|
Tanzania Journal of Health Research
Health User's Trust Fund (HRUTF)
ISSN: 1821-6404
Vol. 13, Num. 1, 2011, pp. 103-108
|
Tanzania Journal of Health Research, Vol. 13, No. 1, January, 2011, pp. 103-108
Recurrent, massive Kaposi’s
sarcoma pericardial effusion presenting without cutaneous lesions in an HIV
infected adult: A Case Report
Rodrick
Kabangila1, William Mahalu1, Nestory Masalu1,
Hyasinta Jaka1 and Robert N. Peck1,2*
1Bugando
University College of Health Sciences, Mwanza, Tanzania
2Weill Cornell Medical
College, New York, USA
Code Number: th11013
Abstract
In this report we describe the case of a
22-year-old female who presented to our hospital with a 2 week history of chest
tightness and easy fatigability. Examination and chest ultrasound revealed a
massive pericardial effusion with evidence of tamponade. A rapid test for HIV
was positive. Diagnostic and therapeutic pericardiocentesis was performed with
good clinical response and revealed serosanguinous, exudative fluid. According
to national guidelines, the patient was empirically treated for tuberculous
pericarditis. Recurrence of the pericardial effusion occurred after 2 weeks and
the cardiothoracic surgeons were consulted. Several days later, the patient was
taken to the operating theatre and a pericardial window was performed with
resultant drainage of over 5 litres of pericardial fluid. Visualization of the
pericardium revealed a purple, multinodular mass of about 4x6cm on the
epicardium consistent with Kaposi’s sarcoma of the pericardium. Five litres of
blood stained fluid were drained. Anti-tuberculosis treatment was stopped and
the patient was referred to the oncology unit. The patient was started on
antiretroviral treatment and Vincristine chemotherapy and the pericardial
effusion resolved completely after 6 cycles of chemotherapy. Kaposi sarcoma
should be considered as differential diagnosis in HIV/AIDS patient presenting
with massive pericardial effusion.
Keywords:
Kaposi’s sarcoma, pericardial effusion, HIV, tamponade, vincristine, Tanzania
Introduction
Pericardial
disease is common among HIV infected individuals worldwide with a reported
average incidence of 21% according to several echocardiography and autopsy
studies (Estok
& Wallach 1998). Most of this pericardial disease is mild and
self limited but severe pericardial disease also occurs.
Over
185 cases of cardiac tamponade among HIV infected individuals have been
reported in the literature (Gowda et al., 2003). In many of these cases,
the cause of cardiac tamponade was never determined. Tuberculosis is the most
commonly diagnosed cause of cardiac tamponade amongst HIV infected individuals,
particularly in Africa (Cegielski et al., 1994; Gowda et al., 2003).
Other commonly reported causes include bacterial infection, lymphoma and
Kaposi’s sarcoma (Gowda et al., 2003).
The
diagnosis and therefore treatment of pericardial disease among HIV infected
individuals remains difficult, particularly in resource limited settings. Due
to the high prevalence of tuberculosis as a cause of pericardial effusion
amongst HIV infected individuals, some have recommended that all large,
persistent pericardial effusions be treated as tuberculous pericarditis in the
absence of some obvious, other etiology Cegielski et al., 1994).
We
report the presentation, workup and treatment of a 22-year-old HIV infected
female who presented to Bugando Medical Centre in Mwanza, Tanzania, with a
massive pericardial effusion and cardiac tamponade. In this report, we describe
the presentation, workup, diagnosis and treatment of this patient who was found
to have a Kaposi’s sarcoma of the pericardium. We also review the literature
related to HIV and Kaposi’s sarcoma of the pericardium.
Case
Presentation
Bugando
Medical Centre (BMC) is an 850 bed referral hospital located in Mwanza city in
northwestern Tanzania on the southern border of Lake Victoria. In 2009, a
22-year-old female was referred to our hospital with a diagnosis of
cardiomyopathy. On review, the patient was found to have a history of chest
pain on exertion, awareness of heart beat, chest tightness and easy
fatigability for two weeks. On exam, the patient was afebrile with a pulse
rate of 130 beats per minute, a blood pressure of 104/74 mmHg and a respiratory
rate of 20 cycles per minute. No cutaneous or mucosal lesions were present.
Multiple, approximately 1-2 cm firm, non-tender, mobile lymph nodes were found
in the cervical, axillary and inguinal regions.
On
cardiac examination, the apex beat was not palpable and the heart sounds were
distant. The jugular vein was distended to the level of the ear with the
patient sitting in the upright position. Tender hepatomegaly was also present
and liver edge was palpable 6cm below the right costal margin. Lower extremity
pitting oedema was present to the level of the knees. The lungs were clear to
auscultation.
The
patient was admitted to the medical ward with a diagnosis of pericardial
effusion. A complete blood count revealed a white blood cell count of 11.4
cells/mm3 (with a differential of 69% neutrophils, 23% lymphocytes, 6% monocytes
and 1% eosinophils), a haemoglobin of 8.9 g/dl and a platelet count of 140
cells/mm3. The ESR was 40 mm/hr. Renal and liver function tests were all within
normal range. A rapid test for HIV was performed and was positive and the
patients CD4 count was reported as 320 cells/ul.
Chest
x-ray revealed an enlarged cardiac silhouette (cardiothoracic ratio 0.7) with a
globular shape. Echocardiogram revealed massive pericardial effusion with right
ventricular collapse. The echocardiogram was otherwise normal with no valvular
dysfunction or pericardial masses.
A
bedside therapeutic pericardiocentesis was performed and 400ml of
serosanguinous fluid were removed with almost immediate clinical improvement.
Two samples of the pericardial fluid were sent to the laboratory for
biochemistry, microbiology and cytology. The total protein of the fluid was
elevated at 60.6 g/l but the glucose was normal (7.1mmol/l). Gram staining and
acid fast staining of the fluid were both negative and bacterial cultures
revealed no growth. On cytology, one sample revealed acellular material but the
other revealed “red blood cells and white blood cells in the same ratio as
peripheral blood with no atypical cells.”
On
hospital day 3 the patient was empirically started on standard four drug
anti-tuberculous treatment and prednisone 40mg PO twice daily according to the
national regimen for treatment of TB pericarditis. She remained on the medical
ward for 7 days. On hospital day 7 the patient was discharged in good condition
through a local HIV clinic to commence counselling for the initiation of
antiretroviral therapy. One week later, 2 weeks after starting anti-TB
chemotherapy, the patient was started on antiretroviral therapy with
zidovudine, lamivudine and efaverenz at standard adult doses.
Two
weeks after discharge, the patient returned to BMC with complaints of
difficulty in breathing, generalized body malaise, night sweats and fevers as
well as weight loss. On examination, the patient had mild dyspnoea. Her blood
pressure was 117/64 mmHg and her pulse rate was 117. On cardiac examination,
the apex beat was once again not palpable and the heart sounds were distant. A
positive pulsus paradoxus and Ewart’s sign were noted on examination.
Repeat
chest x-ray was unchanged from the prior admission with and enlarged, globular
cardiac silhouette but clear lungs. A repeat bedside chest ultrasound revealed
recurrent massive pericardial effusion. A second therapeutic pericardiocentesis
was performed and 1 litre of serosanguinous fluid was removed. Due to the
recurrence of the patient’s pericardial effusion and the apparent lack of
response anti-tuberculosis therapy, the medical team consulted the hospitals
cardiothoracic surgeon.
On
hospital day 21, the patient was taken to the operating theatre. An
anterolateral thoracotomy was made in the left fourth intercostals space. The
parietal pleura was normal with 100ml of effusion. The pericardium bulged into
the incision, obscuring the lung which was compressed posteriorly. The
pericardium was punctured and 20ml of serous fluid were aspirated. A
longitudinal incision was then made into the pericardial sac and a litre of
serosanguinous fluid was sucked out of the sac. This left a redundant
pericardium which was excised to create a window of 10cm in diameter.
On
visualization of the epicardium through the pericardial window, the surface of
the ventricular apex was seen to be studded with a purple, hemorrhagic
multinodular mass that measured approximately 3x5cm and was surrounded by
bloody fluid. An attempt to biopsy these nodules found them to be very friable;
easily bleeding and fixed to the epicardium. Thus biopsy was abandoned, an
intrapleural drain was inserted and the thoracotomy was closed. The patient had
an uneventful recovery from surgery. After draining 3 additional litres of
serosanguinous fluid, the intrapleural drain output became minimal and the
drain was removed on post-operative day 14.
Based
on the clinical history and operative findings, the patient was diagnosed with
Kaposi’s sarcoma of the pericardium. Repeat examination of the skin and mucous
membranes again revealed no mucocutaneous lesions. Anti-tuberculous therapy was
stopped. After recovering from surgery, the patient was transferred to the
hospital’s oncology ward under the care of the hospital’s oncologist.
On
the oncology ward, the patient received 6 cycles of vincristine given in 2
weekly intervals at a dose of 1.4mg/m². Doxorubicin was withheld due to the
pericardial involvement of the disease and the concern for cardiac toxicity. After
a total of 6 cycles of chemotherapy, the patient reported complete resolution
of her symptoms. A repeat chest x-ray revealed a normal cardiac silhouette.
The patient was last seen 3 months after the completion of her chemotherapy and
is doing well.
Discussion
In
this report we describe the case of a 22 year old HIV infected female who
presented to our hospital with massive, recurrent Kaposi’s sarcoma (KS)
pericardial effusion. This case illustrates many important points about
pericardial KS in patients in HIV.
Pericardial
disease is very common among HIV infected individuals. According to 15
echocardiographic and autopsy studies, the average incidence of pericardial
effusion in patients with HIV was 21% (Estok & Wallach, 1998). Most
pericardial effusions in patients with HIV are mild but over 185 cases of
cardiac tamponade due to massive or rapidly accumulating pericardial effusion
have been reported in the literature (Gowda et al., 2003). In our
patient, the pericardial effusion was both massive and recurrent. Despite
several attempts to drain the pericardial effusion, cardiac tamponade recurred
3 times. At the time of pericardial window, over 5 litres of pericardial fluid
were drained from the pericardial space.
The
most common cause of pericardial effusion among HIV infected individuals is
tuberculosis. According to several large case series of pericardial effusion
among patients with HIV living in the United States, Mycobacterium
tuberculosis was the most commonly identified cause of pericardial effusion
and accounted for 15-25% of all pericardial effusions (Estok & Wallach,
1998; Chen et al., 1999; Gowda et al., 2003). Tuberculosis is
even more prevalent as a cause of pericardial effusion in sub Saharan Africa.
In one study from Tanzania, 14/14 patients admitted to a referral hospital with
HIV and massive pericardial effusion who underwent pericardiocentesis had
tuberculous pericarditis (Cegielski et al. 1994). These results have led
to the recommendation that all cases of massive pericardial effusion in HIV
infected patients be treated as tuberculosis unless some other cause can easily
be identified.
Of
course, pericardial effusions can also be caused by many things other than
tuberculosis. In several studies from the United State of patients with HIV and
pericardial effusion the other leading causes were: non-tuberculous
mycobacteria (≈15%), bacterial (≈10%), lymphoma (≈5%) and KS
(≈5%). Other causes included Cryptococcus neoformans, Nocardia
asteroides, Aspergillus species, cytomegalovirus, and herpes
simplex. The cause of pericardial effusion was not determined in ≈20-40%
of cases ((Estok & Wallach, 1998; Chen et al., 1999). Very little
data is available in the literature about non-tuberculous causes of pericardial
effusion in sub Saharan Africa and more study is needed in this area.
Kaposi’s
sarcoma pericardial effusions can be massive and are often hemorrhagic, as in
this case (Stotka et al., 1989; Chyu et al., 1998; Atar et
al., 1999). In most cases, mucocutaneous KS lesions are present but there
has been at least one other reported case of pericardial KS reporting without
evidence of mucocutaneous KS (Chyu et al., 1998). For this reason, the
absence of mucocutaneous KS should not exclude the possibility of KS as the
cause of pericardial effusion.
The
diagnosis of KS as a cause of pericardial effusion is difficult in the absence
of surgical intervention. In 1 case from the United States, laboratory
investigation of the pericardial fluid was negative except for the presence of
red blood cells and the diagnosis of KS was only confirmed after autopsy (Stotka,
Good et al., 1989). In at least one report,
echocardiogram was used to visualize multinodular KS lesions on the apical
epicardium (Chyu et al., 1998). Pericardial KS typically involves the
epicardium (visceral pericardium) and not the parietal pericardium and can be
only visualized after the opening of the parietal pericardium in surgery or
autopsy.
Treatment
of Kaposi’s sarcoma pericardial effusion related to HIV infection involves a
combination of drainage, anti-retroviral therapy and chemotherapy as in this
case. Urgent drainage of KS pericardial effusions is indicated in any patient
signs of cardiac tamponade. This case illustrates how KS pericardial effusions
can be massive and can rapidly re-accumulate after pericardiocentesis. For this
reason, some have recommended early surgical intervention in patients with
massive KS pericardial effusions (Vijay et al., 1996). Antiretroviral
therapy is recommended in all patients with KS, regardless of the CD4 count.
Protease inhibitor based regimens and protease inhibitor regimens are equally
efficacious in inducing resolution of KS (Martinez et al., 2006). Chemotherapy
is indicated for patient with visceral KS as well as for some patients with
severe or disfiguring cutaneous KS. Many chemotherapy regimens have been used
for the treatment of KS but liposomal anthracyclines seem to be superior and
are first-line in most developed countries (Lee & Mitsuyasu, 1996;
Aldenhoven et al., 2006). In some developing countries such as Tanzania,
vincristine alone has been used as in this case due to the reasonable outcomes,
low cost and availability of this regimen (Mlombe, 2008). At our centre, the
vincristine would have been combined with doxorubicin if the pericardium had
not been involved.
In
conclusion, in this report we describe the case of a 22 year old female who
presented to our referral hospital in shock with cardiac tamponade and was
diagnosed with HIV and a massive pericardial effusion. The patient did well
with rapid pericardiocentesis, pericardial window, chemotherapy and
antiretroviral therapy. This case illustrates the difficulty of diagnosing KS
pericardial effusion in resources limited settings but also the good outcomes
that can be achieved with drainage, chemotherapy and antiretroviral therapy.
More research is desperately regarding pericardial effusion among HIV infected
individuals in resource-limited settings.
Acknowledgements
We would like to thank Dr.
Charles Majinge, Director of Bugando Medical Centre, for his support.
References
- Aldenhoven, M.,
Barlo, N. P. & Sanders, C.J. (2006) Therapeutic strategies for epidemic
Kaposi's sarcoma. International Journal of STD and AIDS 17, 571-578.
- Atar, S., Chiu, J.,
Forrester, J.S., & Siegel, R.J. (1999) Bloody pericardial effusion in
patients with cardiac tamponade: is the cause cancerous, tuberculous, or
iatrogenic in the 1990s? Chest 116, 1564-1569.
- Cegielski, J. P.,
Lwakatare, J., Dukes, C.S., Lema, L.E., Lallinger, G.J., Kitinya, J., Reller,
L.B., & Sheriff, F. (1994) Tuberculous pericarditis in Tanzanian patients
with and without HIV infection. Tubercle and Lung Disease 75, 429-434.
- Chen, Y.,
Brennessel, D., Walters, J., Johnson, M., Rosner, F., & Raza, M. (1999)
Human immunodeficiency virus-associated pericardial effusion: report of 40
cases and review of the literature. American Heart Journal 137, 516-521.
- Chyu, K.Y.,
Birnbaum, Y., Naqvi, T., Fishbein, M.C. & Siegel, R.J. (1998)
Echocardiographic detection of Kaposi's sarcoma causing cardiac tamponade in a
patient with acquired immunodeficiency syndrome. Clinical Cardiology 21,
131-133.
- Estok, L. &
Wallach, F. (1998) Cardiac tamponade in a patient with AIDS: a review of
pericardial disease in patients with HIV infection. Mount Sinai Journal of
Medicine 65, 33-39.
- Gowda, R.M. &
Khan, I.A. (2003) Cardiac tamponade in patients with human immunodeficiency
virus disease. Angiology 54, 469-474.
- Lee, F.C. &
Mitsuyasu, R.T. (1996) Chemotherapy of AIDS--related Kaposi's sarcoma. Hematology/Oncology
Clinics of North America 10, 1051-1068.
- Martinez, V.,
Caumes, E., Gambotti, L., Ittah, H., Morini, J.P., Deleuze, J., Gorin, I.,
Katiama, C., Bricaire, F., & Dupin, N. (2006) Remission from Kaposi's
sarcoma on HAART is associated with suppression of HIV replication and is
independent of protease inhibitor therapy. British Journal of Cancer 94,
1000-1006.
- Mlombe, Y. (2008)
Management of HIV associated Kaposi's sarcoma in Malawi. Malawi Medical
Journal 20, 129-132.
- Stotka, J.L., Good,
C.B., Downer, W.R. & Kapoor, W.N. (1989) Pericardial effusion and tamponade
due to Kaposi's sarcoma in acquired immunodeficiency syndrome. Chest 95,
1359-1361.
- Vijay, V., Aloor,
R.K., Yalla, S.M., Bebawi, M. & Kashan, F. (1996) Pericardial tamponade
from Kaposi's sarcoma: role of early pericardial window. American Heart
Journal 132, 897-899.
Copyright 2011 - Tanzania Journal of Health Research
|