Hematological Manifestations in Pediatric Systemic Lupus Erythematosus (SLE) Patients and Their Association with Disease Activity

Hematological involvement is frequent in pediatric SLE patients, and common manifestations include anemia, leukopenia, lymphopenia, and thrombocytopenia. An association has been observed between various hematological parameters and pSLE disease activity. The study aims to compare the hematological manifestations in pSLE patients with healthy controls and evaluate the association of various hematological parameters with SLE disease activity.

This cross-sectional study was conducted at the Pediatric Rheumatology Division, Department of Pediatrics, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh. This study included thirty newly diagnosed pSLE patients who met the ACR revised classification criteria and thirty healthy controls. Detailed history, physical examinations, baseline investigations, reticulocyte count, and Coombs’ test were recorded. Disease activity was assessed by using the SLE Disease Activity Index (SLEDAI).

All the pSLE cases (100.0%) had anemia followed by leukopenia, thrombocytopenia, and lymphopenia. Compared to controls, pSLE cases had significantly lower hemoglobin level, total white cell count, and platelet count. Hematological involvement was present in 100% of cases, followed by constitutional symptoms, mucocutaneous, musculoskeletal, renal, and neurologic involvement. Autoimmune hemolytic anemia was found in 10% of cases in this cohort. The majority (63.3%) of patients had severe disease activity (SLEDAI: >12). There is a significant negative correlation between hemoglobin (Hb) levels and SLEDAI scores in pSLE patients.

Hematological involvement was the most common finding in this cohort, which included anemia, leukopenia, thrombocytopenia, and lymphopenia. These frequencies align with some studies (e.g., KSA thrombocytopenia) but differ from some others (e.g., KSA leukopenia 30%). Fifty percent had a positive Coombs test, consistent with a Bangladeshi study, although lower than the Turkish (76.6%) study. True AIHA was found in 10%, similar to Brazilian (14%) and other Bangladeshi studies (10-16%). pSLE patients had significantly lower Hemoglobin (Hb), Total white blood cell, and Platelet counts compared to healthy controls, consistent with previous literature. The majority of pSLE patients (63.3%) had severe disease activity, with a mean SLEDAI score of 17.1±6.75, similar to an Indonesian study but lower than the mean score in an Egyptian study. Significant negative correlation was found between hemoglobin level and SLEDAI score in pSLE patients, but no significant correlation was found between white blood cell count, platelet count, and SLEDAI score, which agrees with the studies of Samohvalov, Ahmed, and Shamim but does not match the findings of Maher. The reason behind the discrepancies in hematological parameter associations across studies is the wide spectrum of presentation, non-specific symptoms, and the multifactorial origin of cytopenias.

Anemia was the most common hematological abnormality in this cohort. Autoimmune hemolytic anemia was found in 10% of pSLE patients, and hemoglobin level showed a negative association with SLEDAI. Hematological parameters of pSLE patients were significantly reduced compared to healthy controls.