RITUXIMAB IN AUTOIMMUNE HEMOLYTIC ANEMIA OF INFANCY
Author(s): ,
Marina Economou
Affiliations:
Aristotle Universtity of Thessaloniki,Thessaloniki,Greece
,
Aikaterini Teli
Affiliations:
Aristotle Universtity of Thessaloniki,Thessaloniki,Greece
,
Despina Adamidou
Affiliations:
Blood Bank,Hippokration General Hospital,Thessaloniki,Greece
,
Anna Taparkou
Affiliations:
Aristotle Universtity of Thessaloniki,Thessaloniki,Greece
Evaggelia Farmaki
Affiliations:
Aristotle Universtity of Thessaloniki,Thessaloniki,Greece
(Abstract release date: 05/18/17) EHA Library. Economou M. 05/18/17; 181199; E1423
Assoc. Prof. Marina-Kelly Economou
Assoc. Prof. Marina-Kelly Economou
Contributions
Abstract

Abstract: E1423

Type: Eposter Presentation

Background

Autoimmune hemolytic anemia (AIHA) is not commonly seen during childhood, and is extremely rare in infancy. Absence of guidelines renders management of the disease difficult in children – and even more so in infants.

Aims

Aim of the report is to present a number of cases of infantile AIHA, refractory to conventional treatments, demonstrating response in administration of rituximab.

Methods

The report concerns four infants (3 baby girls and one baby boy) who presented with AIHA. Data regarding demographics, personal and family medical history, immunologic assessments, previous treatments and response to rituximab were studied.

Results

Age at diagnosis of AIHA was 4-6 months. In 3 cases (cases number 1, 2 and 3) personal and family history, as well as laboratory screening at diagnosis, did not reveal presence of any other hematologic, autoimmune or immunologic condition. In case number 4 AIHA followed the diagnosis of giant cell hepatitis. Hospitalization before rituximab administration ranged between 1 and 4 months, and included multiple transfusions, administrations of intravenous immunoglobulin (maximum dose 6g/kg), repeated doses of intravenous methyl-prednisolone (30mg/kg) followed by oral prednizolone (max 5mg/kg), all failing to achieve sustained response.
Rituximab was administered at 375mg/m2 in 4 weekly infusions. In 3 infants 5 monthly infusions followed. Stabilization of hemoglobin and improvement of hemolysis parameters were observed after the 3rd-4th weekly infusion in all infants.
In 3 patients (no 1,2,3) CD19+ and CD20+ Β cell assessment before and after rituximab administration was performed. Complete elimination (<1%) was observed in all patients after the 1st-2nd infusion. Despite B cells returning to normal 11 months after treatment, infant no 1 remained in clinical remission during follow-up (22 months post treatment). Infant no 2 remained in clinical remission for the 16 month post treatment follow-up, despite B cell normalization. Infant no 3 relapsed following B cell normalization, 11 months after rituximab administration. Infant no 4 did not undergo B cell measurements and relapsed one year after completing rituximab therapy. The 2 patients that relapsed were re-treated with 4 rituximab infusions: patient no 3 remained well for the 18 month follow-up, whereas patient no 4 remained well for 10 years – again relapsing and receiving her 3rd rituximab treatment with good response for the remaining 7 month follow-up.
None of the patients presented with adverse reactions during the infusions or with severe infections as a result of immunosuppression. However, infant no 1 developed asymptomatic progressive IgG hypogammaglobulinemia 11 months after initial exposure to rituximab, eventually requiring IVIG administration

Conclusion

Rituximab administration in refractory AIHA seems to be efficacious and safe in infants. However, close follow-up is warranted in order to ensure absence of long term complications, including the risk of post-treatment hypogammaglobulinemia, when the drug is administered at such young ages.

Session topic: 31. Other Non-malignant hematopoietic disorders

Keyword(s): Infant, Autoimmune hemolytic anemia (AIHA)

Abstract: E1423

Type: Eposter Presentation

Background

Autoimmune hemolytic anemia (AIHA) is not commonly seen during childhood, and is extremely rare in infancy. Absence of guidelines renders management of the disease difficult in children – and even more so in infants.

Aims

Aim of the report is to present a number of cases of infantile AIHA, refractory to conventional treatments, demonstrating response in administration of rituximab.

Methods

The report concerns four infants (3 baby girls and one baby boy) who presented with AIHA. Data regarding demographics, personal and family medical history, immunologic assessments, previous treatments and response to rituximab were studied.

Results

Age at diagnosis of AIHA was 4-6 months. In 3 cases (cases number 1, 2 and 3) personal and family history, as well as laboratory screening at diagnosis, did not reveal presence of any other hematologic, autoimmune or immunologic condition. In case number 4 AIHA followed the diagnosis of giant cell hepatitis. Hospitalization before rituximab administration ranged between 1 and 4 months, and included multiple transfusions, administrations of intravenous immunoglobulin (maximum dose 6g/kg), repeated doses of intravenous methyl-prednisolone (30mg/kg) followed by oral prednizolone (max 5mg/kg), all failing to achieve sustained response.
Rituximab was administered at 375mg/m2 in 4 weekly infusions. In 3 infants 5 monthly infusions followed. Stabilization of hemoglobin and improvement of hemolysis parameters were observed after the 3rd-4th weekly infusion in all infants.
In 3 patients (no 1,2,3) CD19+ and CD20+ Β cell assessment before and after rituximab administration was performed. Complete elimination (<1%) was observed in all patients after the 1st-2nd infusion. Despite B cells returning to normal 11 months after treatment, infant no 1 remained in clinical remission during follow-up (22 months post treatment). Infant no 2 remained in clinical remission for the 16 month post treatment follow-up, despite B cell normalization. Infant no 3 relapsed following B cell normalization, 11 months after rituximab administration. Infant no 4 did not undergo B cell measurements and relapsed one year after completing rituximab therapy. The 2 patients that relapsed were re-treated with 4 rituximab infusions: patient no 3 remained well for the 18 month follow-up, whereas patient no 4 remained well for 10 years – again relapsing and receiving her 3rd rituximab treatment with good response for the remaining 7 month follow-up.
None of the patients presented with adverse reactions during the infusions or with severe infections as a result of immunosuppression. However, infant no 1 developed asymptomatic progressive IgG hypogammaglobulinemia 11 months after initial exposure to rituximab, eventually requiring IVIG administration

Conclusion

Rituximab administration in refractory AIHA seems to be efficacious and safe in infants. However, close follow-up is warranted in order to ensure absence of long term complications, including the risk of post-treatment hypogammaglobulinemia, when the drug is administered at such young ages.

Session topic: 31. Other Non-malignant hematopoietic disorders

Keyword(s): Infant, Autoimmune hemolytic anemia (AIHA)

By clicking “Accept Terms & all Cookies” or by continuing to browse, you agree to the storing of third-party cookies on your device to enhance your user experience and agree to the user terms and conditions of this learning management system (LMS).

Cookie Settings
Accept Terms & all Cookies