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https://doi.org/10.13112/PC.2017.27

Successful treatment with rituximab in boy with Evans syndrome - case report

Karolina Malić Tudor ; KBC Split
Srđana Čulić
Višnja Armanda
Dubravka Kuljiš


Puni tekst: hrvatski pdf 73 Kb

str. 187-190

preuzimanja: 1.081

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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

https://hrcak.srce.hr/201177

Datum izdavanja:

22.12.2017.

Podaci na drugim jezicima: hrvatski

Posjeta: 1.815 *