How We Use Reticulocyte Parameters in Workup and Management of Pediatric Hematologic Diseases

Parodi, Emilia; Romano, Francesca; Ramenghi, Ugo

doi:10.3389/fped.2020.588617

Cited by 5 publications

(7 citation statements)

References 18 publications

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“…The reticulocyte count distinguishes disorders resulting from the rapid destruction or loss of RBCs caused by bone marrow failure, high being considered as hemolysis and low as bone marrow depression. 34 Being the key HLH feature, histiocytes or macrophages (hemophagocytes) engulfing RBCs cause hemolysis and severe anemia, while markedly increased reticulocyte count was also found in some studies 35,36 just as in the present case, which suggested that reticulocytosis might be a HLH characteristic.…”

supporting

confidence: 68%

Reactive Hemophagocytic Lymphohistiocytosis Secondary to Ovarian Adenocarcinoma: A Rare Case Report

Li¹,

Zhu²,

Li³

et al. 2022

JIR

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Background Hemophagocytic lymphohistiocytosis (HLH), a syndrome of immune hyperactivation and abnormal regulation that causes life-threatening inflammation, is mainly characterized by fever, hepatosplenomegaly, cytopenia, and other symptoms. Reactive HLH (rHLH) is typically secondary to immune deregulation caused by underlying rheumatologic, infectious, or malignant conditions. Malignancy-associated HLH (M-HLH) continues to be a critical health problem worldwide. Most malignancies associated with HLH are hematologic tumors, and M-HLH in non-hematologic tumors very rarely occurs. Case Report A 34-year-old Chinese woman had a history of persistent fever, acute dizziness, and bicytopenia. She was found to have developed bilateral ovarian cancer. Additional tests showed splenomegaly, hemophagocytes in the bone marrow, low natural killer activity, and hyperferritinemia, which met the diagnostic criteria put forth in the Histiocyte Society HLH-2004. The patient was treated with correcting anemia, increased platelets, and glucocorticoid therapy but showed no response. She progressively deteriorated and died 55 days later. Conclusion Hemophagocytic lymphohistiocytosis related to a solid tumor is extremely rare. To the best of the authors’ knowledge, the present case was the first to report rHLH secondary to ovarian adenocarcinoma. It is very significant for a better understanding of the disease mechanisms of HLH and should attract the attention of hematologists and other clinicians as the condition progresses and the cost of treating it increases.

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supporting

confidence: 68%

Reactive Hemophagocytic Lymphohistiocytosis Secondary to Ovarian Adenocarcinoma: A Rare Case Report

Li¹,

Zhu²,

Li³

et al. 2022

JIR

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“…They are released into the bloodstream in response to G6PD deficiency-induced hemolytic anemia [ 24 ]. Moreover, the reticulocyte count is higher at birth than at any other point in a healthy life [ 25 ]. This led us to investigate the effect of reticulocytosis on G6PD activity in Thai newborns using the automated platform.…”

Section: Discussionmentioning

confidence: 99%

Effect of neonatal reticulocytosis on glucose 6-phosphate dehydrogenase (G6PD) activity and G6PD deficiency detection: a cross-sectional study

et al. 2022

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Background Screening for G6PD deficiency in newborns can help prevent severe hemolysis, hyperbilirubinemia, and bilirubin encephalopathy, as recommended by the World Health Organization (WHO). It has been speculated that the presence of a high number of reticulocytes in newborns interferes with the diagnosis of G6PD deficiency since reticulocytes contain higher amounts of G6PD enzyme than mature erythrocytes. Therefore, the purposes of this study were to assess the effect of reticulocytosis in the determination of blood G6PD activity in Thai newborns by using a novel automated UV-based enzymatic assay and to validate the performance of this assay for the detection of G6PD deficiency in newborn samples. Methods The levels of reticulocytes and G6PD activity were measured in blood samples collected from 1,015 newborns. G6PD mutations were identified using TaqMan® SNP genotyping assay, PCR–restriction fragment length polymorphism (PCR–RFLP), and direct sequencing. The correlation between the levels of reticulocytes and G6PD activity was examined. The performance of the automated method was compared with that of the fluorescent spot test (FST) and the standard quantitative assay. Results The automated assay detected G6PD deficiency in 6.5% of the total newborn subjects compared to 5.3% and 6.1% by the FST and the standard method, respectively. The minor allele frequencies (MAFs) of G6PD ViangchanG871A, G6PD MahidolG487A, and G6PD UnionC1360T were 0.066, 0.005, and 0.005, respectively. The reticulocyte counts in newborns with G6PD deficiency were significantly higher than those in normal male newborns (p < 0.001). Compared with normal newborns after controlling for thalassemias and hemoglobinopathies, G6PD-deficient patients with the G6PD ViangchanG871A mutation exhibited elevated reticulocyte counts (5.82 ± 1.73%, p < 0.001). In a group of G6PD normal newborns, the percentage of reticulocytes was positively correlated with G6PD activity (r = 0.327, p < 0.001). However, there was no correlation between G6PD activity and the levels of reticulocytes in subjects with G6PD deficiency (r = -0.019, p = 0.881). The level of agreement in the detection of G6PD deficiency was 0.999, while the area under the receiver operating characteristic (AUC) curve demonstrated that the automated method had 98.4% sensitivity, 99.5% specificity, 92.4% positive predictive value (PPV), 99.9% negative predictive value (NPV), and 99.4% accuracy. Conclusions We report that reticulocytosis does not have a statistically significant effect on the detection of G6PD deficiency in newborns by both qualitative and quantitative methods.

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“…(94,95) The absolute reticulocyte count (ARC), defined as the number of reticulocytes/µL, reflects bone marrow function and effective erythropoiesis. (106) On the day of birth, the reticulocyte count is higher than at any time during healthy life, caused by a relatively high blood concentration of erythropoietin before and at birth. (107) The reticulocyte count is essential for the postnatal diagnosis of TAPS, but in recent review articles, there was no consensus about the units or values that should be used.…”

Section: Discussionmentioning

confidence: 99%

“…(28,75,109) A normal ARC both at pediatric (after the first 3 months of life) and adult ages is ∼25,000 to 75,000/µL (1.0 ± 0.5 percent of the 5 million red cells/µL) and is calculated and reported by many automated cell counters even though there are no standardized cut offs in the newborn. (106) Some authors that tried to study reference intervals for reticulocyte parameters in newborns provided information predominantly from term and late-preterm infants ( ⩾34 weeks gestation), mainly from the first week or two after birth; therefore, robust reticulocyte parameters remain largely undetermined for early-gestation preterm neonates. (107) Furthermore, it is not known how reticulocyte parameters of neonates are affected by conditions such as iron deficiency or hemolytic disorders.…”

Section: Discussionmentioning

confidence: 99%

Twin Anemia-Polycythemia Sequence (TAPS): From Basic Research to Clinical Practice

Rocha

Guedes-Martins

Cunha

2023

CVP

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Twin pregnancy is associated with an increased risk of perinatal and maternal complications, and early establishment of the chorionicity type defines this risk. In monochorionic (MC) pregnancies, the fetuses share the same placental mass and exhibit vascular anastomoses crossing the intertwin membrane, and the combination and pattern of anastomoses determine the primary clinical picture and occurrence of future complications. Twin Anemia-Polycythemia Sequence (TAPS) was first described in 2006 after fetoscopic laser surgery in twin-to-twin transfusion syndrome (TTTS) twins, and in 2007, the first spontaneous cases were reported, recognizing TAPS as an individualized vascular identity in fetofetal transfusion syndromes. There are two types of TAPS: spontaneous (3-5%) and iatrogenic or postlaser (2-16%). TAPS consists of small diameter arteriovenous anastomoses (<1 mm) and low-rate, small-caliber AA anastomoses in the absence of amniotic fluid discordances. There are certain antenatal and postnatal diagnostic criteria, which have progressively evolved over time. New, additional secondary markers have been proposed, and their reliability is being studied. The best screening protocol for TAPS in MC twins is still a matter of debate. This review provides a survey of the relevant literature on the epidemiology, vascular pathophysiology, underlying hemodynamic factors that regulate mismatched vascular connections, and diagnostic criteria of this condition. The aim is to increase awareness and knowledge about this recently identified and frequently unrecognized and misdiagnosed pathology.

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How We Use Reticulocyte Parameters in Workup and Management of Pediatric Hematologic Diseases

Cited by 5 publications

References 18 publications

Reactive Hemophagocytic Lymphohistiocytosis Secondary to Ovarian Adenocarcinoma: A Rare Case Report

Reactive Hemophagocytic Lymphohistiocytosis Secondary to Ovarian Adenocarcinoma: A Rare Case Report

Effect of neonatal reticulocytosis on glucose 6-phosphate dehydrogenase (G6PD) activity and G6PD deficiency detection: a cross-sectional study

Twin Anemia-Polycythemia Sequence (TAPS): From Basic Research to Clinical Practice

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