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Diagnosis and management of autoimmune hemolytic anemia in children.
Weli, M; Ben Hlima, A; Belhadj, R; Maalej, B; Elleuch, A; Mekki, N; Gargouri, L; Kamoun, T; Barbouche, M-R; Mahfoudh, A.
Affiliation
  • Weli M; Department of pediatric emergency and reanimation, Hedi Chaker university hospital of Sfax, Sfax, Tunisia; Faculty of medicine, university of Sfax, Sfax, Tunisia.
  • Ben Hlima A; Department of pediatric emergency and reanimation, Hedi Chaker university hospital of Sfax, Sfax, Tunisia; Faculty of medicine, university of Sfax, Sfax, Tunisia.
  • Belhadj R; Department of pediatrics, Hedi Cheker university hospital of Sfax, Sfax, Tunisia; Faculty of medicine, university of Sfax, Sfax, Tunisia. Electronic address: rimbelhaj87@gmail.com.
  • Maalej B; Department of pediatric emergency and reanimation, Hedi Chaker university hospital of Sfax, Sfax, Tunisia; Faculty of medicine, university of Sfax, Sfax, Tunisia.
  • Elleuch A; Department of pediatric emergency and reanimation, Hedi Chaker university hospital of Sfax, Sfax, Tunisia; Faculty of medicine, university of Sfax, Sfax, Tunisia.
  • Mekki N; Laboratory of transmission, control and immunobiology of infections (LR11IPT02), Tunis, Tunisia.
  • Gargouri L; Department of pediatric emergency and reanimation, Hedi Chaker university hospital of Sfax, Sfax, Tunisia; Faculty of medicine, university of Sfax, Sfax, Tunisia.
  • Kamoun T; Department of pediatrics, Hedi Cheker university hospital of Sfax, Sfax, Tunisia; Faculty of medicine, university of Sfax, Sfax, Tunisia.
  • Barbouche MR; Laboratory of transmission, control and immunobiology of infections (LR11IPT02), Tunis, Tunisia.
  • Mahfoudh A; Department of pediatric emergency and reanimation, Hedi Chaker university hospital of Sfax, Sfax, Tunisia; Faculty of medicine, university of Sfax, Sfax, Tunisia.
Transfus Clin Biol ; 27(2): 61-64, 2020 Apr.
Article in En | MEDLINE | ID: mdl-32280062
ABSTRACT
BACKGROUND AND

AIM:

The aim of this study is to evaluate the clinical, biological and hematological profiles of autoimmune hemolytic anemia (AIHA) in children and to specify its etiologies, therapeutic modalities, and treatment responses.

METHODS:

This is a 14-year retrospective study of AIHA cases collected at the department of pediatric emergency and reanimation of Hedi Chaker University Hospital in Sfax. We included patients under 14 years old with clinical and biological features of hemolysis and a positive direct antiglobulin test (DAT). The selected patients' demographic characteristics, physical signs, laboratory findings, and treatment responses were recorded.

RESULTS:

Thirteen cases of AIHA were collected, including 8 girls and 5 boys. The median age at diagnosis was 4 years and 6 months (range 8 months to 13 years). Consanguinity was reported in 6 cases and 4 patients had a previous infection history. The onset of AIHA was progressive in 9 cases, marked by an anemic syndrome and hemolysis symptoms in 6 and 8 cases, respectively. The clinical triad (pallor, jaundice and splenomegaly) was found in only 4 cases. At the time of diagnosis, the median hemoglobin (Hb) level was 6g/dL (range 4.2 to 9.2g/dL), anemia was non-regenerative in 2 patients. Thrombocytopenia and neutropenia were noted in 5 and 1 patient, respectively. Peripheral smear examination showed spherocytosis in 2 cases. All the patients had a positive DAT. Of these, 10 were positive with IgG and 3 with both IgG and C3d. AIHA was secondary to other conditions in 9 patients infection (3 cases), autoimmune disease (4 cases), and immunodeficiency (2 cases). All the patients received first-line corticosteroid therapy but only 8 of them required blood transfusions due to severe anemia. Complete remission was obtained in 7 cases. Corticosteroid resistance and dependence were noted in 1 and 2 cases, respectively. During evolution, additional therapy was indicated in 4 patients and it included cyclosporine A, azathioprine, and mycophenolate mofetil (MMF). After a median follow-up of 4.5 years, the cure rate was 80% and only 1 patient (a boy) died due to his underlying pathology.

CONCLUSION:

Our study highlights the rarity, severity, and heterogeneity of etiological contexts of AIHA in children. The therapeutic difficulties justify specific expertise in pediatric hematology.
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Thrombocytopenia / Anemia, Hemolytic, Autoimmune / Leukopenia Type of study: Diagnostic_studies / Observational_studies Limits: Adolescent / Child / Female / Humans / Male Language: En Journal: Transfus Clin Biol Year: 2020 Document type: Article

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Thrombocytopenia / Anemia, Hemolytic, Autoimmune / Leukopenia Type of study: Diagnostic_studies / Observational_studies Limits: Adolescent / Child / Female / Humans / Male Language: En Journal: Transfus Clin Biol Year: 2020 Document type: Article