Kaposi Sarcoma With Bilateral Chylothorax Responsive to Octreotide

Humzah Iqbal, MD

Department of Internal Medicine, University of California San Francisco, Fresno, CA, USA

Abstract

Kaposi sarcoma (KS) is a soft tissue malignancy of the endothelial cells that can rarely invade the thoracic duct and cause bilateral chylothorax. Treatment for chylothorax includes drainage and dietary modification. However, octreotide has been reported to improve chylothorax in some pediatric and post-operative cases. We present a case in which a 9-day course of octreotide led to an improvement of non-traumatic malignant chylothorax.

Abbreviation list

• AIDS: acquired immunodeficiency syndrome
• CT: computed tomography
• HIV: human immunodeficiency virus
• KS: Kaposi sarcoma

Introduction

Kaposi sarcoma (KS) is a malignant, multifocal, highly vascularized tumor of the endothelial cells that most commonly affects the skin but may also include the lymph nodes, mucosa, and viscera (1). KS is commonly associated with human immunodeficiency virus (HIV) and can occur at any CD4 count (2). In very rare cases, Kaposi sarcoma can invade the thoracic duct and cause chylothorax (3). Chylothorax occurs when lymphatic fluid accumulates in the pleural cavity and is usually seen after damage to the thoracic duct following trauma or cardiothoracic surgery. It can also be caused by malignancy, however, bilateral chylothorax secondary to KS is rare. Treatment of chylothorax usually involves drainage of the effusion and initiation of a low-fat diet. Octreotide has been reported to improve traumatic chylothorax, but has only been reported in non-traumatic etiologies in a handful of cases (4). Here, we present a case of bilateral chylothorax associated with KS, which was successfully treated with octreotide.

Case Presentation 

A 40-year-old man with a previous diagnosis of acquired immunodeficiency syndrome (AIDS) and KS presented to the emergency department due to progressive tachypnea, dyspnea, bilateral lower extremity edema, and expansion of his KS lesions onto his legs and genital region. His vital signs were significant for a respiratory rate of 25 breaths per minute and pulse of 109 beats per minute. The patient denied recent infection, trauma, or procedures. Chest X-ray showed a large left pleural effusion with midline shift and a small right pleural effusion (Figure 1).

Figure 1. Upright chest X-ray demonstrating large left pleural effusion with midline shift and small right pleural effusion.

Computed tomography (CT) scan of the chest showed large bilateral pleural effusions with collapse of the right lower lobe and partial collapse of the upper lobes bilaterally (Figure 2).

Figure 2. Representative view from computed tomography (CT) scan (axial plane) in lung windows showing bilateral pleural effusions.

The patient developed hypoxemia and underwent thoracentesis with a total of 1.5 liters of pink, milky fluid removed (Figure 3).

Figure 3. Image of pleural fluid obtained from thoracentesis demonstrating pink, milky appearance.

Bilateral PleurX catheters (PleurX; Iskus Health; London, United Kingdom) were placed for persistent drainage. Fluid studies showed a triglyceride count of 147 mg/dL on the right side and 153 mg/dL on the left side. The patient continued to self-drain when symptomatic and drained about 600 mL of light-colored opaque fluid from each side daily. Serum albumin levels decreased to about 2.0 g/dL over the next week with concurrent development of diffuse pitting edema in all four extremities and abdomen. He was started on a high-protein, low-fat diet consuming up to 6-7 nutritional protein supplements per day with little to no improvement in his clinical state or serum protein levels. Given the patient’s poor response to treatment and persistence of his pleural effusions, a trial of octreotide was initiated. The patient was given octreotide 100 mg three times per day. About 3 days after initiating therapy, the patient refrained from draining his PleurX catheters for the first time and the frequency of draining decreased over the remainder of the week due to improvement in symptoms. The fluid was noted to be less opaque and clearer with each drainage. The patient’s tachypnea and oxygen saturation also showed improvement. After day 9 of octreotide, the treatment was discontinued and repeat pleural fluid studies showed a triglyceride count of 69 mg/dL on the right side and 89 mg/dL on the left side. With the resolution of his chylothorax and improvement in oxygenation status as well as his edema, the patient was discharged and will follow up with Oncology for continuation of his KS treatment.

Discussion

KS is known as an AIDS-defining illness that can invade a variety of tissues in the body leading to manifestations beyond the classic skin lesions. It can cause unusual neurologic, cardiac, orbital, laryngeal, endocrine, and gastrointestinal complications in rare cases (5). We present a case of bilateral chylothorax as another rare potential complication of KS. Other reported cases have presented similarly to our patient, such as a case presented by Pennington et al. (6) which also described dyspnea and hypoxemia with transient but significant improvements in ventilation with serial chest drainage as well as repeated reaccumulation of the chylothorax. In their case, however, the patient died as a result of his condition. Other cases of presumed KS-induced chylothorax have also resulted in marked nutritional deficiencies as seen in our patient (7).

Treatment of chylothorax involves therapeutic thoracentesis, a low-fat diet that is high in medium-chain triglycerides which do not pass through the thoracic duct, and surgical correction or embolization of the defect (8). Though not a standard practice, the use of octreotide has been reported to improve chylothorax in some cases. The majority of these cases have been traumatic chylothorax following cardiothoracic surgery in adults or the pediatric population, or neonates with congenital chylothorax (8). There is a paucity of literature regarding octreotide in the management of malignant and other non-traumatic causes of chylothorax in the adult population. One case has been reported by Togashi et al. (9) which describes chylothorax secondary to idiopathic fibrosing mediastinitis that was treated successfully with octreotide. The exact mechanism is unknown, but as a somatostatin analogue, it may involve a decrease in splanchnic blood flow and subsequent reduction in lymphatic flow from the gastrointestinal system and through the thoracic duct (10-11). There is no standard protocol for the administration of octreotide, however, most studies report a 1-2 week course with recognizable improvements after 2-3 days of treatment, as seen in our patient (12).

Conclusion

Bilateral chylothorax is a rare manifestation of KS that can lead to respiratory failure, malnutrition, and death. We present a case of non-traumatic, malignant chylothorax that was treated successfully with octreotide, a somatostatin analogue. Further studies are necessary to elucidate the exact mechanism of its effect on chylothorax and to establish a standardized treatment protocol for the usage of octreotide in this condition. 

References

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Cite as: Iqbal H. Kaposi Sarcoma With Bilateral Chylothorax Responsive to Octreotide. Southwest J Pulm Crit Care Sleep. 2022;25(5):69-72. doi: https://doi.org/10.13175/swjpccs048-22 PDF

Co-Infection with Nocardia and Mycobacterium Avium Complex (MAC) in a Patient with Acquired Immunodeficiency Syndrome

Zahira Babwani DO

Kenneth Wojnowski Jr DO

Sunil Kumar MD

Broward Health Medical Center

Fort Lauderdale, FL USA

Abstract

We present a case in which a patient with acquired immunodeficiency syndrome (AIDS) and nocardiosis was found to have co-infection with Mycobacterium avium complex (MAC). Despite the fact that MAC is a known colonizer of the pulmonary system, ​ it is possible to have co-infection and a high degree of suspicion is necessary to ensure prompt treatment of both organisms. We wish to describe how radiologic findings were instrumental in guiding our differential diagnosis.

Case Report

History of Present Illness​: A 64-year-old man with history of alcohol and tobacco abuse presented with a chronic, productive cough for 5-6 months. Associated symptoms included shortness of breath and 30-pound weight loss. He denied all other symptoms.

Physical Exam​: Pertinent positives revealed temporal wasting, poor dental hygiene, oral thrush and diffuse rhonchi bilaterally. Initial vital signs were within normal limits.

Laboratory and Radiology​: Pertinent laboratory findings revealed leukocytosis with a left shift. Viral respiratory polymerase chain reaction (PCR) testing was negative. Human immunodeficiency virus (HIV) testing was positive with a CD4 count of 46 cells/mm³. QuantiFERON gold testing was negative. Sputum cultures, acid-fast bacilli (AFB) and blood cultures were obtained. Bronchoalveolar lavage (BAL) was performed with no evidence of Pneumocystis jirovecii (PJP). Chest X-ray (CXR) and computed tomography (CT) of the chest (Figure 1) revealed a multifocal right lung abscess with complex pleural fluid, empyema, nodular cavitary lesion in the left lower lobe and hilar lymphadenopathy.

Figure 1. Panel A: initial chest X-ray shows a complex infiltrate and effusion in the right lung. There is a cavitary lesion with air-fluid level vs lung abscess on the right. A nodule or consolidation is present in the left lung base. Panel B: A representative image from the initial CT of the chest showing a multifocal right lung abscess and complex pleural fluid.

Hospital Course: ​After admission, the patient was started on broad spectrum antimicrobials with vancomycin and piperacillin-tazobactam. A thoracentesis was performed due to right sided pleural effusion which yielded 65 cc of thick, purulent, green fluid. Thoracotomy with complete decortication of the right lung was performed with biopsies of the abscesses. Two 32-French chest tubes were placed due to the presence of multiple intraparenchymal lung abscesses, loculations, and empyema. Biopsy and pleural fluid cultures grew gram positive, beaded organisms which were later identified as nocardia, with no evidence of MAC or Mycobacterium tuberculosis (MTB). The patient was started on amikacin, meropenem and trimethoprim-sulfamethoxazole for newly diagnosed pulmonary nocardiosis. MAC prophylaxis was initiated due to his low CD4 count. After initiation of therapy for nocardiosis, three sputum AFB cultures began to stain positive. Since nocardiosis stains weakly positive for AFB, we initially did not suspect non-tuberculous Mycobacteria (NTM). Repeat CT scan of the chest (Figure 2) revealed ground glass opacities, nodular densities and both mediastinal and hilar lymphadenopathy.

Figure 2. Panel A: after initiation of treatment for nocardiosis, improvement of right empyema and cavitary lesion with bilateral patchy airspace disease right greater than left. Panel B: CT of the chest after initiation of treatment for nocardiosis, prominent lymph nodes in the hilar regions and mediastinum. less cavitation than the previous study. There are innumerable ground glass and nodular densities throughout both lungs, right greater than left.

Suspicion for active MAC co-infection was raised, the prophylactic dose of azithromycin was increased to the treatment dose, and ethambutol was initiated. After three weeks of intravenous amikacin, meropenem and trimethoprim-sulfamethoxazole the patient showed considerable improvement in his respiratory symptoms and was transitioned to oral trimethoprim-sulfamethoxazole for outpatient treatment of nocardiosis with continuation of ethambutol and clarithromycin for MAC.

Discussion

The Mycobacterium Avium Complex ​(MAC) is a Non-tuberculous mycobacterium (NTM) that is commonly found in patients with HIV and a CD4 count of less than 50. The diagnosis of NTM is challenging due to the fact that the organism is a known colonizer of the pulmonary system (1) ​. Supportive radiologic evidence is needed to distinguish colonization from active infection (2).

Common CT findings of nocardiosis include ground glass opacities, lung nodules, cavitation, pleural effusion and masses (3)​. The presence of mediastinal and hilar lymphadenopathy is the most common finding in immunosuppressed patients with MAC infection but is not​ a usual feature of pulmonary nocardiosis (3,4) ​. Our​ patient’s repeat CT scan showed mediastinal and hilar lymphadenopathy with improvement of cavitary lesions which suggests improvement of CT findings related to nocardiosis, but persistent findings related to NTM (5). This led us to believe that the patient was appropriately treated for nocardiosis, but with an underlying presence of active MAC infection that presented with atypical radiographic findings. As per the American Thoracic Society (ATS) guidelines for NTM pulmonary infection (6)​ ​, this patient’s pulmonary symptoms, radiological evidence on the chest CT, and positive AFB cultures from at least two separate expectorated sputum samples lends credibility to MAC as a true active infection in the setting of nocardiosis and AIDS. The patient was appropriately placed on clarithromycin and ethambutol as an outpatient, and our suspicions were confirmed for MAC with no evidence of MTB by PCR testing 5 weeks after initial AFB smears were collected.

Co-infection with Nocardiosis and MAC may be underestimated since they both often develop in immunocompromised hosts. MAC, along with other NTM species account for 20% of mycobacterium pulmonary infections in HIV infected patients (5)​. Nocardia accounts for less than 3% of pulmonary infections in HIV infected patients (5)​. A high degree of clinical suspicion is imperative to promptly treat infection with both organisms.

References

1. Young J, Balagopal A, Reddy NS, Schlesinger LS. Differentiating colonization from infection can be difficult Nontuberculous mycobacterial infections: Diagnosis and treatment. Patient Care. 2007. Available at: http://www.patientcareonline.com/infection/differentiating-colonization-infection-can-be-difficult-nontuberculous-mycobacterial-infections (accessed 10/3/18).
2. Trinidad JM, Teira R, Zubero S, Santamaría JM.Coinfection by Nocardia asteroides and Mycobacterium avium- intracellulare in a patient with AIDS. Enferm Infecc Microbiol Clin. 1992 Dec;10(10):630-1. [PubMed]
3. Kanne JP, Yandow DR, Mohammed TL, Meyer CA. CT findings of pulmonary nocardiosis. AJR Am J Roentgenol. 2011 Aug;197(2):W266-72. [CrossRef] [PubMed]
4. Erasmus JJ, McAdams HP, Farrell MA, Patz EF Jr. Pulmonary nontuberculous mycobacterial infection: radiologic manifestations. Radiographics. 1999 Nov-Dec;19(6):1487-505. [PubMed]
5. Benito N, Moreno A, Miro JM, Torres A. Pulmonary infections in HIV-infected patients: an update in the 21st century. Eur Respir J. 2012 Mar;39(3):730-45. [CrossRef] [PubMed]
6. Griffith DE, Aksamit T, Brown-Elliott BA, et al. An official ATS/IDSA statement: diagnosis, treatment, and prevention of nontuberculous mycobacterial diseases. Am J Respir Crit Care Med. 2007 Feb 15;175(4):367-416. [CrossRef] [PubMed]

Cite as: Babwani Z, Wojnowski K Jr, Kumar S. Co-Infection with Nocardia and Mycobacterium avium complex (MAC) in a patient with acquired immunodeficiency syndrome. Southwest J Pulm Crit Care. 2019;18(1):22-5. doi: https://doi.org/10.13175/swjpcc123-18 PDF

32 Year Old Man with “Community-Acquired” Pneumonia

Jill K. Gersh, M.D., MPH¹, Michelle K. Haas MD^2,3,4

¹Department of Medicine, University of Colorado Denver, Aurora, CO; ²Denver Health Medical Center, Denver, CO; ³Denver Metro Tuberculosis Clinic, Denver, CO; ⁴Division of Infectious Diseases, Department of Medicine, University of Colorado Denver, Aurora, CO

Corresponding author: Jill Gersh, M.D., MPH Phone: 303-602-5052 Fax: 303-602-5055. Email: JILL.GERSH@UCDENVER.EDU

All authors declare they have no conflicts of interest to disclose.

Abstract

Background: Community-acquired pneumonia is a common reason for hospital admission; however underlying pathogens vary depending on host immunity and circulating pathogens in the community.

Case Summary: A 32 year old man from Malawi presented with community-acquired pneumonia. After failing outpatient management, he was admitted and found to have underlying HIV disease. His diagnostic work up was initially inconclusive for M. tuberculosis (TB) and thus his diagnostic evaluation and treatment focused on other etiologies. He was ultimately diagnosed with TB after an invasive procedure and had a rapid clinical response after initiating TB treatment.

Conclusion: Both failure to recognize that TB can present with a syndrome similar to bacterial pneumonia and over-reliance on diagnostic testing delayed the diagnosis of TB. Delays in diagnosis contributed to substantial morbidity and risked nosocomial transmission.  Despite declining incidence in the US, providers should remain cognizant of diagnostic limitations for TB disease and have a low threshold for empiric treatment.

Introduction

Community-acquired pneumonia (CAP) is a common reason for presentation to care. The epidemiology of CAP can vary depending on the patient’s community of origin and underlying co-morbidities (1). We present a case of a 32 year old man who presented with CAP in whom his diagnosis was delayed due to failure to fully consider these factors.  

Case

A 32 year old man from Malawi[1] presented to the emergency department (ED) with cough and dyspnea that failed to respond to a 5 day course of azithromycin. Chest radiography (CXR) was performed (Figure 1), demonstrating right middle lobe consolidation with ipsilateral hilar lymphadenopathy (LAD).

Figure 1. PA view of the chest demonstrating right middle lobe consolidation and ipsilateral hilar lymphadenopathy at the time of his first ED presentation and approximately 10 days into his illness.

He was diagnosed with CAP and discharged with a 7 day course of amoxicillin-clavulanate. His symptoms progressed with fevers, and weight loss.  He presented for the second time to the ED and repeat CXR showed worsening right-sided hilar LAD and right middle lobe consolidation (Figure 2).

Figure 2. PA view of the chest demonstrating worsening of right middle lobe consolidation and right sided hilar lymphadenopathy at the time of admission to the hospital and approximately 17 days into his illness.

A rapid HIV test was positive and his CD4 count was 60 cells/µL. He was admitted and started on ceftriaxone, azithromycin and trimethoprim-sulfamethoxazole daily. He was placed on respiratory isolation and three sputum samples for acid-fast bacilli (AFB) smear and culture were collected, all of which were AFB smear negative. He then underwent bronchoscopy and his bronchoalveolar lavage smear was negative for AFB. His tuberculin skin test (TST) was negative as was an interferon gamma release assay (IGRA). He was then removed from respiratory isolation.

He continued to worsen with daily fevers as high as 43ºC while antimicrobial coverage was broadened to vancomycin and cefepime. He eventually underwent mediastinoscopy and lymph node (LN) biopsy. The following day LN tissue was positive for AFB and probe identified Mycobacterium tuberculosis (TB). Twenty-nine days after his initial presentation and 15 days into his hospitalization he was started on anti-tuberculosis therapy with isoniazid, rifampin, pyrazinamide and ethambutol. His cough improved within 2 days, his fevers were gone by day 4 and he was discharged. All sputum cultures grew TB. Antiretroviral therapy was initiated five weeks into his TB treatment. He had an excellent clinical and radiographic response (Figure 3) and completed 9 months of TB treatment.

Figure 3. PA view of the chest after 9 months of treatment for M. tuberculosis. Noted here is resolution of right sided hilar lymphadenopathy and resolution of his right middle lobe consolidation with some residual scarring noted. Sputum culture converted at 2 months.

Diagnosis: Pulmonary tuberculosis.

Discussion

TB is the leading cause of death among HIV-infected individuals globally and the leading cause of morbidity in HIV-infected individuals (2). TB can present as an acute pneumonia with rapid progression of disease including sepsis and respiratory failure. Cough may not be a prominent feature and may be of less than two weeks duration. Additional signs and symptoms include fevers, night sweats, weight loss, hepatosplenomegaly, and lymphadenopathy. Individuals with CD4 counts < 100 cells/µL are more likely to present with disseminated disease and less likely to have cavitary disease. HIV-infected patients are also more likely to present with AFB smear negative disease even when severely ill (3). Chest radiograph findings vary from normal appearing films to hilar lymphadenopathy, diffuse infiltrates, and lobar consolidation.

TST and IGRAs are often negative and serve as poor screening tools for active disease. Up to 25% of individuals may have a negative TST or IGRA while having active disease, particularly HIV-infected individuals with advanced immunodeficiency (4). A negative result should never lower the clinical suspicion for active TB.

Delays in TB treatment are a major contributor to excess mortality in HIV-infected patients (2). The importance of early empiric treatment in HIV-infected individuals cannot be overstated. The World Health Organization (WHO) published guidelines in 2007 for the management of HIV-infected individuals suspected of having TB (5). While WHO guidelines are developed for low resource settings, these guidelines have relevance in the U.S. when managing patients with HIV who have lived or traveled to areas with a high burden of TB. 

The failure to recognize that his clinical syndrome of CAP included TB as the underlying pathogen led to delayed treatment, prolonged hospitalization and risked nosocomial transmission. One unintended consequence of the success of our TB control programs may be the growing lack of clinical experience with TB among our providers. More broadly, how much of what we do as U.S. healthcare providers is because we can, and instead of what we should? Imagine if he couldn't get a mediastinoscopy and biopsy. Is it possible that his treatment course would have been improved by a lack of these resources?  We would do well to learn from our colleagues practicing in resource limited settings where prescribing empiric TB treatment and assessing for a clinical response is standard of care. In this patient’s case, less really would have been more.

References

1. Nyamande K, Lalloo UG, John M. TB presenting as community-acquired pneumonia in a setting of high TB incidence and high HIV prevalence. Int J Tuberc Lung Dis. 2007;11(12):1308-13. [PubMed] 
2. Wong EB, Omar T, Setlhako GJ, et al. Causes of death on antiretroviral therapy: a post-mortem study from South Africa. PloS one. 2012;7(10):e47542. [CrossRef] [PubMed]
3. Elliott AM, Halwiindi B, Hayes RJ, Luo N, Tembo G, Machiels L, Bem C, Steenbergen G, Pobee JO, Nunn PP, et al. The impact of human immunodeficiency virus on presentation and diagnosis of tuberculosis in a cohort study in Zambia. J Trop Med Hyg. 1993;96(1):1-11. [PubMed] 
4. Cattamanchi A, Ssewenyana I, Davis JL, Huang L, Worodria W, den Boon S, Yoo S, Andama A, Hopewell PC, Cao H. Role of interferon-gamma release assays in the diagnosis of pulmonary tuberculosis in patients with advanced HIV infection. BMC Infect Dis. 2010;10:75. [CrossRef] [PubMed]
5. Improving the diagnosis and treatment of smear-negative pulmonary and extrapulmonary tuberculosis among adults and adolescents: recommendations for HIV-prevelent and resource constrained settings. Geneva: World Health Organization;2007.

Acknowledgements

The authors wish to thank Carolyn Welch, MD for her thoughtful review of this case report.

Reference as: Gersh JK, Haas MK. 32 year old man with "community-acquired' pneumonia. Southwest J Pulm Crit Care. 2013;7(6):355-9. doi: http://dx.doi.org/10.13175/swjpcc173-13 PDF