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Presented by Fred Askin, M.D. and prepared by Jeffrey Seibel, M.D. Ph.D.
Case 3: This patient is a 59-year-old man with myelodysplastic syndrome, status post bone marrow transplant.
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Week 84: Case 3
This patient is a 59-year-old man with myelodysplastic syndrome, status post bone marrow transplant. He developed increasing shortness of breath.images/040102case3a.jpg
images/040102case3b.jpg
images/040102case3c.jpgCorrect
Answer: Constrictive bronchiolitis
Histology: There is a layer of dense collagen between the mucosa and the smooth muscle in many of the bronchioles. This phenomenon is highlighted by the use of Movats pentachrome stain which demonstrates both the collagen and the fenestrated elastic lamina of the bronchiole.
Discussion: Constrictive bronchiolitis is also called obliterative bronchiolitis but there has been so much confusion between the term “obliterative bronchiolitis” and “bronchiolitis obliterans organizing pneumonia” (BOOP) that it seems preferable to use the term “constrictive.” Constrictive bronchiolitis is a lesion that will be missed unless one is prepared to specifically look for it. It is a lesion that one should always consider when faced with a lung biopsy which, at first glance, shows no specific pathologic abnormality despite being from a patient who is clinically ill. Bronchiolitis, as Colby has noted (Am J Clin Pathol 1998;109:101-9), represents a cellular mesenchymal reaction involving bronchioles. The cellular infiltrate and the mesenchymal reaction affect the size of the airway lumen, the lamina propria, the muscle layer and the peribronchiolar tissue. The result is a variety of clinical, radiologic and functional patterns of small airway disease. A practical classification of bronchiolitis would include:
1. Respiratory Bronchiolitis (RBILD)
2. Constrictive Bronchiolitis
3. Folliculae Bronchiolitis
4. Bronchiolitis Obliterans Organizing Pneumonia (BOOP).Constrictive bronchiolitis must be separated from BOOP because of the many different ramifications of these distinct diagnoses. Constrictive bronchiolitis occurs in a relatively limited variety of settings, including chronic rejection in lung or heart-lung transplantation and in bone marrow transplantation. Other causes include viral infection (especially in children) and in rheumatoid arthritis or other collagen vascular diseases. Constrictive bronchiolitis has been reported in a variety of other autoimmune disorders such as pemphigus, after inhalation of toxic gasses, associated with certain drugs, and in patients with idiopathic inflammatory bowel disease. (see Annals of Diagnostic Pathology 1998; 2: 321-334).
Constrictive bronchiolitis is characterized by relatively strict localization of the inflammatory fibrotic process to the airway lumen. In contrast, the defining lesion in BOOP is involvement of the airway-airspace junction with a component of inflammatory debris both in small airways and in the adjacent alveolar spaces with the formation of branching fibroblastic “plugs” of collagen. In both constrictive bronchiolitis and BOOP there may be a small amount of interstitial chronic inflammation in the septa near the involved small airways. This process, however, should fade away the further one gets from the airway wall.
Incorrect
Answer: Constrictive bronchiolitis
Histology: There is a layer of dense collagen between the mucosa and the smooth muscle in many of the bronchioles. This phenomenon is highlighted by the use of Movats pentachrome stain which demonstrates both the collagen and the fenestrated elastic lamina of the bronchiole.
Discussion: Constrictive bronchiolitis is also called obliterative bronchiolitis but there has been so much confusion between the term “obliterative bronchiolitis” and “bronchiolitis obliterans organizing pneumonia” (BOOP) that it seems preferable to use the term “constrictive.” Constrictive bronchiolitis is a lesion that will be missed unless one is prepared to specifically look for it. It is a lesion that one should always consider when faced with a lung biopsy which, at first glance, shows no specific pathologic abnormality despite being from a patient who is clinically ill. Bronchiolitis, as Colby has noted (Am J Clin Pathol 1998;109:101-9), represents a cellular mesenchymal reaction involving bronchioles. The cellular infiltrate and the mesenchymal reaction affect the size of the airway lumen, the lamina propria, the muscle layer and the peribronchiolar tissue. The result is a variety of clinical, radiologic and functional patterns of small airway disease. A practical classification of bronchiolitis would include:
1. Respiratory Bronchiolitis (RBILD)
2. Constrictive Bronchiolitis
3. Folliculae Bronchiolitis
4. Bronchiolitis Obliterans Organizing Pneumonia (BOOP).Constrictive bronchiolitis must be separated from BOOP because of the many different ramifications of these distinct diagnoses. Constrictive bronchiolitis occurs in a relatively limited variety of settings, including chronic rejection in lung or heart-lung transplantation and in bone marrow transplantation. Other causes include viral infection (especially in children) and in rheumatoid arthritis or other collagen vascular diseases. Constrictive bronchiolitis has been reported in a variety of other autoimmune disorders such as pemphigus, after inhalation of toxic gasses, associated with certain drugs, and in patients with idiopathic inflammatory bowel disease. (see Annals of Diagnostic Pathology 1998; 2: 321-334).
Constrictive bronchiolitis is characterized by relatively strict localization of the inflammatory fibrotic process to the airway lumen. In contrast, the defining lesion in BOOP is involvement of the airway-airspace junction with a component of inflammatory debris both in small airways and in the adjacent alveolar spaces with the formation of branching fibroblastic “plugs” of collagen. In both constrictive bronchiolitis and BOOP there may be a small amount of interstitial chronic inflammation in the septa near the involved small airways. This process, however, should fade away the further one gets from the airway wall.
