A study of bone marrow failure syndrome in children.
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.


OBJECTIVE
To study the clinico-hematological profile and etiological factors of bone marrow failure syndrome in children.


METHODS
This prospective study was carried out in the Department of Pediatrics of a university teaching hospital over 36 months.


METHODS
Children with pancytopenia (Hb 9 /L, platelet count<100x10(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.


METHODS
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.


CONCLUSIONS
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.
Gupta
V
V
Department of Pediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India. vgupta@bhu.ac.in
Tripathi
S
S
Singh
T B
TB
Tilak
V
V
Bhatia
B D
BD
D000293:Adolescent; D000741:Anemia, Aplastic / Q000175:diagnosis / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007194:India; D008297:Male; D012959:Socioeconomic Factors