Diffuse Gastrointestinal Hemorrhage as a Presentation of Systemic Kaposi Sarcoma

To the Editor:A 73-yr-old sephardic Jewish man was admitted to the hospital with acute gastrointestinal bleeding and fever. The patient had been diagnosed 18 months previously as suffering from systemic vasculitis. This diagnosis had been based on a neurological syndrome of mononeuritis multiplex with demyelinative features on sural nerve biopsy. The patient had been treated with plasmapheresis with some improvement, and later with immunosupressive therapy, which included corticosteroids (up to 60 mg prednison a day for about 1 yr) and azathioprine (75 mg a day for 6 months). Under this treatment his neurological condition had stabilized. The patient had a history of endoscopically diagnosed peptic ulcer disease of both the stomach and the duodenum, which had been treated with ranitidine for several years. On examination, the patient appeared restless and obtunded. His temperature was 37°C, and he was hemodynamically stable. The abdomen was distended, with absence of peristaltic sounds, and a mass could be palpated in the left lower quadrant. Rectal examination revealed melena. On examination of the extremities, a number of discreet, purple elevated lesions were noted on the ankles, feet, and on the left hand. Laboratory values showed a hemoglobin level of 8.3 g/dL, platelet count 95,000/mL, creatinine 430 mMol/L. Blood cultures yielded growths of two Gram-negative rods, Escherich coliand Proteus mirabilis. A serological test for HIV was negative. A chest film demonstrated bilateral pleural fluid, and an abdominal computerized tomography demonstrated a suspected space-occupying lesion above the cecum with inflammatory reaction of the adjacent peritoneum and retroperitoneum. The patient was treated with broad-spectrum antibiotics; blood was transfused, electrolyte derangements were corrected, and an endoscopy of the upper gastrointestinal tract was carried out, which demonstrated multiple grayish-purple elevated lesions on the gastric mucosa. The lesions bled profusely. The duodenal mucosa appeared intact. Biopsy was carried out both on the gastric lesion seen at endoscopy and on the cutaneous lesion described, both proving on pathological examination to be Kaposi sarcoma. Similar rectal lesions were found on colonoscopy, as well as a fungating tumor of the ascending colon, which for technical reasons could not be biopsied, but which macroscopically appeared to be carcinoma. Despite control of sepsis and gradual improvement of renal function, the patient continued to suffer from melena. Since the gastric lesions seemed not to be controlled with local measures, vitamin k was administered, as well as «-aminocaproic acid, ranitidine, and estradiol (for possible uremic coagulopathy), in the attempt to curtail loss of blood. In addition, chemotherapy was begun with intravenous vinblastine. Steroids were tapered and stopped, and azathioprine was discontinued. The patient’s condition continued to deteriorate with the appearance of myocardial ischemia and eventual demise. Kaposi sarcoma of the HIV-negative patient is considered a disease of elderly male patients, frequently of Jewish Mediterranean origin. The disease is frequently associated with immunosuppression (1, 2), an observation that is consistent with the currently held opinion regarding the pathogenetic role of a Herpes virus (Kaposi sarcoma-associated Herpes-like virus, or HHV-8) in its development (3). Immunosuppression-related Kaposi sarcoma has been shown to regress upon cessation of immunosuppressive therapy, when this is possible (4), and the disease has been shown to reappear on recurrent immunosuppressive therapy (5). Although skin involvement is the most common location of Kaposi sarcoma, extradermatological involvement is not rare, and gastrointestinal spread is a common site (6). Despite this, such involvement is usually asymptomatic, and severe hemorrhage caused by Kaposi is considered a rare occurrence (7). We present a case of severe recurrent hemorrhage secondary to diffuse gastrointestinal involvement, both gastric and colonic, in an immunosuppressed patient, who is also of Jewish Mediterranean origin. The therapeutic approach to this clinical setting includes, in addition to cessation of the immunosuppression, standard measures for controlling gastrointestinal hemorrhage, including H 2 blockers, sucralfate, sclerotherapy (8), and general supportive therapy. Radiotherapy has also been described as a potential treatment for hemorrhage caused by this vascular tumor (9). Attempts have been made to treat the disorder as a disseminated malignancy with chemotherapy. This has been associated with relatively good symptomatic response, even in cases in which there was no significant endoscopically demonstrated regression of lesions (10). Several confounding factors complicated the management of our patient, who appeared to have a second, undiagnosed colonic neoplasm, and who also had been diagnosed as suffering from peptic ulcer disease, causing gastrointestinal hemorrhage, years before the diagnosis of Kaposi sarcoma. Nonetheless, the diffuse gastric and colonic involvement, with demonstrated bleeding on endoscopy, indicate that Kaposi disease was indeed a major cause of hemorrhage in this case. The case underscores the importance of recognizing this unusual cause of gastrointestinal hemorrhage in the appropriate clinical setting.