OBJECTIVES: To study the socio-demographic profile and clinico-hematological features of aplastic anemia in children presenting at a single institution over 5 y. METHODS: Patients below the age of 15 y presenting with features of aplastic anemia were included in the study. Epidemiology, clinico-hematological features and treatment received were recorded in all the cases. Serology for hepatitis A, B, C, E, EBV, parvovirus and HIV was carried out. Cytogenetic studies were available in approximately half of the patients. RESULTS: One hundred eighty five patients were diagnosed with aplastic anemia. Ten patients with inherited bone marrow failure syndrome (IBMFS) were excluded. Median age was 8 y (range 4-14 y) with a male to female ratio of 2.4:1. Pallor (100 %) followed by bleeding manifestations (83.8 %) and fever (73.5 %) were most common presenting symptoms. One hundred twenty patients (70 %) were classified as severe, 36 (21 %) very severe and 17 (9 %) non-severe aplastic anemia. Viral markers for parvovirus, Epstein barr virus and hepatitis were positive in 25.8 %, 20 % and 6.7 % patients respectively. Six patients had history of varicella infection in recent past (within 6 mo). Very few patients (30) could afford immunosuppressive therapy (IST) and had suboptimal response (29.7 %). CONCLUSIONS: Majority of patients had idiopathic aplastic anemia. Very severe aplastic anemia and severe aplastic anemia were frequent. Few patients received IST and had suboptimal response. There is need to establish a national registry for aplastic anemia.
OBJECTIVES: To study the socio-demographic profile and clinico-hematological features of aplastic anemia in children presenting at a single institution over 5 y. METHODS:Patients below the age of 15 y presenting with features of aplastic anemia were included in the study. Epidemiology, clinico-hematological features and treatment received were recorded in all the cases. Serology for hepatitis A, B, C, E, EBV, parvovirus and HIV was carried out. Cytogenetic studies were available in approximately half of the patients. RESULTS: One hundred eighty five patients were diagnosed with aplastic anemia. Ten patients with inherited bone marrow failure syndrome (IBMFS) were excluded. Median age was 8 y (range 4-14 y) with a male to female ratio of 2.4:1. Pallor (100 %) followed by bleeding manifestations (83.8 %) and fever (73.5 %) were most common presenting symptoms. One hundred twenty patients (70 %) were classified as severe, 36 (21 %) very severe and 17 (9 %) non-severe aplastic anemia. Viral markers for parvovirus, Epstein barr virus and hepatitis were positive in 25.8 %, 20 % and 6.7 % patients respectively. Six patients had history of varicella infection in recent past (within 6 mo). Very few patients (30) could afford immunosuppressive therapy (IST) and had suboptimal response (29.7 %). CONCLUSIONS: Majority of patients had idiopathic aplastic anemia. Very severe aplastic anemia and severe aplastic anemia were frequent. Few patients received IST and had suboptimal response. There is need to establish a national registry for aplastic anemia.
Authors: J C W Marsh; S E Ball; P Darbyshire; E C Gordon-Smith; A J Keidan; A Martin; S R McCann; J Mercieca; D Oscier; A W W Roques; J A L Yin Journal: Br J Haematol Date: 2003-12 Impact factor: 6.998
Authors: K R Muir; C E D Chilvers; C Harriss; L Coulson; M Grainge; P Darbyshire; C Geary; J Hows; J Marsh; T Rutherford; M Taylor; E C Gordon-Smith Journal: Br J Haematol Date: 2003-12 Impact factor: 6.998