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ROENTGENOGRAM OF THE MONTH
ROE NTG ENO GRA M OF THE MON TH JOHN
H. M.
AUSTIN , M.D.*
San Francisco, California
T
iron therap y. Physical examination revealed a pale, weak, and chronically ill child. Temperatu re is 104°F and respiratory rate 60. Chest examination shows scattered rhonchi. The liver and spleen are not enlarged. The hemato crit is 27 per cent. Tuberculin skin test and bronchoscopy are negative.
H IS 16-MO NTH-O LD MEXIC AN GIRL
presented with a two-day history of fever, tachypnea, and cough produc tive of blood-tinged sputum. Iron deficiency anemia was first noted at the age of 12 month s and has persisted withou t response to oral *From the Departm ent of Radiology, San Francisco Medical Center, University of Californ ia.
••
FlOURS 1
641
Diseases of the Chest
JOHN H. M. AUSTIN
Diagnosis:
IDIOPATHIC PULMONARY
HEMOSIDEROSIS
The roentgenogram reveaJs extensive pulmonary involvement of the alveolar type with acinar nodular densities. These findings, in combination with anemia and hemoptysis, led to sputum staining for iron, which showed many macrophages loaded with hemosiderin. Pulmonary needle biopsy confirmed the diagnosis. Idiopathic pulmonary hemosiderosis is an uncommon disease classically defined as a fatal hemorrhagic disorder of children char~cterized by ~peated episodes of hemoptySIS and anemia. The onset of the disease is usually from age one to six and it is more com " girls than boys.' It also occurs . mon in In young adults and, in this age group is more common in men. The course is one of exacerbation and remission.' .Pathologically, the predominant lesion is Widespread alveolar and interstitial pulmo~ary ?emorrhage of unknown etiology.The lro,n ,IS unavailable for further hematoPOl~IS, ~nd iron deficiency anemia ensues, leading In ,~urn to constitutional symptoms, Us~ally spill-over" of red cells into alveoli occurs, with subsequent cough and
hemoptysis. The diagnosis is readily established by demonstration of hemosiderinladen macrophages in the sputum. Prognosis is poor, with death from respiratory infection or ri g h t heart failure occurring within two to six years of onset. However, the spectrum also includes patients who have had only occasional episodes, with long remissions and survival/ There is no known effective therapy. Differential diagnosis includes the secondary hemosiderosis of increased pulmonary venous pressure (as in chronic left heart failure or mitral stenosis), pneumonia, chronic leukemia,' collagen disease, Goodpasture's syndrome, lung carcinoma with recurrent hemorrhage: and pulmonary contusion secondary to chest trauma. REFERENCES
1
K, H. AND SOMMERS, S. C.: Idiopathic pulmonary hemosiderosis and related syndromes, Amer. J. Med' J 32:499, 1962. 2 BRONSON, M. S.: Idiopathic pulmonary hemosiderosis in adults, Amer. J. Roentgen 83: 260, 1960. " 3 GREENSPAN J R. H.: Chronic disseminated alveolar diseases of the lung, Seminars in Roenjgenology, 2: 77, 1967. 4 GREEN J R. A.: Nodular aspirational pulmonary hemosiderosis, Amer. J. Ro/mtgen. 92'561, 1964-. ' . BENJAMIN FELSON, M.D., Editor HAROLD SPITZJ M.D., Co-Editor SOE~GEL,
Readers . I for the Roentgenogram of the Month. Please submit a brief abstract of your caseare t invited Be' .to SUbmit artlCes o nJamm PelIOn, M.D., Department of Radiology, Cincinnati General Hospital. Cincinnati, Ohio.
H. M.
AUSTIN , M.D.*
San Francisco, California
T
iron therap y. Physical examination revealed a pale, weak, and chronically ill child. Temperatu re is 104°F and respiratory rate 60. Chest examination shows scattered rhonchi. The liver and spleen are not enlarged. The hemato crit is 27 per cent. Tuberculin skin test and bronchoscopy are negative.
H IS 16-MO NTH-O LD MEXIC AN GIRL
presented with a two-day history of fever, tachypnea, and cough produc tive of blood-tinged sputum. Iron deficiency anemia was first noted at the age of 12 month s and has persisted withou t response to oral *From the Departm ent of Radiology, San Francisco Medical Center, University of Californ ia.
••
FlOURS 1
641
Diseases of the Chest
JOHN H. M. AUSTIN
Diagnosis:
IDIOPATHIC PULMONARY
HEMOSIDEROSIS
The roentgenogram reveaJs extensive pulmonary involvement of the alveolar type with acinar nodular densities. These findings, in combination with anemia and hemoptysis, led to sputum staining for iron, which showed many macrophages loaded with hemosiderin. Pulmonary needle biopsy confirmed the diagnosis. Idiopathic pulmonary hemosiderosis is an uncommon disease classically defined as a fatal hemorrhagic disorder of children char~cterized by ~peated episodes of hemoptySIS and anemia. The onset of the disease is usually from age one to six and it is more com " girls than boys.' It also occurs . mon in In young adults and, in this age group is more common in men. The course is one of exacerbation and remission.' .Pathologically, the predominant lesion is Widespread alveolar and interstitial pulmo~ary ?emorrhage of unknown etiology.The lro,n ,IS unavailable for further hematoPOl~IS, ~nd iron deficiency anemia ensues, leading In ,~urn to constitutional symptoms, Us~ally spill-over" of red cells into alveoli occurs, with subsequent cough and
hemoptysis. The diagnosis is readily established by demonstration of hemosiderinladen macrophages in the sputum. Prognosis is poor, with death from respiratory infection or ri g h t heart failure occurring within two to six years of onset. However, the spectrum also includes patients who have had only occasional episodes, with long remissions and survival/ There is no known effective therapy. Differential diagnosis includes the secondary hemosiderosis of increased pulmonary venous pressure (as in chronic left heart failure or mitral stenosis), pneumonia, chronic leukemia,' collagen disease, Goodpasture's syndrome, lung carcinoma with recurrent hemorrhage: and pulmonary contusion secondary to chest trauma. REFERENCES
1
K, H. AND SOMMERS, S. C.: Idiopathic pulmonary hemosiderosis and related syndromes, Amer. J. Med' J 32:499, 1962. 2 BRONSON, M. S.: Idiopathic pulmonary hemosiderosis in adults, Amer. J. Roentgen 83: 260, 1960. " 3 GREENSPAN J R. H.: Chronic disseminated alveolar diseases of the lung, Seminars in Roenjgenology, 2: 77, 1967. 4 GREEN J R. A.: Nodular aspirational pulmonary hemosiderosis, Amer. J. Ro/mtgen. 92'561, 1964-. ' . BENJAMIN FELSON, M.D., Editor HAROLD SPITZJ M.D., Co-Editor SOE~GEL,
Readers . I for the Roentgenogram of the Month. Please submit a brief abstract of your caseare t invited Be' .to SUbmit artlCes o nJamm PelIOn, M.D., Department of Radiology, Cincinnati General Hospital. Cincinnati, Ohio.