Authors: Y. Jabali 1; K. Zdráhalová 2; J. Starý 2; K. Bláhová 3; D. Klodová 4; Z. Černá 5; H. Hrstková 6; Z. Novák 7; D. Procházková 8; K. Toušovská 9; Písačka M. Za Pracovní Skupinu Pro Dětskou Hematologii V České Republice 10 Authors‘ workplace: Dětská klinika, Nemocnice, České Budějovice, 2II. dětská klinika 2. LF UK a FN Motol, Praha, 3I. dětská klinika 2. LF UK a FN Motol, Praha, 4Klinika dětského lékařství FNsP, Ostrava, 5Klinika chorob dětských FNsP, Plzeň, 6Dětská interní klinika, Brno, 7Dě 1 Published in: Transfuze Hematol. dnes,, 2004, No. 2, p. 41-50. Category:
Autoimmune hemolytic anemia is a rare disease in childhood. Its course is variable and unpredictable.The optimal management particularly of the chronic form of the illness is not well defined. A retrospectivestudy of cases occurring in children, excluding newborns, in the Czech Republic from 1998 to 2001has been undertaken using a structured questionnaire. The aim of the study was to survey the state ofdiagnostics and management of this disease as well as to look for pertinent prognostic factors.Autoimmune hemolytic anemia was diagnosed in 32 children aged from 1.2 to 18.8 years (median: 7.6).There were 18 boys and 14 girls in this series (M:F = 1.3 : 1). Twenty-three patients had an acute formand 8 a chronic form of the disease. In 22 cases, the disease was idiopathic (primary) and in 10, it wassecondary with an associated chronic disorder involving the immune system. The acute form was veryoften associated with a recent or concomitant infection, usually of viral origin, and affected mainlyyoung children (median age: 4.6 years), with slightly more boys being afflicted (M:F = 1.3 : 1). By contrast,the chronic form was encountered rather in older children (median age: 12.6 years), equally affectingboth sexes, and was more frequently associated with a chronic underlying disease (6/8 vs 4/23) andrelatively more often presented as Evans’ syndrome (3/8 vs 3/23). Warm-reacting, cold-reacting, mixedand bithermal antibodies were found in 12, 9, 4 and 2 patients, respectively, and data on thermaloptimum and amplitude were not available in 5 cases. One patient was not treated for the anemia, 31received steroids, 8 additional therapy (of these, 4 were given immunosuppressive drugs), and 12required transfusion. None underwent splenectomy. While all children with the acute form recovered,7/8 with the chronic form have been living with evidence of disease.
Key words: autoimmune hemolytic anemia, Evans’ syndrome, children, retrospective study
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