However, the number was small to make statistically significant conclusions. No correlation could be established with exposure to drugs.Conclusion:
Fanconi anemia was responsible for approximately one-tenth of the cases of bone marrow failure syndrome. Majority of the patients had acquired aplastic anemia. Hepatitis B infection was an uncommon cause of acquired aplastic anemia.
Full text:
Available
Index:
IMSEAR (South-East Asia)
Type of study:
Etiology study
/
Observational study
Language:
English
Journal:
Indian J Med Sci
Year:
2008
Type:
Article
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