Transient erythoblastopenia
in coeliac disease
Graziella Guariso, Chiara Messina, Maria Vittoria Gazzola, Silvia
Chiarelli*
Departments of Pediatrics and *Pathology, University of Padova,
Italy
Correspondence: Graziella Guariso, Departments of Pediatrics,
University of Padova, Italy. E-mail: guariso@pediatria.unipd.it
Text
Transient erythroblastopenia has been described by Wranne and Lovric as a distinct entity affecting children aged 1-4 years associated with prodromal symptoms. Granulocytopenia and thrombocytosis are frequently observed.1 The cause of TEC is unknown. A seasonal predominance, previous viruses infection and a genetic predisposition has been postulated.2 Between January 1995 and December 1998, 3 children (2 females, 1 male, median age 24 months) were admitted to our Department for anaemia. No drug intake was reported. Clinical and haematological data are summarised in the table. Blood loss or haemolysis, presence of congenital or renal abnormalities were investigated in all patients. Serological and molecular markers for EBV, CMV, ParvoB19, HHV6 were negative both in bone marrow and peripheral blood. Bone marrow morphological study revealed red blood cell aplasia and maturation arrest of the white series. No cytogenetic abnormalities were found. Since mild intestinal alterations were present, anti-endomysium antibodies were performed and were positives in all cases. Intestinal biopsies showed specific features of active CD. In all patients there was a spontaneous and rapid recovery from TEC before starting a gluten-free diet. All patients expressed HLA-DQ2 (cases #2 and 3 were homozygous for this antigen) strongly associated with CD; moreover, they were all found positive for HLA-A2. Case #1 was also A2 DQ1 positive; this is quite a common HLA typing found in the Italian population, that some authors believe to be correlated with TEC.2 Coeliac disease is considered an abnormal immunological response to gluten in genetically predisposed people, leading to characteristic enteropathy. Moreover, the association of CD with autoimmune diseases is object of specific interest and has already been reported.3 The first case of TEC in association with gluten intolerance was described by Skenner and Wranne1 among their large study on 53 cases of TEC, observed over 3 years. Other 2 cases were observed in Italy by Dufour et al.4 and another one by la Placa.5 Dufour performed marrow cell cultures, demonstrating reduced number of erythroid colonies (BFU-E, CFU-E). Colonies formation was increased by patient's T-lymphocyte depletion (4). Unfortunately, in our experience colonies were performed only at the second BM aspirate. Normal value for BFUE and CFU-GM confirmed the rapid recovery from the transient erythroblastopenia. The folate deficiency found in all our patients seems to be little unrelated with the anaemia, since we observed reticulocytosis and increased HB values before folate supplementation. The association CD/TEC was found in these 3 cases reported here, which represent all patients with TEC observed at our Department; while in the same period, from 1995 to 1998, 70 cases of CD were diagnosed. TEC was associated to CD in 4.2% of the cases therefore. Due to the high frequency of CD in the Italian population6 we cannot exclude that this combination of pathological events might well only be a coincidence. Nevertheless our experience suggest that screening for CD should be done not only in case of iron, vitamin B12 or folate deficiency, but also in other haematological disorders. Antibodies screening for CD in patients with present or past TEC might contribute to understand better both diseases and help to establish the real frequency of this probable association.
