Quantification of siderophages in bronchoalveolar fluid in transfusional and primary pulmonary hemosiderosis

Abstract

Transfusional iron overload may occur in the lungs. We hypothesized that quantitating siderophages in the bronchoalveolar fluid (BALF) of heavily transfused patients may prove to be a useful tool in determining lung iron overload in transfusion-dependent patients. The study included six patients (7-20 years) with thalassemia major (TM) who had received multiple blood transfusions, one with hereditary spherocytosis (four blood transfusions) and one with sickle cell disease (never transfused); they were compared to three children with idiopathic pulmonary hemosiderosis (IPH) (2.5-7.0 years) as positive controls. Fiberoptic bronchoscopy with bronchoalveolar lavage was performed in seven patients under general anesthesia for elective surgery and the rest were bronchoscoped electively under sedation. Spirometry was also performed in eight patients. There was no significant difference between children with TM and IPH in siderophages as percentage of total count (95% CI -31.0 to 1.5, P = 0.068). There were positive relationships between both mean serum ferritin values during the preceding year and the total number of units of transfused blood, and percent siderophage count among multiply transfused patients (P = 0.010, P = 0.052, respectively); similar findings were noted for the Golde score (P = 0.001, P = 0.031, respectively). None of the patients showed lung function impairment. In conclusion, in this small study, we found that the BALF of multiply transfused patients with benign hematological disorders contain similar numbers of siderophages to that of patients with IPH; this is strongly suggestive of secondary pulmonary hemosiderosis. The correlation between the patients' serum ferritin, and the BALF siderophages suggests that the later may serve as a marker of pulmonary iron overload in patients requiring blood transfusion and appear to be more sensitive than standard pulmonary function tests.

Iron quantification with susceptibility

“…Hemosiderin is rarely found in the healthy brain, but is especially abundant after hemorrhages, and can accumulate regionally in pathologies such as hemochromatosis, sickle cell anemia or thalassemia. Siderophages, also called heart failure cells, are typical hemosiderin‐laden alveolar macrophages which can be found in the lungs . These observations suggest that hemosiderin serves as a buffer against excess free iron.…”

HFE gene variants and iron-induced oxygen radical generation in idiopathic pulmonary fibrosis

“…Increased levels of haemosiderin iron are also found in pulmonary veno-occlusive disease, where Prussian blue stained alveolar macrophages and type II pneumocytes are seen in the absence of frank fibrosis [31], and in diffuse alveolar damage, concomitant with moderate to severe alveolar haemorrhage [32]. Finally, in some transfusion-dependent thalassaemia patients, systemic iron overload has been associated with functional and histopathological abnormalities, including bronchial iron deposition and fibrosis [33][34][35].…”

Talasemilerde Solunum Sistemi Bozuklukları

“…Talasemi hastalarında solunum fonksiyonlarını değerlendiren bir grup çalışmada ise, baskın SFT bozukluğunun küçük hava yolları obstrüksiyonu olduğu bildirilmiştir 78,[90][91][92] . Priftis ve arkadaşlarının çalışmasında yaşları 7-20 arasında değişen transfüzyon programındaki 6 talasemi majör, 4 kez transfüzyon öyküsü olan 1 herediter sferositoz ve transfüzyon öyküsü bulunmayan 1 orak hücreli anemi tanılı hastaya SFT ve fiberoptik bronkoskopi yapılmıştır.…”

“…Bu hastalardaki alveoler lavaj örneklerinin histopatolojik incelemesi sonucunda hemosiderin yüklü makrofajlar görülmüş, baskın solunum fonksiyon bozukluğu tipinin küçük hava yolları obstrüksiyonu olduğu bildirilmiştir. Bu çalışmada küçük hava yolu obstrüksiyonuna neden olan faktörün bronş epitel hücrelerinde ve müköz bezlerde demir birikimi olduğu, bunun sonucunda da bronşiollerde obstrüksiyon ve yamalı tarzda inflamasyon geliştiği belirtilmiştir 92 .…”