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.

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

SETTING AND DESIGN: This prospective study was carried out in the Department of Pediatrics of a university teaching hospital over 36 months.

MATERIALS AND 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.

STATISTICAL ANALYSIS: 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.