Immune Thrombocytopenia: A Disease With Many Unresolved Questions

Yaman, Samet; Candır, Burcu Aslan; Bozan, Ersin; Secilmis, Sema; Başçı, Semih; Ulu, Bahar Uncu; Yiğenoğlu, Tuğçe Nur; Çakar, Merih Kızıl; Dal, Mehmet Sinan; Altuntaş, Fevzi

doi:10.5505/aot.2022.89166

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“…Corticosteroids and IVIG are accepted as the first-line treatment for AIC in patients with IEI; however, the relapse rate is higher in those patients [27, 28]. Different prognostic factors have been reported for IVIG and corticosteroids as the first-line treatment response of AIC [29, 30]. In our study, the presence of lymphoproliferation, having diseases of immune dysregulation, low WBC and neutrophil counts, and high CD3+ T-lymphocyte counts were negatively associated with better response (Table 5).…”

Section: Discussionmentioning

confidence: 60%

Autoimmune Cytopenias Are Highly Associated with Inborn Errors of Immunity and They May Be the Initial Presentations in Cases without Severe Infections

Taskin,

Topyıldız,

Edeer Karaca

et al. 2023

Int Arch Allergy Immunol

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Introduction: Inborn errors of immunity (IEIs) are inherited disorders that present with increased susceptibility to infections as well as noninfectious complications. Due to the aberrant immune functions of patients with IEI, autoimmune cytopenia (AIC) may be the initial finding, which makes diagnosis a challenge. We aimed to evaluate the clinical course, laboratory findings, and treatment response of AIC in children with IEI. Methods: Data of children with autoimmune hemolytic anemia (AIHA) and/or immune thrombocytopenic purpura (ITP) were obtained from a retrospective chart review of IEI patients diagnosed and followed in our center. Demographic and clinical features and therapeutic outcomes were evaluated. Immunologic findings were compared between patients with AIHA, ITP, and Evans syndrome (ES). The patients were also divided into two subgroups based on the presence or absence of immune dysregulation diseases (IDDs), and all data were compared between these two groups. Results: Out of 562 patients with IEI, 6% (n: 34) had AIC which were ITP (23.5%), AIHA (35.5%), and ES (41.2%). AIC was the initial finding in 50% of these 34 patients. Patients with ES had a higher mean percentage of CD8+ T lymphocytes than ITP patients (40.77 ± 20.21% vs. 22.33 ± 12.48%, p = 0.011). Patients with IDDs were more likely to develop ES (p = 0.004), lymphoproliferation (p = 0.005), and resistance to first-line therapy (p = 0.021) than other IEI groups. Conclusion: This study shows that AIC may be the initial finding of IEI, particularly when lymphoproliferation and resistance to first-line therapy co-occur. Therefore, detailed investigation should be offered to all patients to avoid diagnostic delay.

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Section: Discussionmentioning

confidence: 60%