Information from the abstract
BACKGROUND: Hereditary pyropoikilocytosis (HPP) is the most common among rare forms of severe hemolytic anemia in Thailand. Erythropoiesis and iron metabolism in patients with HPP are understudied, leading to a lack of pathology-guided transfusion and iron chelation management. METHODS: We conducted a multicenter cross-sectional study to quantify biomarkers of erythropoiesis and iron metabolism in 46 young patients with HPP and to compare these findings with those observed in 80 HbE/β-thalassemia. Clinical data were collected to identify factors associated with the severity of abnormal erythropoiesis or iron metabolism in patients with HPP. RESULTS: We identified elevated erythroferrone levels in HPP patients, supporting increased erythropoietic activity and consistent with a contribution from ineffective erythropoiesis. These levels were comparable to those observed in HbE/β-thalassemia. Patients with HPP had earlier iron overload than those with HbE/β-thalassemia (median [IQR] age 2.2 [1.9-2.9] vs. 3.4 [2.4-5.2] years, p < 0.001), and this was associated with a higher transfusion burden and a more pronounced hepcidin suppression. Among HPP patients, more severe hepcidin suppression was significantly associated with younger age at diagnosis and initiation of regular transfusion. Hepcidin suppression persisted in HPP patients with splenectomy (n = 23), and its levels were significantly lower than those of patients with intact spleen (n = 23). Additionally, liver iron concentration was strongly correlated with ferritin levels (R = 0.82) in HPP (n = 15). CONCLUSIONS: This study provides new insights into the pathological characteristics of HPP, specifically, comparable levels of erythroferrone and a greater degree of hepcidin suppression than those observed in HbE/β-thalassemia.
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Related topics: Hemoglobinopathies and Related Disorders · Iron Metabolism and Disorders · Erythrocyte Function and Pathophysiology
Thai researcher and institutional participation
Nitirat Boonyaniwas · Punnavit Khongphithakskul · Sumonmaln Klamchuen · Patcharee Komvilaisak · Pimlak Charoenkwan · Manliga Ponlakan · Praguywan Kadegasem · Tanyanee Khlangtan · Noppawan Tangbubpha · Ketsuda Kempka · Supawadee Maneekesorn · Nongnuch Sirachainan · Duantida Songdej · Mahidol University · Ramathibodi Hospital · Maharat Nakhon Ratchasima Hospital · Sappasithiprasong Hospital · Khon Kaen University · Srinagarind Hospital, Faculty of Medicine, Khon Kaen University · Chiang Mai University · Buddhachinaraj Hospital
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