- Email: [email protected]
Obstructing Tracheal Pulmonary Langerhans Cell Histiocytosis
tive heart failure. Circulation 1992; 86:909 –918 18 Milic-Emili J, Ruff F. Effects of pulmonary congestion and edema on the small airway. Bull Physiopathol Respir (Nancy) 1971; 7:1181–1196 19 Petermann W, Barth J, Entzian P. Heart failure and airway obstruction. Int J Cardiol 1987; 17:207–209 20 Cabanes LR, Weber SN, Matran R, et al. Bronchial hyperresponsiveness to methacholine in patients with impared left ventricular function. N Engl J Med 1989; 320:1317–1322 21 Light RM, George RB. Serial pulmonary function in patients with acute heart failure. Arch Intern Med 1983; 143:429 – 433 22 Depeursinge FB, Depeursinge CD, Boutaleb AD, et al. Respiratory system impedance in patients with acute left ventricular failure: pathophysiology and clinical interest. Circulation 1986; 73:386 –395 23 Cherniack RM, Cuddy TE, Armstrong JB. Significance of pulmonary elastic and viscous resistance in orthopnea. Circulation 1957; 15:859 – 864 24 Tucker DH, Sieker HO. The effect of change in body position on lung volumes and intrapulmonary gas mixing in patients with obesity, heart failure and emphysema. Am Rev Respir Dis 1960; 82:787–791 25 Marini JJ, Tyler ML, Hudson LD, et al. Influence of headdependent positions on lung volume and oxygen saturation in chronic airflow obstruction. Am Rev Respir Dis 1984; 129: 101–105 26 Dikshit K, Vyden JK, Forrester JS, et al. Renal and extrarenal hemodynamic effects of furosemide in congestive heart failure after acute myocardial infarction. N Engl J Med 1973; 288:1087–1090 27 Pouleur H, Covell JW, Ross J Jr. Effects of nitroprusside and central blood volume in the absence and presence of acute heart failure. Circulation 1980; 61:328 –337 28 O’Donnell DE. Breathlessness in patients with chronic airflow limitation: mechanisms and management. Chest 1994; 106:904 –912 29 O’Donnell DE. Effect of dynamic airway compression on breathing pattern and respiratory sensation in severe chronic obstructive pulmonary disease. Am Rev Respir Dis 1987; 135:912–918 30 Tucker DH, Sieker HO. Effect of change in body position on lung volumes and intrapulmonary gas mixing in patients with obesity, heart failure and emphysema. Am Rev Respir Dis 1960; 82:787–791 31 Hogg JC, Agarawal JB, Gardiner AJS, et al. Distribution of airway resistance with developing pulmonary edema in dogs. J Appl Physiol 1972; 32:20 –24 32 Ding DJ, Martin JG, Macklem PT. Effects of lung volume on maximal methacholine-induced bronchoconstriction in normal humans. J Appl Physiol 1987; 62:1324 –1330
Obstructing Tracheal Pulmonary Langerhans Cell Histiocytosis* Zvi G. Fridlender, MD; Mendel Glazer, MD; Gail Amir, MD; and Neville Berkman, MBBCh
Pulmonary Langerhans cell histiocytosis, also known as eosinophilic granuloma, is an uncommon interstitial lung disease. A solitary nodule, usually parenchywww.chestjournal.org
mal, may rarely be the only manifestation of the disease. We describe a case of Langerhans cell histiocytosis presenting as an obstructing tracheal lesion in a 55-year-old woman. Following complete resection of the lesion via flexible bronchoscopy, full recovery was achieved. This case represents a unique cause for tracheal obstruction, as well as an unreported manifestation of pulmonary Langerhans cell histiocytosis. (CHEST 2005; 128:1057–1058) Key words: endobronchial obstruction; pulmonary Langerhans cell histiocytosis; trachea Abbreviations: FOB ⫽ fiberoptic bronchoscopy
Langerhans cell histiocytosis, also known as P ulmonary pulmonary eosinophilic granuloma and pulmonary
histiocytosis X, is an uncommon interstitial lung disorder that is characterized by the abnormal proliferation of Langerhans cells.1,2 The majority of the patients are smokers, usually in their third to fourth decade of life.2 The characteristic presentation is of nonspecific respiratory complaints, together with reticulonodular pulmonary infiltrates.1 Occasionally, Langerhans cell histiocytosis presents with a solitary, parenchymal pulmonary nodule.1 There are only isolated cases in the literature3–5 of endobronchial Langerhans cell histiocytosis, mostly in young adults. Two children have been described in which Langerhans cell histiocytosis of the head and neck invaded the trachea causing significant obstruction.6 In this report, we describe a very unusual case of Langerhans cell histiocytosis in an adult woman, in which the only manifestation of disease was a partially obstructing tracheal mass.
Case Report A 55-year-old, nonsmoking, female physician was referred to a pulmonary clinic due to continuous dry cough. She had been complaining of dry cough for ⬎ 2 years. Initially, she had cough attacks lasting a few minutes about once a week. Gradually, the attacks became daily, occurring mostly during the day, and rarely at night. The cough was dry, but occasionally was accompanied by the production of white sputum and bloody strikes. The patient attempted to treat herself with inhaled corticosteroids and oral antihistamines, with only minor improvement. She had been previously healthy aside from hypertension treated with atenolol. Her husband is a current smoker. Pulmonary spirometry was compatible with significant upper airway obstruction. A fiberoptic evaluation of the upper airway showed no significant findings. A chest CT scan demonstrated a round, partially obstructing, space-occupying lesion in the lower third of the trachea (Fig 1, top left, a). No other focal findings *From the Institute of Pulmonology (Drs. Fridlender and Berkman) and the Department of Pathology (Dr. Amir), Hadassah University Hospital, Jerusalem, Israel; and Rokah Pulmonary Institution (Dr. Glazer), Kupat Cholim Clalit, Israel. Manuscript received January 21, 2005; revision accepted March 9, 2005. Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal. org/misc/reprints.shtml). Correspondence to: Zvi G. Fridlender, MD, Institute of Pulmonology, Hadassah University Hospital, PO Box 12000, Jerusalem, Israel; e-mail: [email protected] CHEST / 128 / 2 / AUGUST, 2005
1057
Figure 1. Top left, a: CT scan showing an intratracheal obstructing lesion (arrow). Top right, b: intratracheal view of the lesion by FOB. Bottom left, c: representative specimen showing Langerhans cells (arrowheads) and eosinophils (left side) [hematoxylineosin, original ⫻40]. Bottom right, d: representative specimen, showing positive staining for S100 protein (original ⫻40). were detected in the lungs. Flexible foberoptic bronchoscopy (FOB) was performed. At the lower third of the trachea, a round, smooth lesion was found with the obstruction of 80% of the tracheal lumen (Fig 1, top right, b). A biopsy specimen from the lesion was compatible with Langerhans cell histiocytosis, and therefore a second FOB was performed with full excision of the lesion using laser therapy with a Nd-YAG laser (30 W; total energy, 3,000 J). The mass rapidly “melted” in response to laser therapy and was easily removable with FOB forceps. Equipment for the performance of rigid bronchoscopy was available on hand if required. A histologic evaluation of the specimen demonstrated the mixed infiltration of lymphocytes, plasma cells, and eosinophils, with aggregates of Langerhans cells (Fig 1, bottom left, c). Immunohistochemical staining was strongly positive for S-100 protein (Fig 1, bottom right, d), was weakly positive for CD1a, and was negative for CD30 and CD68. The histologic findings were considered to be diagnostic of Langerhans cell histiocytosis with reparative changes. Following excision of the lesion, an immediate dramatic improvement in the patient’s symptoms was noted. Two months later, she was completely asymptomatic, with normalization of pulmonary function. A follow-up FOB showed only a small endotracheal scar at the site of the excised mass.
We found only three cases of patients with pulmonary Langerhans cell histiocytosis presenting with endobronchial obstruction since then, all occurring in young adults. In two of these cases the endobronchial nodule was the only manifestation of the disease,3 while in the third case the nodule was part of a more diffuse involvement.4 Two cases of Langerhans cell histiocytosis obstructing the trachea have been described in children,6 with significant disease of the head and neck invading the trachea and causing substantial obstruction. Our patient represents the first report of eosinophilic granuloma in which the only manifestation was a discrete obstructing endotracheal mass in the absence of disease elsewhere. In summary, we have described a unique case of Langerhans cell histiocytosis in an adult, nonsmoking woman, in which the sole manifestation of the disease was a partially obstructing tracheal mass. Local excision of the mass was the only treatment required for our patient. To the best of our knowledge, this is the first report of Langerhans cell histiocytosis limited to the trachea, as well as being a very unusual cause of tracheal obstruction.
References 1 Khoor A, Myers JL, Tazelaar HD, et al. Pulmonary Langerhans cell histiocytosis presenting as a solitary nodule. Mayo Clin Proc 2001; 76:209 –211 2 Sundar KM, Gosselin MV, Chung HL, et al. Pulmonary Langerhans cell histiocytosis. Chest 2003; 123:1673–1683 3 Loukides S, Karameris A, Lachanis S, et al. Eosinophilic granuloma of the lung presenting as an endobronchial mass. Monaldi Arch Chest Dis 2000; 55:208 –209 4 Suzuki M, Betsuyaku T, Suga M, et al. Pulmonary Langerhan’s cell histiocytosis presenting with an endobronchial lesion. Intern Med 2004; 43:227–230 5 O’Donnell AE, Tsou E, Awh C, et al. Endobronchial eosinophilic granuloma: a rare cause of total lung atelectasis. Am Rev Respir Dis 1987; 136:1478 –1480 6 Herman TE, Shackelford GD, Borders JL, et al. Unusual manifestations of Langerhans cell histiocytosis of the head and neck: case report with pseudoaneurysm of external carotid artery, tracheal, mandibular, and sphenoid involvement. Pediatr Radiol 1993; 23:41– 43 7 Lacronique J, Roth C, Battesti JP, et al. Chest radiological features of pulmonary histiocytosis X: a report based on 50 adult cases. Thorax 1982; 37:104 –109
Discussion Pulmonary Langerhans cell histiocytosis, or pulmonary eosinophilic granuloma, is an uncommon interstitial lung disorder that is characterized by the abnormal proliferation of Langerhans cells.1,2 Most patients present with nonspecific respiratory complaints such as dyspnea, cough, and chest pain, and sometimes hemoptysis occurs.2 The characteristic pattern seen on a chest radiograph is of ill-defined nodules and reticular opacities with or without cysts.7 Cysts and/or nodules can be found on chest CT scans in virtually all patients.2 Langerhans cell histiocytosis presents occasionally with a solitary, parenchymal pulmonary nodule, which may even reoccur following resection.1 Endobronchial eosinophilic granuloma was first reported in 1987 in a 12-yearold male child.5 1058
Continuous Murmur Caused by a Ventricular Septal Defect Post-Myocardial Infarction* Visual Display With a Handheld Computer Jeff Olson, DO and Morton Tavel, MD
Ventricular septal defects do not typically result in a continuous murmur. The only previously reported case involved a congenital ventricular septal defect. We report a case of an acquired ventricular septal Selected Reports
Obstructing Tracheal Pulmonary Langerhans Cell Histiocytosis* Zvi G. Fridlender, MD; Mendel Glazer, MD; Gail Amir, MD; and Neville Berkman, MBBCh
Pulmonary Langerhans cell histiocytosis, also known as eosinophilic granuloma, is an uncommon interstitial lung disease. A solitary nodule, usually parenchywww.chestjournal.org
mal, may rarely be the only manifestation of the disease. We describe a case of Langerhans cell histiocytosis presenting as an obstructing tracheal lesion in a 55-year-old woman. Following complete resection of the lesion via flexible bronchoscopy, full recovery was achieved. This case represents a unique cause for tracheal obstruction, as well as an unreported manifestation of pulmonary Langerhans cell histiocytosis. (CHEST 2005; 128:1057–1058) Key words: endobronchial obstruction; pulmonary Langerhans cell histiocytosis; trachea Abbreviations: FOB ⫽ fiberoptic bronchoscopy
Langerhans cell histiocytosis, also known as P ulmonary pulmonary eosinophilic granuloma and pulmonary
histiocytosis X, is an uncommon interstitial lung disorder that is characterized by the abnormal proliferation of Langerhans cells.1,2 The majority of the patients are smokers, usually in their third to fourth decade of life.2 The characteristic presentation is of nonspecific respiratory complaints, together with reticulonodular pulmonary infiltrates.1 Occasionally, Langerhans cell histiocytosis presents with a solitary, parenchymal pulmonary nodule.1 There are only isolated cases in the literature3–5 of endobronchial Langerhans cell histiocytosis, mostly in young adults. Two children have been described in which Langerhans cell histiocytosis of the head and neck invaded the trachea causing significant obstruction.6 In this report, we describe a very unusual case of Langerhans cell histiocytosis in an adult woman, in which the only manifestation of disease was a partially obstructing tracheal mass.
Case Report A 55-year-old, nonsmoking, female physician was referred to a pulmonary clinic due to continuous dry cough. She had been complaining of dry cough for ⬎ 2 years. Initially, she had cough attacks lasting a few minutes about once a week. Gradually, the attacks became daily, occurring mostly during the day, and rarely at night. The cough was dry, but occasionally was accompanied by the production of white sputum and bloody strikes. The patient attempted to treat herself with inhaled corticosteroids and oral antihistamines, with only minor improvement. She had been previously healthy aside from hypertension treated with atenolol. Her husband is a current smoker. Pulmonary spirometry was compatible with significant upper airway obstruction. A fiberoptic evaluation of the upper airway showed no significant findings. A chest CT scan demonstrated a round, partially obstructing, space-occupying lesion in the lower third of the trachea (Fig 1, top left, a). No other focal findings *From the Institute of Pulmonology (Drs. Fridlender and Berkman) and the Department of Pathology (Dr. Amir), Hadassah University Hospital, Jerusalem, Israel; and Rokah Pulmonary Institution (Dr. Glazer), Kupat Cholim Clalit, Israel. Manuscript received January 21, 2005; revision accepted March 9, 2005. Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal. org/misc/reprints.shtml). Correspondence to: Zvi G. Fridlender, MD, Institute of Pulmonology, Hadassah University Hospital, PO Box 12000, Jerusalem, Israel; e-mail: [email protected] CHEST / 128 / 2 / AUGUST, 2005
1057
Figure 1. Top left, a: CT scan showing an intratracheal obstructing lesion (arrow). Top right, b: intratracheal view of the lesion by FOB. Bottom left, c: representative specimen showing Langerhans cells (arrowheads) and eosinophils (left side) [hematoxylineosin, original ⫻40]. Bottom right, d: representative specimen, showing positive staining for S100 protein (original ⫻40). were detected in the lungs. Flexible foberoptic bronchoscopy (FOB) was performed. At the lower third of the trachea, a round, smooth lesion was found with the obstruction of 80% of the tracheal lumen (Fig 1, top right, b). A biopsy specimen from the lesion was compatible with Langerhans cell histiocytosis, and therefore a second FOB was performed with full excision of the lesion using laser therapy with a Nd-YAG laser (30 W; total energy, 3,000 J). The mass rapidly “melted” in response to laser therapy and was easily removable with FOB forceps. Equipment for the performance of rigid bronchoscopy was available on hand if required. A histologic evaluation of the specimen demonstrated the mixed infiltration of lymphocytes, plasma cells, and eosinophils, with aggregates of Langerhans cells (Fig 1, bottom left, c). Immunohistochemical staining was strongly positive for S-100 protein (Fig 1, bottom right, d), was weakly positive for CD1a, and was negative for CD30 and CD68. The histologic findings were considered to be diagnostic of Langerhans cell histiocytosis with reparative changes. Following excision of the lesion, an immediate dramatic improvement in the patient’s symptoms was noted. Two months later, she was completely asymptomatic, with normalization of pulmonary function. A follow-up FOB showed only a small endotracheal scar at the site of the excised mass.
We found only three cases of patients with pulmonary Langerhans cell histiocytosis presenting with endobronchial obstruction since then, all occurring in young adults. In two of these cases the endobronchial nodule was the only manifestation of the disease,3 while in the third case the nodule was part of a more diffuse involvement.4 Two cases of Langerhans cell histiocytosis obstructing the trachea have been described in children,6 with significant disease of the head and neck invading the trachea and causing substantial obstruction. Our patient represents the first report of eosinophilic granuloma in which the only manifestation was a discrete obstructing endotracheal mass in the absence of disease elsewhere. In summary, we have described a unique case of Langerhans cell histiocytosis in an adult, nonsmoking woman, in which the sole manifestation of the disease was a partially obstructing tracheal mass. Local excision of the mass was the only treatment required for our patient. To the best of our knowledge, this is the first report of Langerhans cell histiocytosis limited to the trachea, as well as being a very unusual cause of tracheal obstruction.
References 1 Khoor A, Myers JL, Tazelaar HD, et al. Pulmonary Langerhans cell histiocytosis presenting as a solitary nodule. Mayo Clin Proc 2001; 76:209 –211 2 Sundar KM, Gosselin MV, Chung HL, et al. Pulmonary Langerhans cell histiocytosis. Chest 2003; 123:1673–1683 3 Loukides S, Karameris A, Lachanis S, et al. Eosinophilic granuloma of the lung presenting as an endobronchial mass. Monaldi Arch Chest Dis 2000; 55:208 –209 4 Suzuki M, Betsuyaku T, Suga M, et al. Pulmonary Langerhan’s cell histiocytosis presenting with an endobronchial lesion. Intern Med 2004; 43:227–230 5 O’Donnell AE, Tsou E, Awh C, et al. Endobronchial eosinophilic granuloma: a rare cause of total lung atelectasis. Am Rev Respir Dis 1987; 136:1478 –1480 6 Herman TE, Shackelford GD, Borders JL, et al. Unusual manifestations of Langerhans cell histiocytosis of the head and neck: case report with pseudoaneurysm of external carotid artery, tracheal, mandibular, and sphenoid involvement. Pediatr Radiol 1993; 23:41– 43 7 Lacronique J, Roth C, Battesti JP, et al. Chest radiological features of pulmonary histiocytosis X: a report based on 50 adult cases. Thorax 1982; 37:104 –109
Discussion Pulmonary Langerhans cell histiocytosis, or pulmonary eosinophilic granuloma, is an uncommon interstitial lung disorder that is characterized by the abnormal proliferation of Langerhans cells.1,2 Most patients present with nonspecific respiratory complaints such as dyspnea, cough, and chest pain, and sometimes hemoptysis occurs.2 The characteristic pattern seen on a chest radiograph is of ill-defined nodules and reticular opacities with or without cysts.7 Cysts and/or nodules can be found on chest CT scans in virtually all patients.2 Langerhans cell histiocytosis presents occasionally with a solitary, parenchymal pulmonary nodule, which may even reoccur following resection.1 Endobronchial eosinophilic granuloma was first reported in 1987 in a 12-yearold male child.5 1058
Continuous Murmur Caused by a Ventricular Septal Defect Post-Myocardial Infarction* Visual Display With a Handheld Computer Jeff Olson, DO and Morton Tavel, MD
Ventricular septal defects do not typically result in a continuous murmur. The only previously reported case involved a congenital ventricular septal defect. We report a case of an acquired ventricular septal Selected Reports