Fatal pulmonary hypertension and resolving immune-complex glomerulonephritis in mixed connective tissue disease. A case report and review of the literature.

Mixed connective tissue disease (MCTD) has been characterized as a benign rheumatic syndrome with a favorable response to immunosuppressive therapy. Serious renal and pulmonary involvement are reported to be rare in MCTD. We are describing a female adolescent with MCTD in whom fatal cor pulmonale developed due to recurrent thromboembolic primary pulmonary hypertension. Death occurred after two years of therapy with prednisone and azathioprine for an immune-complex glomerulonephritis. Paramesangial and intramembranous electron-dense deposits had been identified in several glomeruli at the start of treatment. Improved renal function and apparent histologic improvement were demonstrated four months after the institution of prednisone and azathioprine therapy, and stable renal function was maintained until death. The pulmonary hypertension was progressive and apparently not altered by either the prednisone or azathioprine. At necropsy, there was no evidence of a pulmonary arteritis or vascular immune-complex deposition to account for the recurrent thromboembolic lesions in the small pulmonary arteries and arterioles. This is a clinical course not previously described in patients with MCTD and may represent an extreme of the clinical spectrum of this syndrome.

Acute myelomonocytic leukemia with oral manifestations: report of case.

A patient seeking dental care was diagnosed with acute myelomonocytic leukemia and referred to a hematologic-oncologic service for confirmation and treatment. The typical oral findings and a discussion of the disease process of acute leukemia are presented.

Pulmonary hyaline membrane disease occurring in the course of VM-26 therapy.

During treatment with VM-26 a 20-year-old patient with metastatic medulloblastoma developed an acute, fulminating pulmonary illness. Open lung biopsy revealed advanced alveolar hyaline membrane formation with interstitial round cell infiltrate. This is the first reported case of podophyllotoxin-related pulmonary disease.

Multiple myeloma associated with immune thrombocytopenic purpura.

Thrombocytopenia in patients with multiple myeloma is usually due to chemotherapy or marrow replacement with myeloma cells. Two patients with multiple myeloma who fulfilled criteria for the diagnosis of immune thrombocytopenic purpura are presented. The etiologic and therapeutic implications of this unusual association are discussed.

New pulmonary infiltrates in granulocytopenic cancer patients being treated with antibiotics.

During a 5 1/2-year period, 34 of 829 episodes of granulocytopenia during which broad spectrum antibiotics were being administered for fever and/or infections were complicated by the development of new pulmonary infiltrates. In 12 patients the infiltrates were due to fungal pneumonia, while in 6 patients the infiltrates were due to a variety of other causes. In the remaining 16 cases the etiology of the infiltrates was not determined. Time to development of infiltrate, radiographic appearance of the infiltrate, patient temperature and absolute granulocyte count failed to predict the etiology of the infiltrate. Conversely, development of the infiltrate or its radiographic progression in the absence of bone marrow recovery correlated significantly with the diagnosis of fungal pneumonia. While empiric alterations of antibiotics at the time that the infiltrate appeared were not associated with improved survival, the early use of amphotericin B was associated with a significant decrease in fatal fungal pneumonia. We suggest that the diagnostic and therapeutic approach to the febrile, granulocytopenic patient who develops a new pulmonary infiltrate while receiving broad spectrum antibiotic therapy may be guided by the state of marrow recovery at the time of infiltrate appearance. Patients developing an infiltrate coincident with granulocyte recovery may be managed conservatively while patients whose infiltrate develops or progresses in the absence of granulocyte recovery should be considered to be at high risk for fungal pneumonia and if possible undergo a diagnostic lung biopsy and/or empiric antifungal therapy.

Bone marrow transplantation for good-risk patients with leukemia in a university affiliated hospital.

Of the first 14 patients with acute or chronic leukemia to undergo bone marrow transplantation at our hospital, 9 (64%), all good-risk, are still alive in remission at 18 to 42 months of follow-up (mean, 29 months) with their Karnofsky performance status between 80% and 100%. The conditioning regimen of fractionated-dose irradiation and high-dose chemotherapy eradicated their disease; only two patients relapsed after transplantation. Toxicity was acceptable. Acute graft-versus-host disease developed in six patients (43%) (grade I or II in four, grade IV in two) and progressed to chronic graft-versus-host disease in four. Viral pneumonitis developed in three patients (21%), but none had idiopathic interstitial pneumonitis. The mean hospital charge was $54,355. These preliminary results suggest that good-risk patients with acute or chronic leukemia can be treated with bone marrow transplantation in a university affiliated hospital with appropriate staff and support facilities and achieve results comparable to those in research institutions at a competitive cost.

[Infectious complications from intraventricular route of administration in cancer patients]. / Powiklania zakazne wystepujace przy zastosowaniu zbiornika dokomorowego u chorych z choroba nowotworowa.

Intraventricular drug administration via the IR is often used in the therapy of leukemic and carcinomatous meningitis. We reviewed our 10-year experience with 32 patients, with IR placed for intraventricular chemotherapy to characterize infectious complications associated with IR. Infectious complications occurred in 9 patients (27.1%). Local cellulitis (S. aureus) occurred at the site of IR in 2 patients. Seven patients (21.8%) had 12 episodes of bacteriologically proven or clinically suspected meningitis, or positive IR CSF cultures without symptoms (4-P. acnes). While patients with local infection may require IR removal, more than half of those with meningitis may be treated with antibiotics alone without IR removal. Patients with positive cultures in the absence of symptoms may require no therapy at all.