relative risk of VTE of about 2.0.11 In contrast to the most common cause of congenital thrombophilia, a point mutation at position 1691 in the factor V gene (ie, FVL),12,16 FII G20210A has been proposed as being an independent risk factor for PE.17 Whereas FVL is not associated with CTEPH,18 the occurrence of FII G20210A in CTEPH patients has not yet been reported. The calculated prevalence for the combination of congenital type 1 AT deficiency and the FII G20210A mutation is estimated to be approximately 1 in 500,000 cases.19 So far, only four cases of such a combination of AT deficiency and the heterozygous FII G20210A mutation have been reported.20,21 It seems that the concomitant presence of FII G20210A renders patients with inherited thrombophilia more susceptible to thrombotic episodes, but more reliable information about the interaction of FII G20210A with other prothrombotic effects is required. The greatest progress in the management of CTEPH is the clinical awareness of the disease and the diagnostic workup of surgical thrombus accessibility.1,5–7 The present case is unique in that it demonstrates a rare combined thrombophilic defect underlying severe unilateral CTEPH. To this date, no knowledge exists on the precise mechanism causing CTEPH. Although a combined coagulation defect found in only 1 of 500,000 patients is unlikely to have a great bearing even on a rare disease such as CTEPH, this case illustrates a potential pathophysiologic contribution of plasmatic coagulation and thromboembolism in patients with CTEPH. ACKNOWLEDGMENT: We are indebted to Ingrid Pabinger for providing the molecular data on the patient‘s AT deficiency.
15 16 17 18
References 1 Fedullo PF, Auger WR, Channick RN, et al. Chronic thromboembolic pulmonary hypertension. Clin Chest Med 1995; 16:353–374 2 Presti B, Berthrong M, Sherwin RM. Chronic thrombosis of major pulmonary arteries. Hum Pathol 1990; 21:601– 606 3 Hartz RS. Surgery for chronic thromboembolic pulmonary hypertension. World J Surg 1999; 23:1137–1147 4 Lang IM, Marsh JJ, Olman MA, et al. Parallel analysis of tissue-type plasminogen activator and type 1 plasminogen activator inhibitor in plasma and endothelial cells derived from patients with chronic pulmonary thromboemboli. Circulation 1994; 90:706 –712 5 Simonneau G, Azarian R, Brenot F, et al. Surgical management of unresolved pulmonary embolism: a personal series of 72 patients. Chest 1995; 107:52S–55S 6 Mayer E, Dahm M, Hake U, et al. Mid-term results of pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension. Ann Thorac Surg 1996; 61: 1788 –1792 7 Ando M, Takamoto S, Okita Y, et al. Operation for chronic pulmonary thromboembolism accompanied by thrombophilia in 8 patients. Ann Thorac Surg 1998; 66:1919 –1924 8 Wolf M, Boyer-Neumann C, Parent F, et al. Thrombotic risk factors in pulmonary hypertension. Eur Respir J 2000; 15: 395–399 9 Lane DA, Mannucci PM, Bauer KA, et al. Inherited thrombophilia: part 1. Thromb Haemost 1996; 76:651– 662 10 Lane DA, Bayston T, Olds RJ, et al. Antithrombin mutation database: 2nd (1997) update; for the Coagulation Inhibitors
Subcommittee of the Scientific and Standardization Committee of the International Society on Thrombosis and Hemostasis. Thromb Haemost 1997; 77:197–211 Poort SR, Rosendaal FR, Reitsma PH, et al. A common genetic variation in the 3⬘-untranslated region of the prothrombin gene is associated with elevated plasma prothrombin levels and an increase in venous thrombosis. Blood 1996; 88:3698 –3703 Bertina RM, Koeleman BPC, Koster T, et al. Mutation in blood coagulation factor V associated with resistance to activated protein C. Nature 1994; 369:64 – 67 Jamieson SW, Auger WR, Fedullo PF, et al. Experience and results with 150 pulmonary thrombendarterectomy operations over a 29-month period. Thorac Cardiovasc Surg 1993; 106:116 –126 Minnema MC, Chang AC, Jansen PM, et al. Recombinant human antithrombin III improves survival and attenuates inflammatory responses in baboons lethally challenged with Escherichia coli. Blood 2000; 95:1117–1123 Hirsch AM, Moser KM, Auger WR, et al. Unilateral pulmonary artery thrombotic occlusion: is distal arteriopathy a consequence? Am J Respir Crit Care Med 1996; 154:491– 496 Vandenbroucke JP, Bertina RM, Holmes ZR, et al. Factor V Leiden and fatal pulmonary embolism. Thromb Haemost 1998; 79:511–516 Reuner KH, Ruf A, Litfin F, et al. The mutation G20210-⬎A in the prothrombin gene is a strong risk factor for pulmonary embolism. Clin Chem 1998; 44:1365–1366 Lang I, Klepetko W, Pabinger I. No increased prevalence of the factor V Leiden mutation in chronic major vessel thromboembolic pulmonary hypertension. Thromb Haemost 1996; 76:476 – 477 Laffan M, Tuddenham E. Assessing thrombotic risk. BMJ 1998; 317:520 –523 Van Boven HH, Vandenbroucke JP, Briet E, et al. Gene-gene and gene-environment interactions determine risk of thrombosis in families with inherited antithrombin deficiency. Blood 1999; 94:2590 –2594 De Stefano V, Chiusolo P, Paciaroni K. Prevalence of the factor II G20210A mutation in symptomatic patients with inherited thrombophilia. Thromb Haemost 1998; 80:342–343
Bilateral Thumb Burns Leading to the Diagnosis of Crack Lung* David Gatof, MD; Richard K. Albert, MD, FCCP
Bilateral thumb burns on a young woman admitted to the hospital with the diagnosis of communityacquired pneumonia led us to consider the diagnosis of crack lung despite the fact that the woman denied cocaine use. Cocaine was found on a urine toxicology study, and its use was subsequently confirmed by history. The patient was treated for crack lung with complete resolution of her symptoms and radiographic findings. Inspection of the hands for burns consistent with handling cocaine pipes should prompt a consideration of crack lung in patients with pulmonary infiltrates. (CHEST 2002; 121:289 –291) CHEST / 121 / 1 / JANUARY, 2002
finding led to the suspicion of crack lung. The diagnosis was confirmed by urine toxicology studies and by subsequent history.
Case Report A 29-year-old woman presented to the emergency department with the acute onset of dyspnea and chest pain accompanied by a productive cough. She had a 29-pack-year history of tobacco use and a recent episode of acute bronchitis that had been treated with antibiotics. She denied fever, chills, hemoptysis, and illicit drug use. Her only current medications were conjugated estrogens and medroxyprogesterone. The patient was thin, pale, and diaphoretic, and was in moderate respiratory distress. Her temperature was 38°C, heart rate was 136 beats/min, respiratory rate was 22 breaths/min, and BP was 126/36 mm Hg. Coarse breath sounds were heard in the lower lobes bilaterally, along with fine crackles. Blistering lesions with black discolorations were found on both thumbs (Fig 1). Laboratory study findings were normal, with the exception of a WBC count of 18,900/L; hematocrit, 35%; lactate dehydrogenase, 450 U/L. Room air arterial blood gas measurements were as follows: pH, 7.41; Paco2, 38 mm Hg; and Pao2, 47 mm Hg. Her chest radiograph showed diffuse alveolar opacities (Fig 2). The skin findings were thought to represent thermal burns from lighting a crack cocaine pipe with a butane lighter directed downward onto the pipe. Urine toxicology study findings were positive for cocaine; on further questioning, the patient admitted to smoking large quantities of free-base cocaine only a few hours before the onset of her symptoms, and repeatedly burning her thumbs with the lighter. She was administered IV methylprednisolone, inhaled bronchodilators, and oxygen, resulting in complete clearing of her symptoms and radiographic abnormalities within 72 h.
Discussion Free-base cocaine is prepared from baking soda and cocaine hydrochloride. The combination is boiled, cooled,
Figure 1. Photographs of the patient’s hands (top), left thumb (middle), and right thumb (bottom).
Key words: cocaine; crack lung; drug-induced lung disease
use has increased markedly over the last 15 C ocaine years, and cocaine-related mortality has been esti-
mated to account for 5 of every 1,000 deaths.1 We recently encountered a patient who presented with acute respiratory failure but denied illicit drug use. An unusual physical
*From the Denver Health Medical Center University of Colorado Health Sciences Center, Denver, CO. Manuscript received January 22, 2001; revision accepted June 6, 2001. Correspondence to: Richard K. Albert, MD, FCCP, Denver Health Medical Center, 777 Bannock, MC 4000, Denver, CO 80204-4507; e-mail: [email protected]
Figure 2. Posteroanterior chest radiograph of the patient showing diffuse alveolar infiltrates. Selected Reports
Table 1—Pulmonary Complications Associated With Cocaine Use Barotrauma Pneumothorax Pneumomediastinum Pneumopericardium Pulmonary edema Diffuse alveolar damage Pulmonary hemorrhage Pulmonary infarction Eosinophilic lung disease Asthma Interstitial pneumonitis Bronchiolitis obliterans organizing pneumonia Bronchitis Thermal upper airway injury Tracheal stenosis
and extracted with ether. The solvent is then evaporated leaving a heat-stable, crystalline precipitant called crack (after the popping sound made by the burning crystals). Crack is typically smoked through a glass or brass pipe using a butane torch and can cause numerous pulmonary toxicities (Table 1).2,3 In the proper setting, finding thermal injury on the fingertips should suggest crack cocaine use. Because establishing a diagnosis of crack lung has important diagnostic and therapeutic implications, this physical finding should prompt physicians to obtain appropriate laboratory studies and seek a more directed history.
References 1 Gawain F, Ellinwood EH. Cocaine and other stimulants: actions, abuse and treatment. N Engl J Med 1988; 318:1173– 1182 2 Forrester JM, Steele AW, Waldron JA, et al. Crack lung: an acute pulmonary syndrome with a spectrum of clinical and histopathologic findings. Am Rev Respir Dis 1990; 142:462– 467 3 Haim DY, Lippmann ML, Goldberg SK, et al. The pulmonary complications of crack cocaine: a comprehensive review. Chest 1995; 107:233–240
Varicosities of the Valleculae* An Unusual Cause of Hemoptysis? Richard Booton, MRCP; and Badie K. Jacob, MD, FCCP
Hemoptysis is a common respiratory symptom causing a great deal of anxiety. The cause is often apparent following a clinical history, upper-airway examination, bronchoscopy, and CT scanning of the thorax. We present a case of massive hemoptysis, the etiology of which was not readily apparent despite this conventional approach. Vallecular hemorrhage has been previously reported but is usually minor
unless associated with surgical trauma, and can be readily missed if not aware of the possibility. We speculate about the etiology and mechanism for recurrent hemorrhage. (CHEST 2002; 121:291–292) Key words: hemoptysis; valleculae; varicosities Abbreviation: AFB ⫽ acid-fast bacilli
or life-threatening hemoptysis requires a thorS evere ough and timely evaluation. The presence of concomitant chest disease may delay this evaluation, compromising the abilities to deliver definitive treatment, and to reduce morbidity and mortality in patients who rebleed. In addition, unrecognized or rare causes of massive hemoptysis may have a similar effect. We review a case of an elderly man who presented with substantial hemoptysis without an apparent source despite conventional management. Delays in diagnosis in this setting not only cause considerable patient anxiety and morbidity but should also prompt us to consider rare conditions.
Case Report A 72-year-old man with COPD presented in July 1998 with a short history of cough and dyspnea succeeded by frank hemoptysis and blood clots. There was no history of weight loss or night sweats. He was a current smoker of two ounces of tobacco per week for 60 years. Clinical examination confirmed airflow limitation without evidence of clubbing or lymphadenopathy or additional respiratory signs. Arterial oxygen saturation on room air was 91%. Initial hematologic, biochemical, and clotting profile findings were normal. Chest radiography demonstrated overinflation, prominent pulmonary arteries, and patchy inflammatory change in the right upper zone. Sputum culture findings demonstrated a scanty growth of Haemophilus influenzae, with smear and culture negativity for acid-fast bacilli (AFB) on three samples. No evidence of malignancy was seen on cytologic examination. Flexible fiberoptic bronchoscopy was undertaken, which demonstrated no evidence of active bleeding and no mucosal lesion. A subsequent CT scan of the thorax noted consolidation in the right upper lobe but without evidence of bronchiectasis. BAL fluid was smear-negative for AFB. His hemoptysis resolved after 1 week, and he was subsequently discharged. At review 1 month later, no further hemoptysis had been encountered. Lavage fluid revealed AFB after 3 weeks of culture, and he was prescribed rifampicin, isoniazid, and pyrazinamide. Over the next 2 weeks, he was admitted to the hospital as an emergency patient on two occasions with further, profuse, free-flowing hemoptysis (2 g/dL reduction in hemoglobin); on both occasions, coagulation and biochemical parameters were otherwise normal. An ear, nose, and throat review revealed no active bleeding or significant pathology on examination of the postnasal space or indirect laryngoscopy. He underwent further bronchoscopic examinations *From the Department of Respiratory Services, Bradford Royal Infirmary, Bradford Hospitals NHS Trust, Bradford, West Yorkshire, UK. Manuscript received January 25, 2001; revision accepted June 19, 2001. Correspondence to: Badie K. Jacob, MD, FCCP, Consultant Chest Physician and Chief of Respiratory Services, Bradford Royal Infirmary, Bradford Hospitals NHS Trust, Duckworth Lane, Bradford, West Yorkshire BD9 6RJ, UK CHEST / 121 / 1 / JANUARY, 2002