Protein-losing enteropathy in acquired immunodeficiency syndrome due to intestinal Kaposi's sarcoma

Patients with the acquired immunodeficiency syndrome (AIDS) often have hypoalbuminemia. We report the case of a patient with AIDS in whom marked hypoalbuminemia developed due to a protein-losing enteropathy caused by small intestinal Kaposi's sarcoma--an entity not previously reported in AIDS. The patient presented with ankle edema, pleural effusions, and a decrease in albumin from 3.0 g/dL (30 g/L) to 1.7 g/dL (17 g/L) over one month. Protein-losing enteropathy was confirmed by a marked elevation in fecal alpha-1 antitrypsin, and extensive evaluation of the gastrointestinal tract revealed the source to be small intestinal Kaposi's sarcoma. A protein-losing enteropathy should be considered when hypoalbuminemia is encountered in a patient with AIDS.     

Kaposi's sarcoma in the acquired immunodeficiency syndrome

Kaposi's sarcoma is the most common malignancy seen in association with AIDS. Although the pathogenesis of AIDS-KS has not been clearly established, the clinical course and prognosis are closely related to the patient's immune status, prior history of opportunistic infection, and hematologic status. Treatment methods include local or regional radiation therapy, cytotoxic chemotherapy, and interferon therapy. Evaluation of the results of clinical therapeutic studies in this tumor should consider its natural history and peculiar biologic behavior and associated complications of AIDS. Effective treatment strategy would take into account the immune status of the patient, the rate of progression of tumor, the presence or risk of developing life-threatening opportunistic infections, associated hematologic or neurologic abnormalities, the toxicities of treatment, and the desire of the patient for treatment. Investigations of antiviral, antiproliferative, and immunomodulating agents singly or in combination currently are in progress. Additionally innovative approaches with biologic response modifies, adjunctive hematopoietins, and growth factor modulators may lead to newer approaches to the control of this malignancy.     

Kaposi's sarcoma associated with acquired immunodeficiency syndrome

AIDS-related Kaposi's sarcoma (KS) accounts for 15-20% of cases of AIDS. Clinical presentation is similar to that of classical KS but with an overall more aggressive evolution and cutaneous dissimilation. There is no particular predilection for the lower limbs and frequent lesions are located on the face. Most of the cases have occurred in homosexual males with a moderate immune deficiency (mean CD.4 = 300/mm3). This predilection for gay people has not yet received explanation and the physiopathology of KS is unclear. Treatment of KS with interferon or chemotherapy (Bleomycin) is effective in 30-50% of cases.     

Vinblastine therapy for Kaposi's sarcoma in the acquired immunodeficiency syndrome

Single-agent intravenous vinblastine, 4 to 8 mg/week, was used to treat 38 patients with Kaposi's sarcoma related to the acquired immunodeficiency syndrome. The dose was titrated in relation to the total leukocyte count. Ten patients had an objective response, and 19 had stable disease during therapy. Apart from expected modest neutropenia, toxicity was minimal. A lower response rate was seen in patients with anemia, an elevated erythrocyte sedimentation rate, or any lymphoma-like B symptom. Opportunistic infections were common regardless of type of response but were commoner in patients who did not respond. Vinblastine used in low doses weekly is effective in treating Kaposi's sarcoma related to the acquired immunodeficiency syndrome and has minimal associated toxicities.     

Pericardial effusion and tamponade

Pericardial effusion may occur as a result of a variety of clinical conditions, including viral, bacterial, or fungal infections and inflammatory, postinflammatory, autoreactive, and neoplastic processes. More common causes of pericardial effusion and tamponade include malignancy, renal failure, viral and bacterial infectious processes, radiation, aortic dissection, and hypothyroidism. It can also occur after trauma or acute myocardial infarction (as in postpericardiotomy syndrome following cardiac or thoracic surgery) or as an idiopathic pericardial effusion. Although pericardial effusion is common in patients with connective tissue disease, cardiac tamponade is rare. Among medical patients, malignant disease is the most common cause of pericardial effusion with tamponade. Table 1 shows the causes of pericardial tamponade. The effusion fluid may be serous, suppurative, hemorrhagic, or serosanguineous. The pericardial fluid can be a transudate (typically occurring in patients with congestive heart failure) or an exudate. The latter type, which contains a high concentration of proteins and fibrin, can occur with any type of pericarditis, severe infections, or malignancy. Once the diagnosis of pericardial effusion has been made, it is important to determine whether the effusion is creating significant hemodynamic compromise. Asymptomatic patients without hemodynamic compromise, even with large pericardial effusions, do not need to be treated with pericardiocentesis unless there is a need for fluid analysis for diagnostic purposes (eg, in acute bacterial pericarditis, tuberculosis, and neoplasias). The diagnosis of pericardial effusion/tamponade relies on a strong clinical suspicion and is confirmed by echocardiography or other pericardial imaging modalities. Alternatively, when the diagnosis of cardiac tamponade is made, there is a need for emergency drainage of pericardial fluid by pericardiocentesis or surgery to relieve the hemodynamic compromise. Following pericardiocentesis, it is necessary to prevent recurrence of tamponade. Intrapericardial injection of sclerosing agents, surgical pericardiotomy, and percutaneous balloon pericardial window creation are techniques used to prevent reaccumulation of pericardial fluid and recurrence of cardiac tamponade.     

Pericarditis with tamponade due to cytomegalovirus in the acquired immunodeficiency syndrome.

A 35-year-old male homosexual with AIDS presented with headache and fever and was found to have cryptococcal meningitis. During the patient's hospital course, his hemodynamic status deteriorated as a result of pericardial tamponade. Antemortem analysis of the fluid was unrevealing; however, postmortem examination of the pericardium revealed typical intranuclear inclusions of cytomegalovirus infection. Cytomegalovirus should be included in the differential diagnosis of pericardial effusion in patients with AIDS.     

The natural history of Kaposi's sarcoma in the acquired immunodeficiency syndrome

Kaposi's sarcoma is a multifocal systemic neoplasm histologically characterized by proliferating fibroblastic and microvascular elements. Initial signs include macules, papules, or nodules on the skin or mucosal surface. Lesions are frequently found on the trunk, arms, and head and neck. In general, sites of involvement and tumor load do not correlate with prognosis. A general decrease in the functional capacities of T and B cells is found in most patients. Kaposi's sarcoma is reported as the initial manifestation of the acquired immunodeficiency syndrome (AIDS) in approximately 30% of cases. Most cases are in men, although it has been reported in all risk groups. Kaposi's sarcoma in AIDS is more frequent among whites and homosexuals than blacks and intravenous drug abusers. Overall mortality is approximately 41%, with over 60% of patients alive at 1 year and 50% at 22 months. Overall survival is 18 months; however, some patients who have had the disease for 3 to 4 years are still doing well.     

Kaposi's sarcoma causing pulmonary infiltrates and respiratory failure in the acquired immunodeficiency syndrome

Although an aggressive form of Kaposi's sarcoma often develops in patients with the acquired immunodeficiency syndrome, most patients die due to opportunistic infections rather than the direct effects of this tumor. Because Kaposi's sarcoma has caused pulmonary dysfunction in a number of our patients, we attempted to characterize features of pulmonary dysfunction induced by Kaposi's sarcoma. In 66 patients with Kaposi's sarcoma treated between 1982 and 1984 there were 30 episodes of pulmonary dysfunction that resulted in a biopsy. Six episodes were due to pulmonary Kaposi's sarcoma alone, and 6 additional episodes were due to Kaposi's sarcoma and associated opportunistic infections. Clinical and radiologic features of pulmonary Kaposi's sarcoma an infection were indistinguishable. Pulmonary Kaposi's sarcoma could only be documented in large tissue sections available from open-lung biopsy or autopsy samples. Because chemotherapy or radiation therapy appears to provide palliation, clinicians should recognize Kaposi's sarcoma as a cause of pulmonary disease in patients with the acquired immunodeficiency syndrome.     

Upper airway obstruction secondary to acquired immunodeficiency syndrome-related Kaposi's sarcoma

A 45-year-old man with a history of intravenous drug use presented with acute respiratory distress. A pulsus paradoxus of 42 mm Hg, accessory respiratory muscle use and stridor were present. Examination of the oropharynx revealed multiple 1 to 2 cm purple lesions of the gingiva and hard palate. A purple tumor mass in the posterior pharynx obstructed the view of the larynx. An emergency tracheostomy was performed resulting in hemorrhage into the respiratory tract. Autopsy revealed disseminated Kaposi's sarcoma and large blood clots in the trachea and main stem bronchus. This case illustrates the occurrence of life threatening involvement of the upper aerodigestive tract with Kaposi's sarcoma and hemorrhagic complications resulting from surgical manipulation.     

Treatment of Kaposi's sarcoma in acquired immunodeficiency syndrome with an alternating vincristine-vinblastine regimen

Twenty-four patients with acquired immunodeficiency syndrome and Kaposi's sarcoma (KS) were treated with a regimen alternating vincristine with vinblastine on a weekly basis. Objective responses to this regimen were observed in nine patients (45%), and seven (35%) had stabilization of progressive KS. Few toxic effects were observed. Alternating vincristine-vinblastine therapy is well-tolerated and effective palliative treatment for KS in acquired immunodeficiency syndrome.     

Gastrointestinal Kaposi's sarcoma in patients with acquired immunodeficiency syndrome. Endoscopic and autopsy findings

Homosexual men with acquired immunodeficiency syndrome and Kaposi's sarcoma (KS) have been studied to define the extent of gastrointestinal KS. In 50 patients examined by both upper endoscopy and flexible fiberoptic sigmoidoscopy, 20 (40%) had visible lesions consistent with KS. Ten patients (20%) had upper and lower tract involvement, 6 (12%) patients had upper tract involvement alone, and 4 (8%) had lower tract disease alone. Only 23% of endoscopic biopsies of KS lesions were positive for KS. Gastrointestinal KS was not more frequent in patients with nodal KS (41%) than in patients with skin KS (50%). No clinical sequelae of gastrointestinal KS lesions were seen. Mortality was significantly greater in those patients with endoscopic evidence of gastrointestinal KS. Postmortem examination of 13 men with KS and acquired immunodeficiency syndrome revealed abdominal visceral involvement in 10 patients (77%). Antemortem endoscopic findings correlated with subsequent autopsy data in 5 patients. Our findings suggest that visceral involvement, though rarely symptomatic, may be associated with a poorer prognosis.     

Treatment of Kaposi's sarcoma and thrombocytopenia with vincristine in patients with the acquired immunodeficiency syndrome

Kaposi's sarcoma occurs in about one third of patients with the acquired immunodeficiency syndrome. Although in some patients the tumor is principally a cosmetic problem, other patients have progressive disease with significant morbidity. Twenty-three patients with Kaposi's sarcoma related to the acquired immunodeficiency syndrome were treated with vincristine. Three patients had a coexisting immune thrombocytopenia. Of the 18 patients evaluable for response, 11 had a partial response and 7 had a minor response. The median duration of partial response was 4 + months. All 3 thrombocytopenic patients developed a significant increase in platelet count, which in 2 was sustained with continued treatment for 6 and 9 months, respectively. We conclude that vincristine has antitumor activity in the epidemic form of Kaposi's sarcoma and that it is also effective in the treatment of associated immune thrombocytopenia.     

Echocardiographic detection of kaposi's sarcoma causing cardiac tamponade in a patient with acquired immunodeficiency syndrome

Pericardial effusions are common in patients with acquired immunodeficiency syndrome (AIDS). The differential diagnosis is diverse, and in most cases the etiology cannot be established. A cardiac tamponade was diagnosed in a 32-year-old male with AIDS and systemic Kaposi's sarcoma. Transthoracic echocardiography revealed a large pericardial effusion with right atrial collapse and a mobile multilobular mass at the apex protruding into pericardial space. Autopsy showed that this mass was Kaposi's sarcoma confined to the epicardial fat. This is the first case of cardiac Kaposi's sarcoma detected premortem by echocardiography.