Essential Pulmonary Haemosiderosis as an Immuno-haematological Problem

The 30 cases of essential pulmonary haemosiderosis hitherto published supply ample data to present not only the classical pattern of this disease but also its latent form. The condition mostly affects children; in adolescents (16 to 20 years of age) it was observed in two instances only (Waldenstrom, 1944; Walton and Williams, 1951) and the true nature of the adult cases reported by BorsosNachtnebel (1942) and by Ellman and Gee (1951) is still disputed. The collapse-like state repeatedly occurring at intervals of weeks or months is characteristic of the disease, whose predominant symptoms are cachexia, fatigue, pallor and cyanosis, dyspnoea and cardiac insufficiency; haemoptysis is frequent and at other times haematemesis, with abdominal complaints. The fingers may become clubbed. The skin is pale, sometimes more or less icteric, and in certain cases bluish pink. The radiological findings may-in rare instances-be entirely negative or show large foci or an intense density in both lungs, but in a considerable number of cases the change suggests miliary tuberculosis. After the attacks the intensity of radiological signs may decrease, remain unchanged or occasionally progress. In the presence of characteristic symptoms diagnosis is not difficult. The fewer the symptoms the more difficult it is to differentiate the condition from iron-deficiency or haemolytic anaemia, or the radiograph from that of miliary tuberculosis. In the latent form bronchitis alone may be the guiding symptom (Scheidegger and Dreyfus, 1945). In the initial stage recognition of such a case may be possible only by special means. As long as the disease could not be cured and was attended by a 100% mortality, early diagnosis had no particular significance. Since, however, independently of each other, in England Paterson (1946), in Portugal de Castro Freire and Cordeiro (1948; Cordeiro, 1952) and in Hungary Steiner in 1952 have attempted not only to check the progress of the disease, but also to make it regress by means of splenectomy, diagnosis in the early stage is of paramount importance.