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idiopathic pulmonary hemosiderosis (hemorrhage)
Etiology:
1) unknown, a diagnosis of exclusion
2) implicated factors
a) heritable defect
b) immunologic disorder associated with antibodies to cow's milk
c) cold agglutinins
d) increased serum IgA
e) viral infection
f) primary disorder of airway epithelial cells
g) structural defect in pulmonary capillaries
3) disease associations
a) idiopathic thrombocytopenic purpura (ITP)
b) autoimmune hemolytic anemia
c) non-tropical sprue (celiac disease)
4) pediatric form of the disease thought to be related to spores growing in humid basements
Epidemiology:
1) most cases begin in childhood
2) in childhood, the male:female ratio is 1:1
3) in adults, the male:female ratio is 3:1
Pathology:
1) recurrent intra-alveolar hemorrhage
2) hemosiderin-laden macrophages
3) fibrosis & cor pulmonale may occur
4) kidneys are not involved
Clinical manifestations:
1) chronic cough
2) intermittent hemoptysis
3) fever
4) weight loss
5) generalized lymphadenopathy (25%)
6) hepatosplenomegaly (20%)
7) clubbing (15%)
8) course of the disease is generally prolonged
9) often fatal
Laboratory:
1) evidence of iron-deficiency anemia
a) complete blood coutn (CBC)
b) iron studies: serum iron, TIBC, serum ferritin
c) low reticulocyte count for degree of anemia
2) cold agglutinins in some patients
3) eosinophilia (10%)
Radiology:
1) chest X-ray
a) transient blotchy, perihilar infiltrates in mid & lower lung fields
b) small nodules may be observed
2) computed tomography:
- intrathoracic lymphadenopathy in 25% of patients
Management:
1) blood transfusions
2) iron therapy
3) corticosteroids
4) cytotoxic agents may be useful
5) prognosis: 5 year mortality is 30%
Related
alveolar hemorrhage
General
pulmonary siderosis
hemosiderosis
References
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 786