Intravascular lymphomatosis (malignant angioendotheliomatosis) presenting as pulmonary hypertension
Intravascular lymphomatosis is a rare lymphoma characterized by proliferation of malignant cells within the lumen of small blood vessels. We describe a case of intravascular lymphomatosis resulting in pulmonary hypertension, hypoxemia, and dyspnea. This lymphoma occasionally responds to combination chemotherapy, suggesting that pulmonary hypertension secondary to intravascular lymphomatosis may be reversible. Intravascular lymphomatosis should be considered in the differential diagnosis of pulmonary hypertension.
Intravascular lymphomatosis is a rare lymphoma with its primary clinical manifestations in the CNS and skin. It is characterized by proliferation of malignant cells within the lumen of small blood vessels including capillaries, veins, and arteries.[1,2] The disease frequently involves many organs, including the lung, liver, kidney, spleen, and adrenal glands. Organ involvement without cutaneous manifestations can occur and portends a poor prognosis.
Approximately 60 cases of intravascular lymphomatosis have been reported in the literature. The clinical features of the disease are varied and include cutaneous manifestations (plaques, nodules), neurologic symptoms (dementia, ischemic infarcts), hematologic abnormalities (hemolytic anemia), and nonspecific systemic symptoms (fever, weight loss, malaise). Lung involvement can be demonstrated pathologically at autopsy in approximately 60 percent of cases, although pulmonary symptoms are distinctly unusual at presentation.[1,2] This report describes the first case, to our knowledge, of intravascular lymphomatosis resulting in pulmonary hypertension, profound hypoxemia, and breathlessness.
A 79-year-old man, a nonsmoker, presented with a six-week history of dyspnea, fatigue, and intermittent fever. Physical examination revealed a blood pressure of 120/80 mm Hg, pulse 110 beats/min, and a respiratory rate of 32 breaths/min. Results of abdominal and neurologic examinations were normal; cardiopulmonary examination showed a right ventricular heave with no murmurs, and the lungs were clear.
Laboratory examination results revealed a hemoglobin of 11.5 g, WBC count of 5,400/cu mm, Westergren ESR of 68 mm/h, BUN of 47 mg/dl, creatinine of 1.6 mg/dl, bilirubin of 2.6 mg/dl (0.9 direct); arterial blood gas levels were: pH, 7.44; [PO.sub.2], 48 mm Hg; [PCO.sub.2], 21 mm Hg on room air; ANA positive at 1:64 (speckled pattern), and a reticulocyte count of 6.5...
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