Q J Med 2013; 106:961–962 doi:10.1093/qjmed/hcs171 Advance Access Publication 5 September 2012
Clinical picture Fatal alveolar hemorrhage due to Kaposi sarcoma Discussion KS is low grade vascular tumor that has been linked with human herpesvirus 8, also known as the KS-associated herpesvirus (KSHV). Its prevalence and aggressiveness have decreased associated with the widespread use highly active ART. KS initially occurred in 6–20% of HIV-infected men and a small number of non-HIV-infected patients from other risk groups.1,2 The clinical presentation of KS varies from indolent skin lesions to extensive visceral disease. Cutaneous lesions are the most common presentation for KS.3
Figure 1. Bilateral ground-glass admixed with consolidative opacity consistent with alveolar hemorrhage (arrow). Numerous bilateral, mid and lower lung solid pulmonary nodules (arrow head).
Figure 2. Coronal view of the chest showing bilateral ground-glass right more than left. Note the markedly enlarged left ventricle and right atrium due to advanced dilated cardiomyopathy (arrow).
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A 43-year-old African-American man with history of diabetes mellitus, chronic systolic heart failure with ejection fraction of 15%, atrial fibrillation and stage III kidney disease HIV (Human immunodeficiency virus) diagnosed in 1994 on antiretroviral therapy (ART). Kaposi sarcoma of the skin and lung diagnosed a year ago with lung wedge biopsy and on chemotherapy, presented with 1-week history of progressive worsening shortness of breath with minimal efforts, pleuritic chest pain and productive cough with ‘penny-sized blood clots’ that were both dark and bright red in color. Furthermore, he reported nocturnal diaphoresis over the same period. Denies any recent exposures or travel outside the USA has been compliant with Trimethoprim/Sulfamethoxazole (TMP/SMX) for Pneumocystis jiroveci Pneumonia (PJP) prophylaxis three times a week for many years. On exam, he was febrile (38.18C) and hypoxic (SpO2 91% on 4 L/min O2 per nasal cannula). He had bilateral coarse crepitations at bases on posterior chest exam. Blood work showed hemoglobin 7.9 g/dl (down from basal of 12.2 g/dl a week prior). Chest roentgenogram (CXR) showed diffuse bilateral mixed interstitial and alveolar infiltrates with bilateral mild pleural effusions. Giving his clinical presentation and history, CT scan of the chest was done and showed diffuse ground-glass opacity admixed with consolidative opacity in the right upper, middle, and lower lobes and the left upper lobes. This was associated with numerous bilateral, mid and lower lung solid pulmonary nodules with soft tissue infiltration along bronchovascular bundles, these findings represent Kaposi’s sarcoma (KS) with diffuse alveolar hemorrhage (Figures 1 and 2). Intravenous antibiotics were administered including azithromycin, piperacillin/tazobactam and vancomycin; TMP/ SMX was continued. He had rapidly progressive deterioration of his respiratory status, requiring 100% high-flow oxygen and subsequently airway intubation and mechanical ventilation. Few hours later he had pulseless electrical activity (PEA) cardiac arrest and passed away after 30 min of advanced cardiopulmonary resuscitation efforts.
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Clinical picture
Patients with pulmonary KS can present with dyspnea, fever, cough, hemoptysis or chest pain.4 These symptoms can be attributed to infectious process. Respiratory failure due to diffuse alveolar hemorrhage can occur and considered the most common cause of mortality in patients with pulmonary KS.4 Among patients with known KS who present with respiratory symptoms, up to 50% have parenchymal involvement by KS.5 CXR, CT scanning and nuclear imaging are useful tools in the diagnostic work-up of HIV-infected patients presenting with pulmonary symptoms. The diagnostic evaluation of suspected parenchymal pulmonary KS requires bronchoscopy in most cases. Endobronchial and transbronchial biopsies have a low diagnostic yield.4,6 The most accurate diagnostic test for pulmonary KS is detection of HHV 8 in bronchoalveolar lavage fluid. Although biological modifiers such as interferon-a have a role early or late in the course of KS, pulmonary involvement usually requires chemotherapy.7,8 Furthermore, ART should be given in most cases.8–10
References 1. Cote TR, Howe HL, Anderson SP, Martin RJ, Evans B, Francis BJ. A systematic consideration of the neoplastic spectrum of AIDS: registry linkage in Illinois. AIDS 1991; 5:49–53. 2. Serraino D, Franceschi S, Tirelli U, Monfardini S. The epidemiology of acquired immunodeficiency syndrome and associated tumours in Europe. Ann Oncol 1992; 3:595–603. 3. Krown SE, Testa MA, Huang J. AIDS-related Kaposi’s sarcoma: prospective validation of the AIDS Clinical Trials Group staging classification. AIDS Clinical Trials Group Oncology Committee. J Clin Oncol 1997; 15:3085–92. 4. Garay SM, Belenko M, Fazzini E, Schinella R. Pulmonary manifestations of Kaposi’s sarcoma. 1987. Chest 2009; 136(Suppl. 5):e30. 5. White DA, Matthay RA. Noninfectious pulmonary complications of infection with the human immunodeficiency virus. Am Rev Respir Dis 1989; 140:1763–87. 6. Meduri GU, Stover DE, Lee M, Myskowski PL, Caravelli JF, Zaman MB. Pulmonary Kaposi’s sarcoma in the acquired immune deficiency syndrome. Clinical, radiographic, and pathologic manifestations. Am J Med 1986; 81:11–8. 7. Stallone G, Schena A, Infante B, Di Paolo S, Loverre A, Maggio G, et al. Sirolimus for Kaposi’s sarcoma in renal-transplant recipients. N Engl J Med 2005; 352:1317–23. 8. Murphy M, Armstrong D, Sepkowitz KA, Ahkami RN, Myskowski PL. Regression of AIDS-related Kaposi’s sarcoma following treatment with an HIV-1 protease inhibitor. AIDS 1997; 11:261–2. 9. Conant MA, Opp KM, Poretz D, Mills RG. Reduction of Kaposi’s sarcoma lesions following treatment of AIDS with ritonovir. AIDS 1997; 11:1300–1. 10. De Milito A, Catucci M, Venturi G, Romano L, Incandela L, Valensin PE, et al. Antiretroviral therapy with protease inhibitors in human immunodeficiency virus type 1- and human herpesvirus 8-coinfected patients. J Med Virol 1999; 57:140–4.
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Photographs and text from: M.C. Alraies, Department of Hospital Medicine Institute, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland Clinic, Cleveland Clinic 9500 Euclid Avenue, mail code A13, Cleveland, OH 44195, USA; A.H. Alraiyes, Department Pulmonary Diseases, Critical Care, & Environmental Medicine, Tulane University Health Sciences Centre, 1430 Tulane Avenue, SL-9, New Orleans, LA 70112, USA; C.M. Kabach, Department of Hospital Medicine, Cleveland Clinic, Cleveland, OH, USA; M. Auron, Department of Hospital Medicine, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland Clinic, Cleveland, OH, USA. email:
[email protected]
Conflict of interest: None declared.
