Isolated anti-Ro/SSA thrombocytopenia: a rare feature of neonatal lupus

Ayadi, I.; Hamida, Emira Ben; Boukhris, Mohamed Riadh; Bezzine, Ahlem; Chaouachi, S.; Marrakchi, Z.

doi:10.11604/pamj.2015.22.312.6839

Cited by 7 publications

(6 citation statements)

References 10 publications

(15 reference statements)

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“…Some of these factors were similar to those found in the literature. For example, two studies conducted in 2013 and 2016 reported that IUGR and septicemia were the most common causes of early-and late-onset NTCP, respectively [11,20]. Another risk factor identified in our study was oligohydramnios.…”

Section: Discussionsupporting

confidence: 53%

Accuracy of Umbilical Cord Complete Blood Count in Detecting Early-Onset Neonatal Thrombocytopenia

Al-Hindi,

Qari,

Fatani

et al. 2023

Cureus

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Background: Neonatal thrombocytopenia (NTCP) is a common hematological disorder whose platelet count falls below the normal limit of 150 x 10 9 /L. NTCP can cause late complications if left untreated. The current study aimed to evaluate the accuracy of the umbilical cord complete blood count (UC CBC) in detecting early-onset neonatal thrombocytopenia (EO-NTCP). Further, the prevalence of NTCP was also investigated.Methods: A cross-sectional study with a matched control was conducted on all newborns delivered at a tertiary care center in Jeddah, Saudi Arabia, between May 2016 and 2019. After exclusions, 40 neonates with EO-NTCP (cases) and 80 without EO-NTCP (controls) were included. The case-to-control ratio was 1:2. The results of UC CBC were compared with those of follow-up CBC, performed within 72 hours. A p-value of <0.05 was considered statistically significant. All data were analyzed using IBM SPSS version 28 for Windows (IBM Corp., Armonk, NY).Results: The prevalence of NTCP was approximately 1.02% (111/10,936). Lack of antenatal care was found in 12 (30%) neonates with EO-NTCP vs. 10 (12.5%) neonates without EO-NTCP (p = 0.02). Neonates with EO-NTCP were more likely to have experienced intrauterine growth restriction (5 (37.5%) vs. 5 (6.3%), p < 0.001) and oligohydramnios (5 (12.5%) vs. 0 (0%), p = 0.003). Neonates who developed EO-NTCP were more likely to be admitted to the NICU (34 (85%) vs. 35 (43.8%), p < 0.001) and receive antibiotics (22 (55%) vs. 25 (31.3%), p = 0.012). Also, neonates with EO-NTCP were more frequently diagnosed with neonatal sepsis (7 (17.5%) vs. 3 (3.8%), p = 0.015) and more likely to receive platelet transfusions (15 (37.5% vs. 1 (1.3%), p < 0.001). They also had a higher median length of hospital stay (13 (interquartile range (IQR) 3-28) vs. 4 (IQR 2-9) days, p = 0.006). The mortality rates of neonates with EO-NTCP and those without were 6 (15%) vs. 2 (2.5%) neonates (p = 0.016). The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of UC CBC were 62.50%, 97.50%, 20.40%, and 99.61%, respectively. Conclusion:The prevalence of EO-NTCP in King Abdulaziz Medical City is comparable to international and national figures, and it is associated with preceding maternal comorbidities, serious neonatal morbidity, and even mortality. Therefore, proper antenatal care is vital in preventing maternal and neonatal morbidities, including the risks of NTCP and its related complications. With high NPV, using UC CBC as a universal screening method could assist in safely discharging newborns. However, because of its low sensitivity, a comprehensive clinical examination with confirmatory laboratory tests are still the cornerstone in diagnosing EO-NTCP. Future trials should aim to study the cost-effectiveness of universal UC CBC and the long-term outcomes of infants diagnosed with EO-NTCP.

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Section: Discussionsupporting

confidence: 53%

Accuracy of Umbilical Cord Complete Blood Count in Detecting Early-Onset Neonatal Thrombocytopenia

Al-Hindi,

Qari,

Fatani

et al. 2023

Cureus

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“… 36 Although no studies have revealed the association of anti-SSA or anti-Ro52 with thrombocytopaenia, their potential role in the pathogenesis of thrombocytopaenia was indicated by a case report. 37 The present study found anti-SSA and anti-Ro52 were the most common autoantibodies among ENA present in patients with primary ITP and were risk factors for CTD development. Further studies focusing on the role of SSA and Ro52 in the pathogenesis of thrombocytopenia might help us have a better understanding of ITP.…”

Section: Discussionmentioning

confidence: 48%

ANA-positive primary immune thrombocytopaenia: a different clinical entity with increased risk of connective tissue diseases

et al. 2021

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ObjectivePrimary immune thrombocytopaenia (ITP) is highly heterogeneous. ANA-positive primary ITP may resemble the preclinical stage of connective tissue diseases (CTDs), but is still considered primary ITP due to a controversial CTD risk assessment in this group. The objective of this study was to clarify the risk of CTD in ANA-positive patients with primary ITP.MethodsWe performed a retrospective cohort study and a meta-analysis. 586 patients with newly diagnosed primary ITP were followed up and Cox regression analyses were used to analyse the associations of ANA positivity and other immune parameters with CTD development.ResultsThe mean follow-up time was 37 (19–56) months. ANA was positive in 21.33% (125 of 586) of patients with primary ITP in our retrospective cohort, and the overall rate of ANA positivity in the meta-analysis was 17.06% (369 of 2163). The adjusted HR for CTD in ANA-positive primary ITP was 6.15 (95% CI 2.66 to 14.23, p<0.001). Five patients in the ANA-positive group developed SLE (5 of 125, 4.0%), significantly higher than in the ANA-negative group (0 of 461, 0%). A clinical model combining ANA, anti-Sjogren’s syndrome A antibody and C3 was successfully developed to predict the risk of CTD in patients with primary ITP. Increased risk of CTD (risk ratio=12.43, 95% CI 7.91 to 19.55, p<0.00001), especially SLE (risk ratio=30.41, 95% CI 13.23 to 69.86, p<0.00001), among ANA-positive patients with primary ITP was confirmed by a meta-analysis of previous studies and the present study.ConclusionsThe findings suggest that ANA-positive primary ITP is a clinical entity distinct from other primary ITPs and is associated with increased risk of developing CTDs, especially SLE.

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“…More importantly they designated the negative correlation between the severity of the SGA and platelet count and plateletcrit, meaning the lower the birth weight centile within the SGA group, the lower the platelet values and the plateletcrit. Ayadi et al 44 and Ulusoy et al 45 conducted observational studies in the NICU and both agreed that IUGR was a major risk factor for early-onset thrombocytopenia. The same conclusion was also made by Christensen et al 46 and Vlug et al, 47 despite involving a different NICU population, Christensen et al with extremely low birth weight infants and Vlug et al with infants with polycythemia.…”

Section: Resultsmentioning

confidence: 98%

“…IGR is undeniably one of the main causes of early-onset thrombocytopenia. 6 32 33 37 44 45 46 47 The degree of growth restriction, as displayed by the weight birth centile 19 25 43 48 50 55 and Doppler findings such as UA-PI and AEDV, 59 60 61 62 63 is related to the absolute number of platelets and duration of thrombocytopenia. 48 49 The lowest levels of platelets are usually found on 4 to 5th day of life, with spontaneous resolution within the first 1 to 2 weeks of life.…”

Section: Discussionmentioning

confidence: 99%

Assessment of Hemostatic Profile in Neonates with Intrauterine Growth Restriction: A Systematic Review of Literature

Karapati

Sokou

Iliodromiti

et al. 2023

Semin Thromb Hemost

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Intrauterine growth restriction (IUGR) affects nearly 10 to 15% of pregnancies and is responsible for many short- and long-term adverse consequences, including hemostatic derangement. Both thrombotic and hemorrhagic events are described in the perinatal period in these neonates. The aim of this study was to systematically review the literature on the laboratory studies used to evaluate the hemostatic system of the IUGR small for gestational age neonate. We reviewed the current literature via PubMed and Scopus until September 2022. Following our inclusion/exclusion criteria, we finally included 60 studies in our review. Thrombocytopenia, characterized as hyporegenerative and a kinetic upshot of reduced platelet production due to in utero chronic hypoxia, was the main finding of most studies focusing on growth-restricted neonates, in most cases is mild and usually resolves spontaneously with the first 2 weeks of life. In regard to coagulation, growth-restricted newborns present with prolonged standard coagulation tests. Data regarding coagulation factors, fibrinolytic system, and anticoagulant proteins are scarce and conflicting, mainly due to confounding factors. As thromboelastography/rotational thromboelastometry (TEG/ROTEM) provides a more precise evaluation of the in vivo coagulation process compared with standard coagulation tests, its use in transfusion guidance is fundamental. Only one study regarding TEG/ROTEM was retrieved from this population, where no difference in ROTEM parameters compared with appropriate for gestational age neonates was found. Despite the laboratory aberrations, no correlation could be achieved with clinical manifestations of bleeding or thrombosis in the studies included. More studies are needed to assess hemostasis in IUGR neonates and guide targeted therapeutic interventions.

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Isolated anti-Ro/SSA thrombocytopenia: a rare feature of neonatal lupus

Cited by 7 publications

References 10 publications

Accuracy of Umbilical Cord Complete Blood Count in Detecting Early-Onset Neonatal Thrombocytopenia

Accuracy of Umbilical Cord Complete Blood Count in Detecting Early-Onset Neonatal Thrombocytopenia

ANA-positive primary immune thrombocytopaenia: a different clinical entity with increased risk of connective tissue diseases

Assessment of Hemostatic Profile in Neonates with Intrauterine Growth Restriction: A Systematic Review of Literature

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