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. 2025 Apr 1;64(4):2153-2161.
doi: 10.1093/rheumatology/keae414.

Severe haematological involvement in children with systemic lupus erythematosus and clinical associations

Hakan Kisaoglu  1 , Seher Sener  2 , Kaan Can Demirbas  3 , Yasemin Demir Yigit  4 , Pinar Garipcin  5 , Serkan Coşkun  6 , Hatice Melisa Kacmaz  7 , Rabia Miray Kisla Ekinci  8 , Kubra Ozturk  9 , Oya Koker  10 , Kubra Ucak  11 , Serife Tuncez  12 , Gulsah Kilbas  13 , Nihal Karacayir  14 , Gulcan Ozomay Baykal  15 , Sema Nur Taskın  16 , Burcu Bozkaya  17 , Ozge Baba  1 , Selcan Demir  18 , Ozge Basaran  2 , Sezgin Sahin  3 , Esra Baglan  12 , Nihal Sahin  11 , Vildan Gungorer  6 , Aysenur Pac Kısaarslan  5 , Betul Sozeri  15 , Selcuk Yuksel  13 , Sevcan Bakkaloglu  14 , Ayse Balat  7 , Metin Kaya Gurgoze  4 , Ozgur Kasapcopur  3 , Seza Ozen  2 , Mukaddes Kalyoncu  1
Affiliations

Severe haematological involvement in children with systemic lupus erythematosus and clinical associations

Hakan Kisaoglu et al. Rheumatology (Oxford). .

Abstract

Objectives: To investigate the severe haematological involvement in children with SLE and assess its clinical associations, treatments, outcome and damage accrual.

Methods: The medical charts of children with SLE in whom haematological involvement was observed were reviewed. Severe haematological indices were defined as autoimmune haemolytic anaemia with a haemoglobin concentration <8 g/dl, thrombocyte count <30 000/µL and neutrophil count <500/µL.

Results: Among the 224 patients included, 102 (45.5%) displayed severe indices, predominantly at the initial involvement, and most frequently as severe anaemia in 54 (24.1%) and severe thrombocytopenia in 45 (20.1%). Disease activity did not differ according to the presence of severe disease indices. In addition, the presence of severe indices at initial involvement did not affect the damage accrual. However, a higher rate of damage (51.1% vs 29.9%, P = 0.002) and steroid-induced damage (28.9% vs 8.2%, P < 0.001) was evident in patients with flares of the haematological system. Regression analysis revealed that rituximab treatment during the initial episode (OR: 4.5, P = 0.006) and the presence of anticardiolipin antibodies (OR: 2.3, P = 0.014) significantly increases the odds for haematological system flare. However, severe indices at initial involvement did not increase the odds of a haematological flare.

Conclusion: Severe haematological indices at onset are common but not related with disease outcomes. Prevention of flares is important to improve outcomes, and a more rigorous maintenance strategy would benefit most to children who display haematological indices refractory to conventional immunosuppressants and those with anti-cardiolipin antibodies.

Keywords: children; damage; flare; haematological involvement; systemic lupus erythematosus; treatment.

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