Pulmonary venoocclusive disease in pregnancy.

A 23-year-old woman in her 27th week of gestation presented with clinical findings of progressive pulmonary hypertension. After cardiac catheterization she went into labor and was delivered by cesarean section. She died shortly thereafter from right heart failure. Pulmonary venoocclusive disease was found at autopsy. Hemodynamic changes during pregnancy, labor, delivery, and the postpartum period may have contributed to her deterioration and death. This is the first description of pulmonary venoocclusive disease in pregnancy.

Pulmonary oxalosis caused by Aspergillus niger.

A case report involving a pleuropulmonary mixed bacterial infection in association with Aspergillus niger is described. The patient responded to a combination of antibiotics, aerosolized and intravenously administered amphotericin B, and surgery. Aspergillus niger appeared to be a secondary invader and caused lung damage by the production of oxalic acid.

Pulmonary veno-occlusive disease associated with microangiopathic hemolytic anemia and chemotherapy of gastric adenocarcinoma.

A 68-year-old man was treated with FAM (5-fluorouracil, doxorubicin [Adriamycin], mitomycin-C) for metastic gastric adenocarcinoma. Twelve months later while in complete clinical remission, pulmonary hypertension and microangiopathic hemolytic anemia were recognized, progressed, and ended in his demise 6 months later. At necropsy, minimal residual cancer and severe pulmonary veno-occlusive disease was found. Pulmonary veno-occlusive disease may occur in association with microangiopathic hemolytic anemia and cancer chemotherapy.

Invasive pulmonary aspergillosis associated with aspergilloma in sarcoidosis.

We have described two patients with corticosteroid-treated sarcoidosis and with autopsy-proven aspergilloma and diffuse invasive pulmonary aspergillosis. In one, growth of the fungus within a cyst was probably followed by progression to diffuse pulmonary infection. In the second, lung necrosis from invasive infection may have led to the formation of the mycetoma found at autopsy.

Pulmonary disease as a complication of chlorozotocin chemotherapy.

Two of 200 patients treated with chlorozotocin have evidenced interstitial pulmonary disease. The cumulative doses of chlorozotocin were 480 and 670 mg/m2 when symptoms developed. Pulmonary function tests in both patients showed impaired diffusion, and an alveolar-arterial gradient which worsened with exercise. Open-lung biopsy in one patient showed typical interstitial inflammation and early fibrosis, and excluded lymphangitic spread of tumor. Discontinuation of chlorozotocin resulted in either improvement or stabilization of the pulmonary disease.

Hemochromatosis heart disease: an unemphasized cause of potentially reversible restrictive cardiomyopathy.

Cardiac involvement in hemochromatosis typically results in congestive cardiomyopathy; a restrictive cardiomyopathy due to hemochromatosis is distinctly rare. A restrictive cardiomyopathy, which developed in the patient described in this report, was due to hemochromatosis which mimicked constrictive pericarditis clinically, echocardiographically and hemodynamically, and resulted in a thoracotomy for attempted surgical therapy. The fact that hemochromatosis represents the only cause of a restrictive cardiomyopathy that is potentially reversible by medical therapy makes early recognition of hemochromatosis heart disease important.

Familial erythrophagocytic lymphohistiocytosis: treatment with vinblastine-loaded platelets.

Identical twin Caucasian boys, age 3 months, were seen with fever of unknown origin, hepatosplenomegaly, and pancytopenia. The diagnosis of familial erythrophagocytic lymphohistiocytosis (FEL) was suspected after examination of Twin A's bone marrow and confirmed by an open liver biopsy of Twin B. Twin A died shortly after diagnosis despite treatment with vincristine and prednisone. At autopsy, the diagnosis was confirmed. Twin B responded initially to a three-week course of weekly vincristine and daily prednisone, but symptoms soon recurred. In an effort to enhance delivery of chemotherapy to the active macrophage target, platelets were loaded with vinblastine and then administered intravenously to th patient every 7-10 days. There was an encouraging response reflected by the disappearance of symptoms and the return of peripheral blood count to the normal range, although increased number of histiocytes was still demonstrable in his bone marrow. After nine weeks, he lapsed completely and became refractory to treatment. He died of pseudomonas sepsis four months after diagnosis. This is the first known attempt to deliver a chemotherapeutic agent directly to the macrophages in treating this disease and represents an interesting concept that merits further exploration.

Parathyroid adenoma with coexistent sarcoid granulomas. A hypercalcemic patient.

A 56-year-old woman had hypercalcemia. Clinical symptoms and chest roentgenograms were suggestive of sarcoidosis. Results of laboratory studies, however, were compatible with primary hyperparathyroidism. At surgical exploration of the neck, a parathyroid adenoma was found. Microscopically, sarcoid-like granulomas were present within the adenoma. The association between hypercalcemic sarcoidosis and primary hyperparathyroidism is reviewed, and the importance of this finding is discussed.

Sarcoidosis of anterior mediastinal nodes, pancreas, and uterine cervix: three unusual sites in the same patient.

Sarcoidosis rarely involves anterior mediastinal nodes, pancreas, or uterine cervix. A 47-yr-old woman with sarcoidosis was found to have enlargement of the anterior mediastinal lymph nodes with "eggshell" calcifications on a lateral chest roentgenogram. This finding was confirmed by lateral tomography and thoracic computerized tomography. A review of 100 lateral chest roentgenograms of patients with documented sarcoidosis failed to reveal other examples of anterior mediastinal node enlargement. At laparotomy for total abdominal hysterectomy, a mass in the head of the pancreas and diffuse, nodular enlargement of this organ was found. A pathologic study of the pancreas and uterine endocervix demonstrated noncaseating granulomas. Sarcoidosis in these 3 unusual sites is discussed.

Medial calcinosis of Mönckeberg. A review of the problem and a description of a patient with involvement of peripheral, visceral and coronary arteries.

Massive medial calcific deposits (Mönckeberg's calcinosis) are described in the peripheral and visceral arteries, and similar but small-sized deposits in the coronary arteries of a 41 year old woman with diabetes mellitus. Although observed by roentgenogram fairly commonly during life in the muscular arteries of the legs in middle-aged men, medial calcinosis infrequently involves the visceral arteries and has never, to our knowledge, been documented in the coronary arteries. Although it may be associated with intimal atherosclerosis, medial calcinosis, per se, does not obstruct the lumens of the arteries and, therefore, does not lead to symptoms or signs of limb or organ ischemia. The cause of medial calcinosis remains a mystery, but it appears to affect people with diabetes more frequently than those without.