Description, Causes and Risk Factors:
Accumulation of hemosiderin in tissue, particularly in liver and spleen.
Hemosiderin is a blood protein that is formed when red blood cells breakdown. The protein stores a tiny amount of iron to supply body tissues and keep iron levels stable in the body. If excessive hemorrhaging occurs in a lung, the red blood cells that build up inside the organ can create huge quantities of hemosiderin proteins. Several different health problems can lead to pulmonary hemorrhage and hemosiderosis, most notably autoimmune inflammatory disorders, chronic bronchitis, and exposure to environmental toxins and molds. Many cases of childhood hemosiderosis are idiopathic, meaning that doctors are unable to identify a true underlying cause.
Pulmonary hemosiderosis is an uncommon disorder characterized by accumulation of iron in form of hemosiderin in the lungs. The first pathologic description of the disease was written by Virchow in 1864. In 1918, Goodpasture reported the association of pulmonary hemorrhage and glomerulonephritis which has subsequently been termed Goodpasture's syndrome. Ceelen in 1931 first documented the clinical findings associated with pulmonary hemosiderosis. In 1962, Heiner described four patients with the disorder who improved markedly with the elimination of cow's milk from their diets.
Pulmonary hemosiderosis may occur as a primary phenomenon, most commonly in children, or it may complicate another underlying illness such as cardiac disease or collagen vascular disease. The secondary form of pulmonary hemosiderosis is seen most frequently in the adult population. Patients with primary pulmonary hemosiderosis may be further classified into four groups: those with accompanying glomerulonephritis (Goodpasture's syndrome); those with cow's milk sensitivity; those with associated cardiac or pancreatic disease (myocarditis, pancreatic insufficiency); and those with idiopathic disease.
Idiopathic pulmonary hemosiderosis is a very rare disease that causes diffuse alveolar hemorrhage. Its clinical course is exceedingly variable, and delayed diagnosis is common. Effective treatment of this disease requires high degree of clinical suspicion and accurate differential diagnosis. The estimated incidence was 0.24 per million children in Sweden and 1.23 per million in Japan. Most cases occur in children and young adults. The etiology is unknown. The disease is characterized by the triad of hemoptysis, pulmonary infiltrates on chest radiograph and iron deficiency anemia. The course of disease is variable and may involve spontaneous remission.
The pulmonary hemorrhage may be clinically insignificant or it may be massive with early death. Mean survival after diagnosis is 2.5-5 years. Anemia may be the only presenting feature, which is due to occult pulmonary hemorrhage.
The prognosis for the patients with pulmonary hemosiderosis is variable. Recurrent episodes of pulmonary hemorrhage may lead to respiratory insufficiency with clubbing, dyspnea, pulmonary hypertension, and right heart failure. However, death more commonly follows massive pulmonary hemorrhage. The prognosis appears to be poorest in Goodpasture's syndrome, with death resulting from pulmonary or renal disease an average of 6 months after diagnosis in one report. For idiopathic pulmonary hemosiderosis, the average survival after diagnosis was 2.5 years. However, survival for longer than 10 years after diagnosis has more recently been reported in patients receiving aggressive therapy.
The most common symptoms of acute hemosiderosis include coughing up blood, breathing difficulties, chest tightness, and dizziness. A person who suffers a major hemorrhage may turn pale and possibly lose consciousness. The heartbeat may initially race but then slow to a dangerous level. If hemosiderosis becomes a frequent or chronic problem, a person can experience symptoms of iron deficiency anemia, such as extreme fatigue, severe headaches, and irritability.
A specialist can diagnose hemosiderosis by asking about symptoms and medical history and checking blood samples for signs of anemia. Chest x-rays and computerized tomography scans are taken to look for active pulmonary hemorrhages and sections of scar tissue where bleeding has occurred in the past. Depending on the severity of symptoms, a patient may be immediately hospitalized or scheduled for a follow-up visit after receiving a diagnosis.
Serum ferritin is a low cost, readily available, and minimally invasive method for assessing body iron stores. However, the major problem with using it as an indicator of hemosiderosis is that it can be elevated in a range of other medical conditions unrelated to iron levels including infection, inflammation, fever, liver disease, renal disease and cancer.
Ventilation/perfusion scanning - Important if pulmonary embolism is suspected.
Hemosiderosis that causes serious complications may require oxygen therapy and heart-stabilizing clinical procedures. A patient might need to take medications to stabilize blood pressure and chelating drugs that flush iron from the body. A blood transfusion may be necessary if a massive amount of blood has been lost. In order to reduce the chances of lung hemorrhages in the future, patients usually need to take immunosuppressant steroids such as prednisone for several months or years. When an underlying cause is found, it is treated accordingly.
Treatment of secondary hemosiderosis is usually directed toward the underlying condition. A gluten-free diet is indicated in cases of celiac disease associated with pulmonary hemosiderosis, even in the absence of GI symptoms.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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