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Biomedical subjects

A Saven

Publications and source records attributed to A Saven.

68 records · Page 4Linked to original sources
PubMed1992

2-Chlorodeoxyadenosine: an active agent in the treatment of cutaneous T-cell lymphoma.

Cutaneous T-cell lymphomas are disfiguring malignant lymphoproliferative disorders for which standard therapy has been principally palliative. 2-Chlorodeoxyadenosine (2-CdA), a new purine analogue resistant to degradation by adenosine deaminase that has substantial activity against lymphoid neoplasms, was administered to 16 patients with cutaneous involvement by T-cell lymphoma. All patients had failed topical treatment modalities and/or systemic therapies. Fifteen patients were evaluable; one patient was not evaluable due to incomplete therapy and follow-up. The overall response rate was 47%. Three of 15 patients (20%) achieved complete responses and four of 15 patients (27%) achieved partial responses. The median duration of response was 5 months. One patient remains in unmaintained complete remission at 52+ months. Therapy was well tolerated. Myelosuppression was the principal toxicity encountered, occurring in 8 of 15 (53%) patients. 2-CdA is an effective new agent for the treatment of cutaneous T-cell lymphoma and warrants further study both as a single agent and in combination regimens.

2-Chloroadenosine↗
PubMed1992

2-Chlorodeoxyadenosine treatment of low-grade lymphomas.

PURPOSE: Because of the need to identify effective new agents in the treatment of non-Hodgkin's lymphoma and because of the high activity of the purine analog 2-chlorodeoxyadenosine (2-CdA) against chronic lymphocytic leukemia and hairy cell leukemia, a phase II trial of 2-CdA was initiated in patients with low-grade lymphocytic lymphomas. PATIENTS AND METHODS: Forty patients with low-grade lymphocytic lymphomas including diffuse small lymphocytic, follicular small-cleaved, and follicular mixed histologies were enrolled onto the study. Conventional therapies had failed in all patients, and six patients had lymph node biopsies showing evidence of histologic evolution to a higher-grade lymphoma. A total of 107 courses of 2-CdA were administered. There were 27 males and 13 females. The median age was 59 years (range, 37 to 80 years). Patients had received a median of three prior therapies (range, one to six therapies). RESULTS: An overall response rate of 43% was achieved, with eight patients experiencing complete responses (CRs) and nine patients experiencing partial responses (PRs). The duration of responses ranged from 1 to greater than 33 months without maintenance therapy (median duration of response, 5 months). Histology and prior therapy history did not seem to correlate with responses. Significant toxicity was limited to bone marrow suppression; 18% of patients developed neutropenia, and 30% developed thrombocytopenia. CONCLUSIONS: This phase II trial demonstrates that 2-CdA is an effective antilymphocyte, antineoplastic agent with significant activity as a single agent in patients with recurrent or refractory low-grade lymphocytic lymphoma. Responses were achieved with an acceptable toxicity profile. Further trials of this agent in previously untreated patients and in combination regimens are indicated and will be developed.

2-Chloroadenosine↗
PubMed1992

Gaucher disease. Clinical, laboratory, radiologic, and genetic features of 53 patients.

We have reviewed our experiences with the clinical, laboratory, radiologic, and genetic features of 53 patients with Gaucher disease. Most were evaluated during early adult life, with a mean age of 33 years. Our patients were evaluated in a referral center, and therefore the data need to be interpreted with caution when applied to the general patient population, which includes a greater proportion of very mild cases. Thirty-nine patients were Ashkenazi Jews, 13 were non-Jewish and 1 was half-Jewish. The most common presenting symptom was bleeding related to splenomegaly and thrombocytopenia. The chronic symptoms, evaluated an average of 20 years after the diagnosis had been established, were mainly skeletal. Splenectomy had been performed in 43% of our patients and there was no evidence that this procedure accelerated the progression of liver and bone involvement. DNA from the patients was examined for 20 different mutations. The association between the 1226G/1226G genotype and a milder clinical course, and between the 1226G/84GG and 1226/1448C genotypes with more severe clinical manifestations, was confirmed. Repeated follow-up examinations in 29 patients revealed that in the majority of the patients, progression of the disease occurs during childhood, adolescence, or early adulthood with a marked tendency for stabilization thereafter. This observation suggests that Gaucher disease in most of the patients is not a relentless progressive disorder but a rather stable disorder during adulthood. The indications for the newly introduced intravenous enzyme replacement therapy as well as of future experimental treatments should be examined in the light of the natural history of the disease.

Adolescent↗
PubMed1991

Enzyme replacement therapy for Gaucher disease.

Four patients with moderately severe type I Gaucher disease were treated with commercially available mannose terminated glucocerebrosidase (Ceredase; Genzyme, Boston, MA) for up to 13 months. The enzyme was administered at the rate of three to four times weekly at one fourth the total recommended dosage, greatly decreasing the cost. Marked regression of hepatomegaly and improvement in liver function tests, peripheral blood counts, and serum angiotensin-converting enzyme levels were documented. The two patients with pulmonary involvement manifested improvement in pulmonary function tests. Skeletal disease remained unchanged.

Adult↗
PubMed1990

Dilutional acidosis.

Rapid, extreme expansion of the extracellular fluid with solutions devoid of acid or alkali theoretically can produce a metabolic acidosis, due to buffer dilution. This phenomenon has previously been demonstrated only in experimental animal studies. We have reported what we believe to be the first documented case of hypobicarbonatemia and metabolic acidosis consequent to massive saline infusion, other causes having been excluded.

Acidosis↗
PubMed1985

Morbidity trends in the medical wards at Groote Schuur Hospital--1971 and 1982.

In 1971, 5 544 patients were admitted to the 261 Department of Medicine beds at Groote Schuur Hospital. During 1982 8 005 patients were admitted although the number of beds remained essentially unchanged. Whites constituted 54,7% of all admissions in 1971 and 39,3% in 1982. The overall mortality rate fell from 14,9% in 1971 to 9,6% in 1982, and the respective autopsy rates from 25,9% to 18,2%. Patterns of morbidity observed in the various population groups differed greatly and changed over the period studied. Comparisons between morbidity patterns in our patients and national mortality patterns showed close parallels within each population group and major differences between the different groups--those those in whites closely resemble patterns in industrialized countries, whereas for the patients of mixed race and black patients they more closely approximate those in developing countries. Attention is drawn to the inadequate growth of medical facilities in relation to population expansion, and to the urgent need for a far-sighted, ambitious, comprehensive, non-discriminatory preventive and curative health service.

Black or African American↗
PubMedDate not supplied

Splenectomy for hairy cell leukemia in pregnancy.

OBJECTIVE: Hairy cell leukemia is a rare hematologic malignant disease characterized by pancytopenia and splenomegaly. Its occurrence during pregnancy limits treatment with standard chemotherapeutic agents. A woman was found to have hairy cell leukemia and massive splenomegaly late in the second trimester. A review of the literature did not provide a carefully formulated treatment plan. It was believed that splenectomy (the historic therapy for hairy cell leukemia) would likely result in improved hematologic values and allow the pregnancy to progress. STUDY DESIGN: Case report with literature review. RESULTS: Preoperative laboratory studies revealed platelet count 65,000/mm3, hematocrit 28.6%, white blood cell count 4000/mm3, and hairy cell index 0.06 to 0.07. At 24 weeks' gestation, the patient underwent splenectomy with resolution of thrombocytopenia and normal progression of pregnancy without complication. Repeat cesarean section at 38 weeks produced a 2875 gm healthy male infant with Apgar scores of 9 and 9. CONCLUSION: If antepartum management of hairy cell leukemia is warranted, splenectomy is a safe and effective treatment option during the second trimester.

Adult↗