Kurzweil P R, Miller D R, Freeman J E, Reiman R E, Mayer K
Am J Dis Child. 1984 Aug;138(8):746-8. doi: 10.1001/archpedi.1984.02140460038012.
The diagnosis of idiopathic pulmonary hemosiderosis (IPH) may be elusive. A 6-year-old boy had microcytic hypochromic anemia and a hemolytic component. Hemosiderin-laden macrophages were not found in the gastric aspirate. He had no pulmonary signs or symptoms. Extensive hematologic and roentgenologic investigations failed to reveal the cause of the anemia. Quantitative serial scintigraphic scanning showed significant (35%) pulmonary sequestration of autologous erythrocytes labeled with sodium chromate Cr51. The half-life of the RBCs was moderately decreased (half-life, 15 days; normal, 25 to 35 days). An open-lung biopsy specimen confirmed the diagnosis of IPH. A diagnosis of IPH should be considered when children have iron deficiency anemia and pulmonary signs or symptoms. Organ sequestration studies may be helpful in equivocal cases.
特发性肺含铁血黄素沉着症(IPH)的诊断可能具有挑战性。一名6岁男孩患有小细胞低色素性贫血和溶血成分。胃抽吸物中未发现含铁血黄素巨噬细胞。他没有肺部体征或症状。广泛的血液学和放射学检查未能揭示贫血的原因。定量连续闪烁扫描显示,用铬酸钠Cr51标记的自体红细胞在肺部有显著(35%)的滞留。红细胞半衰期中度缩短(半衰期为15天;正常为25至35天)。开胸肺活检标本确诊为IPH。当儿童出现缺铁性贫血和肺部体征或症状时,应考虑IPH的诊断。器官滞留研究可能有助于诊断不明确的病例。