Page 106 - Abstract Book KONIKA 18

Allergy Immunology

P-AI-007
Secondary Evans Syndrome in Children with Systemic Lupus Erythematosus:
A Case Report

Friando Glorio Boneka, Raynald Oktafianus Takumansang, Diana Devi Sondakh
Department of Child Health, Medical Faculty Universitas Sam Ratulangi/Prof. DR. Dr. Kandou General Hospital,
Manado, North Sulawesi, Indonesia

Abstract
Background Evans syndrome is an autoimmune condition that consists of immune thrombocytopenia and
autoimmune haemolytic anaemia (AIHA). Primary ES is a diagnosis of exclusion and secondary ES is
related to various immunodeficiency and autoimmune diseases, such as systemic lupus erythematosus (SLE).
Although anemia and thrombocytopenia are common features of SLE, ES seems to be a rare manifestation
in SLE (1.7-2.7%). Objective To present a case report of secondary ES in SLE to emphasize the importance
of further evaluation in children presenting with ITP symptoms, as it may be a part of other underlying
diseases. Case A 12-year-old girl presented with recurrent nose bleeding accompanied with red spots on
her extremities for 2 weeks. The patient has similar symptoms on July 2020 and was diagnosed as ITP.
Physical examination showed a pale appeared child with anemic conjunctiva, and petechiae all over her arms,
trunks, and legs. Chest radiograph was unremarkable. Laboratory result showed severe anaemia and marked
thrombocytopenia, with positive Coombs’s and anti-dsDNA test. SLE criteria based on 2019 EULAR/ACR
classification was fulfilled with total score of 14. The patient was diagnosed as SLE with secondary Evans
syndrome, received packed red cell and platelet transfusion, and treated with oral prednisone. Her condition
improved after 1 week of treatment, and subsequently discharged with continued therapy. Conclusion
Identifying secondary Evans syndrome as associated with other disease such as SLE is important because
because treatment and responses considerably differ between primary and secondary ES, where secondary
ES responds better than primary ES.
Keywords: SLE; Evans syndrome; ITP; AIHA

P-AI-008
Refractory Anaphylaxis in A-16 year-old Girl: A Rare Case

Maria, Mahrani Lubis, Lily Irsa, Rita Evalina
Department of Child Health, Faculty of Medicine, Universitas Sumatera Utara/Haji Adam Malik General Hospital,
Medan, North Sumatera, Indonesia

Abstract
Background Refractory anaphylaxis means an anaphylaxis that unresponsive to the treatment with at least
two doses of a minimum 300 micrograms adrenaline (with unresponsiveness being defined as “a lack of
expected normalization of clinical symptoms”). The incidence is about 3% to 5% of anaphylaxis cases.
Objective To report a case of refractory anaphylaxis in a-16 years old-girl caused by multiple allergens. Case
A-16 years old-girl was referred to Haji Adam Malik Hospital with dyspnea since the morning, angioedema
on lips, eyelids, arms, urticaria on face, arms and leg. She was admitted in PICU with recurrent apnea (4
times), and persistent abdominal pain. Initially, we gave epinephrine 0.01 mg/kg intramuscularly twice but
apnea and abdominal pain persisted. Then, we gave epinephrine 1 mg epinephrine 1: 1000 in 1000 ml sodium
chloride 0.9% continuously, started at 2 mcg/minute (2 ml/minute), and increased gradually to 6 mcg/minute.
Diphenhydramine, methylprednisolone, and ranitidine were given as adjunctive treatment. She had never
got apnea and abdominal pain, but angioedema still persisted. IgE RAST showed sensitization to various
allergens (shellfish, bermuda grass, timothy grass, grass mix, honey bee venom, almond, garlic, acacia, latex,
wheat flour, crab, tomato, strawberry, oil palm, dermatophagoides microceras, kapok, cockroach German,
candida albicans, peanut, hazelnut). She got improvement after we eliminated all allergens. Conclusion
Epinephrine continuously infusion may help patient with refractory anaphylaxis. Elimination of allergens
has an important role in the treatment of anaphylaxis.
Keywords: refractory anaphylaxis; epinephrine; angioedema

58 KONIKA XVIII Abstract Book