Paediatria Croatica, Vol. 61 No. 4, 2017.
Stručni rad
https://doi.org/10.13112/PC.2017.27
Successful treatment with rituximab in boy with Evans syndrome - case report
Karolina Malić Tudor
orcid.org/0000-0002-4951-0592
; KBC Split
Srđana Čulić
Višnja Armanda
Dubravka Kuljiš
Sažetak
Autoimmune haemolytic anaemia (AIHA) is caused by production of antibodies against antigens on the surface of the red blood
cells. It can be classifi ed as haemolytic anaemia due to warm and cold reactive antibodies and divided into primary and secondary
AIHA (associated with an underlying disease: systemic lupus erythematodes, immunodefi ciency, lymphoproliferative disorders). The
main feature of this anaemia is positive direct Coombs test which demonstrates antibodies of IgG class and complement (mostly
anti-C3) on the surface of the red blood cells. Evans syndrome is the presence of simultaneous or sequential direct Coombs-positive
AIHA in conjunction with immune thrombocytopenia (ITP). We report on a 2-year-old boy with diagnosed AIHA at age of 10 months
with a repetitive course. At age of 17 months concomitant ITP occurs and diagnosis of Evans syndrome was made.
The patient was treated with glucocorticoids, human immunoglobulins, red blood cells and platelet transfusions many times, without
the stabile remission. Finally, treatment with rituximab was followed by clinical improvement and disease remission lasting for months.
Ključne riječi
autoimmune haemolytic anaemia; immune thrombocytopenia; Evans syndrome; rituximab
Hrčak ID:
201177
URI
Datum izdavanja:
22.12.2017.
Posjeta: 2.635 *