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J D Rain

Publications and source records attributed to J D Rain.

At least 37 records · Page 2Linked to original sources
PubMed1998

[Treatment of polycythemia. II.--Comparison of hydroxyurea with pipobroman in 294 patients less than 65 years of age].

AIMS: To compare by a prospective study in low risk polycythemia vera (PV) patients alone two drugs: hydroxyurea and pipobroman. Toxicity, efficiency, and leukemogenic potential were studied. PATIENTS: 294 patients with a documented PV, aged less than 65 years, have been included since 1980 in a prospective study comparing hydroxyurea and pipobroman. Blood cell counts were performed every two months and a clinical evaluation by a specialist was obtained every four or six months. RESULTS: Hematologic toxicity of both drugs was higher than expected, requiring strict surveillance. These drugs were tolerated in some (gastric pain and diarrhea on pipobroman, buccal aphtosis and chronic leg ulcers on hydroxyurea), leading to a change of arm in 10% of the cases. Hydroxyurea did not control the megakaryocitic hyperplasia in 40% of the cases, which probably explains a high rate of progression to myelofibrosis with myeloid metaplasia in this arm. Both drugs were leukemogenic with an actuarial risk of about 15% at the 15th year, not significantly lower than that observed in the 32P treated patients. A significant risk of cutaneous malignancy was observed in the hydroxyurea arm. The mean expectancy of life cannot yet be accurately evaluated, but seems significantly lower than that of the reference population. CONCLUSION: The treatment of PV by hydroxyurea or pipobroman has to account for these results less optimistic than those traditionally well-known to hematologists and internists.

Adult↗
PubMed1998

Epidemiological data in polycythaemia vera: a study of 842 cases.

An epidemiological study of 842 polycythaemic patients (entered between 1980 and 1997 in the French investigational prospective protocols) is presented. The global incidence is approximately 0.8-1.5/100,000/year in the reference area (Ile-de-France and surrounding areas). It increases linearly with age until 80, which suggests that several mutational somatic events are necessary. There was a slight male excess (sex-ratio 1.2, after correction for the percentage of male and female French people still living at risk). We did observe a slight excess of PV in the population of Jewish ancestry. A surprising excess of former blood donors (20.7% of the PV cases, compared to 8% estimated in the reference population) was observed. Only a few cases of familial myeloproliferative diseases and occurence of leukemia in the family of our patients have been observed; even if slight, this excess is statistically significant. In contrast, no excess of carcinomas was observed either in the family or in the patients' antecedents. We did not find any excess of radiation exposure in our cases. When analysing the previous occupation of our patients a possible excess of physicians and of patients previously working in occupations using solvents and glues was found, but this finding needs confirmation.

Adhesives↗
PubMed1998

[Radioimmunotherapy of lymphomas and leukemias].

Radioimmunotherapy offers an exciting new therapeutic modalities for patients with recurrent hematologic malignancies or resistant to conventional chemotherapy. Clinical trials involving hematologic malignancies have produced more impressive results than these involving solid tumors. In recurrent non Hodgkin's lymphoma Seattle trials have demonstrated objective responses in 90% of patients, complete responses in 85% of patients, a progression free survival of 62%, and an overall survival of 93% with a median follow-up of 2 years. In recurrent acute myelogenous leukemia, or myelodysplasia treated with radiolabeled antibodies, total body irradiation, and high dose chemotherapy 67% of patients remain disease free with a median follow-up of 33 months.

Animals↗
PubMed1998

[Favorable outcome of a severe carcinoid cardiopathy after complete resection of the primary ovarian tumor].

Carcinoid heart disease induced by a primary ovarian carcinoid tumor is very uncommon but offers the opportunity to study its specific outcome. Indeed, unlike to other carcinoid syndromes that occur in the setting of liver metastasis, a complete removal of the primary carcinoid ovarian tumor is often possible in such cases. We report a case of severe carcinoid heart disease secondary to a carcinoid ovarian tumor. Its interest is related to the exhaustive assessment of the cardiopathy and its complete regression after removal of the ovarian tumor. This outcome emphasizes the need for a as complete as possible reduction of humoral secretions in carcinoid heart disease by medical or surgical therapies before discussing valve replacement surgery.

Aged↗
PubMed1997

Treatment of polycythemia vera: the use of hydroxyurea and pipobroman in 292 patients under the age of 65 years.

Nonradiomimetic drugs, hydroxyurea (HU) and pipobroman (Pi), were administred to relatively young subjects with polycythemia vera (PV) in an attempt to decrease the leukemogenic risk observed in patients treated with 32P. Clinical safety, hematological efficacy, risk of carcinoma or leukemia, and frequency of progression to myelofibrosis have not yet been defined in long-term studies, and no comparative studies of HU and Pi have been conducted. Since 1980, 292 patients with PV diagnosed before the age of 65 years were randomized to receive treatment with HU (25 mg/kg/d, followed by low-dose maintenance) or Pi (1.2 mg/kg/d, followed by low-dose maintenance). Patients were followed until death or until May 1997. Drug tolerance was often poor; leg ulcers and buccal aphthous ulcers (with HU) and gastric pain and diarrhea (with Pi) sometimes required treatment change, mainly in the HU arm. Hematological stability, especially in terms of platelet count, was very often insufficient with HU (45% of cases), but the risk of thrombo-embolic event was similar in both arms. Actuarial survival was similar in the two arms and shorter than that of the reference population. The risk of leukemia was approximately 10% at the 13th year, with no significant difference between the two arms. The risk of carcinoma (when excluding the skin cancers) was similar in both groups. There was a high risk of progression to myelofibrosis in the patients treated by HU, which was significantly higher than with Pi.

Antineoplastic Agents↗
PubMed1997

Treatment of polycythemia vera: use of 32P alone or in combination with maintenance therapy using hydroxyurea in 461 patients greater than 65 years of age. The French Polycythemia Study Group.

Despite myelosuppression, polycythemic (PV) patients greater than 65 years of age have a high risk of vascular complications, and the leukemic risk exceeds 15% after 12 years. Is the addition of low-dose maintenance treatment with hydroxyurea (HU) after radiophosphorus (32P) myelosuppression able to decrease these complications? Since the end of 1979, 461 patients were randomized to receive (or not) low-dose HU (5 to 10 mg/kg/d), after the first 32P-induced remission, and were observed until death or June 1996. Maintenance treatment very significantly prolonged the duration of 32P-induced remissions and reduced the annual mean dose received to one-third. However, despite this maintenance, 25% of the patients had an excessive platelet count and the rate of serious vascular complications was not decreased, except in the most severe cases with short-term relapse of polycythemia. Furthermore, the leukemia rate was significantly increased beyond 8 years and a significant excess of carcinomas was also observed. The continuous use of HU did not decrease the risk of progression to myelofibrosis (incidence of 20% after 15 years). Life expectancy was shorter (a median of 9.3 years v 10.9 years with 32P alone), except in the most severe cases (initial 32P-induced remission lasting <2 years) in which maintenance treatment moderately prolonged the survival by reducing the vascular risk. In most cases of PV, in which the duration of the first 32P-induced remission exceeded 2 years, the introduction of HU maintenance did not reduce the vascular risk. Although it considerably decreased the mean dose of 32P received, HU maintenance therapy significantly increased the leukemia and cancer risks and reduced the mean life expectancy by 15%. However, in cases with more rapid recurrence, the introduction of maintenance treatment reduced the vascular risks and moderately prolonged survival. The use of HU as a maintenance therapy is therefore only justified in this situation.

Actuarial Analysis↗
PubMed1997

Red cell and platelet kinetics in chronic cytopenias following transplantation.

Thirty seven patients with unexplained anemia and/or thrombocytopenia after bone marrow, kidney, liver or heart transplantation were referred to the Department of Nuclear Medicine for erythrocyte or platelet kinetic studies in order to determine the mechanism of the cytopenia: accelerated destruction, or production defect. We observed only one definite case of thrombocytopenia due to accelerated autologous platelet destruction, while the life span was normal in the other 16 cases. Anemia was due to accelerated hemolysis in 7 cases, while the red blood cell life-span was normal in 12 other cases. Kinetic studies can therefore be useful, by demonstrating the mechanism of cytopenia observed after transplantation, and by facilitating the choice of appropriate treatment.

Adult↗
PubMed1997

Short- and long-term follow-up of thyroid dysfunction after allogeneic bone marrow transplantation without the use of preparative total body irradiation.

We studied the incidence and potential prognostic value of thyroid abnormalities after allogeneic bone marrow transplantation (BMT) without total body irradiation (TBI) conditioning. 77 consecutive patients who received a chemotherapy-alone-based conditioning regimen pretransplant were included. Free serum thyroxine (FT4), free serum triiodothyronine (FT3) and serum thyrotropin (TSH) levels were assayed before and 3 and 14 months after BMT. Patients were classified in three categories: normal thyroid profile if FT3 and FT4 were within the normal range and TSH was normal or low, peripheral thyroid insufficiency (PTI) if TSH was >4 mIU/l, or an 'euthyroid sick syndrome' (ETS) if FT3 and/or FT4 were low and TSH was normal or low. The incidence of thyroid dysfunction at 3 months was 57%, and 29% at 14 months. This was mostly due to the occurrence of ETS which was more frequent at 3 months (48%, 29/61) than at 14 months (19%, 9/48). Furthermore, at 3 months, survival was significantly lower in the ETS group (34.5%) than in the euthyroid group (96.2%), or in the PTI group (83.3%) (P < 0.0001). PTI was observed even in the absence of TBI in 11 patients (14%) and was equally distributed at 3 months (n = 6) and 14 months (n = 5). In conclusion, thyroid dysfunction is not a rare complication even without pretransplant TBI conditioning regimen. Hypothyroidism prevalence was 10%, and ETS, which was more frequently observed, displayed a dismal predictive value at 3 months.

Bone Marrow Transplantation↗
PubMed1997

The site of destruction of autologous 111In-labelled platelets and the efficiency of splenectomy in children and adults with idiopathic thrombocytopenic purpura: a study of 578 patients with 268 splenectomies.

The indication for splenectomy in chronic idiopathic thrombocytopenic purpura (ITP) remains a controversial subject. The mortality rate of persistent thrombocytopenia is very low, except in severe cases. Conversely, the risks of splenectomy are significant (in the present series, morbidity: 4.1% mortality: 1.4%), with a success rate of only 60-75%. It is therefore useful to define a parameter able to predict the efficacy or failure of splenectomy. An analysis of 578 cases of chronic ITP, where the site of platelet destruction has been determined, is presented. 268 of these cases had been splenectomized. When platelet destruction was splenic, 96% of subjects aged 5-30 years and 91% of cases over the age of 30 years obtained a remission. Conversely, when platelet destruction was hepatic or diffuse, failure or incomplete results were observed in 92% of cases. The site of platelet destruction therefore constitutes a parameter which can help the clinician to make the decision to perform splenectomy.

Adolescent↗
PubMed1996

Efficacy of photochemotherapy on severe pruritus in polycythemia vera.

Pruritus may cause severe chronic discomfort to PV patients. Most of the usual treatments are not at all or only weakly efficient. Photochemotherapy using psoralen and ultraviolet A light may largely improve the clinical symptom of intractable itching, as observed in ten of the 11 cases presented, but maintenance therapy is generally necessary. The treatment may then improve the patient's quality of life.

Aged↗
PubMed1996

Effect of glucose and lipids on intestinal absorption of sorbitol: role of gastric emptying.

The aim of our study was to test the hypothesis that the better absorption of sorbitol when ingested with glucose could be related to a delayed gastric emptying. We tested the effect of the ingestion of glucose and lipids on the gastric emptying and intestinal absorption of sorbitol in six healthy volunteers, using gastric scintigraphy and hydrogen breath test. After an overnight fast, subjects ingested in random order, on 48-h test periods separated by at least one week, the following solutions: (a) 20 g sorbitol alone; (b) 20 g sorbitol and 20 g glucose; (c) 20 g sorbitol and 9 g lipids. Isotopic acquisitions were taken for 3 h following the ingestion of sorbitol labelled with 111Indium. Hydrogen concentration was measured in end-expiratory samples during 5 h, and the areas under the breath hydrogen curve, reflecting the amounts of sorbitol unabsorbed in the small bowel, were compared between periods. Mean area under the curve was 397 +/- 159 when sorbitol was ingested alone, and this was significantly lower when ingested with glucose or lipids (313 +/- 181 and 337 +/- 135, respectively; P < 0.05). The three curves of sorbitol gastric emptying differed significantly from each other, the gastric emptying being the slowest for sorbitol plus lipids, and the fastest for sorbitol taken alone. We found a positive correlation between the half-emptying time and the hydrogen areas under the curve (r = 0.46, P = 0.05). In conclusion, our study demonstrates that adding glucose or lipids to a solution of sorbitol slows the gastric emptying of sorbitol, resulting in a better intestinal absorption of sorbitol.

Adult↗
PubMed1996

Bone marrow scintigraphy as a useful method for estimating the physiological status of bone marrow and spleen in polycythaemia vera.

Bone marrow scintigraphy is a simple and noninvasive examination useful to define the status of the bone marrow and spleen in polycythaemia vera (P.V.). Despite the absence of specificity of Indium 111 labelled transferrin (In-Tf) for myelopoietic tissue, there is a close correlation between bone marrow In-Tf uptake and bone marrow cellularity and between splenic In-Tf uptake and splenic metaplasia. The results of scintigraphy are compared to clinical data, radioactive iron kinetics, bone marrow and spleen histology and the course of the disease. The diagnostic and prognostic value of bone marrow scintigraphy is discussed, particularly at the stage of transformation of P.V. into postpolycythaemia myeloid metaplasia (Post-P.V.M.M.).

Bone Marrow↗
PubMed1996

Risk of leukaemia, carcinoma, and myelofibrosis in 32P- or chemotherapy-treated patients with polycythaemia vera: a prospective analysis of 682 cases. The "French Cooperative Group for the Study of Polycythaemias".

An analysis of the risk of progression towards leukemia, carcinoma and myelofibrosis was performed in 93 patients treated by 32P alone (PVSG protocols) since 1970-1979, 395 patients over the age of 65 years treated by 32P with or without maintenance therapy using hydroxyurea (French protocol) since 1980-1994, and 202 patients under the age of 65 treated by either hydroxyurea or pipobroman since 1980. The risk of leukemia, or myelodysplasia, or lymphoma in the 32P-treated patients was 10% at the 10th year, but increase after that time to reach a value of about 30% at the 20th year, in the surviving case. This risk was not dose-related. Despite a marked reduction of the cumulative 32P dose in the patients maintained by hydroxyurea, the actuarial risk was 19% at the 10th year. In the patients treated exclusively by non radio-mimetic agents (hydroxyurea or pipobroman) a risk of 10% at the 10th year was observed. The risk of carcinoma (excluding skin cancers) was about 15% at the 10th year in the 32P-treated cases, a value similar to that generally reported by the French statistics. There was no prevalence of digestive carcinomas. In contrast, the patients receiving 32P and hydroxyurea as maintenance had an excess risk: 29% at the 10th year. In the relatively young cases treated by non radio-mimetic agents, the risk was similar in both arms: 9% at the 10th year, similar to the expected incidence at this age. The risk of myelofibrosis with myeloid metaplasia was still relatively low at the 10th year, about 15% in all arms, but increased towards a value higher than 30% in the patients surviving at the 20th year. At the present time, but in only a few cases with long-term following, no myelo-fibrosis with splenic metaplasia has been observed in the pipobroman-treated cases. The present results, which need to be confirmed (the present analysis has been done in spring 95) suggest that:-the use of non radio-mimetic agents does not protect against leukemic transformation, which may be a consequence of the disease; rather than of the treatment,-maintenance therapy after initial use of 32P increases the risk of both leukemia and carcinoma,-and hydroxyurea does not delay the risk of developing myelo-fibrosis, in comparison with 32P alone.

Actuarial Analysis↗
PubMed1996

Advantages of SPECT in technetium-99m-sestamibi parathyroid scintigraphy.

UNLABELLED: We demonstrate several advantages of SPECT in parathyroid scintigraphy. METHODS: Forty-four parathyroid 99mTc-MIBI scintigrams were obtained before surgery in 43 patients suffering from hyperparathyroidism. For each patient, we obtained dynamic views and planar and SPECT images of the neck and thorax. For 15 patients, we also acquired a delayed static view of the neck 2 hr after tracer injection. Abnormal thyroid-area glands were detected with factor analysis of dynamic structure (FADS) of the initial dynamic acquisition. In the 15 patients with delayed views of the neck, we compared FADS and the double-phase study results to detect glands in the thyroid uptake area. Glands outside the thyroid area were demonstrated on planar views. The location of enlarged glands was more precisely defined on the tomographic slices. The anatomic and histologic findings and the evolution of hypercalcemia after surgery were taken as reference. RESULTS: Sixty-four abnormal glands were found during surgery, including 39 observed in patients who underwent reoperation for persistent or recurrent hyperparathyroidism. Twenty-two of these glands were in an abnormal location, including 10 in the mediastinum. SPECT allowed the detection of three glands not demonstrated on planar views or FADS. Fifty-eight glands were correctly localized scintigraphically, including 34 in patients who underwent reoperation. Therefore, SPECT raised the sensitivity from 86% to 90.5% and from 79.5% to 87% in the reoperated patients. Tracer uptake in the low mediastinal area was better analyzed on tomographic slices than on planar views. Only seven false-positive results were depicted by planar views or FADS; none were depicted on SPECT. CONCLUSION: A combination of FADS and SPECT permits detection of small glands, even in a posterior location, inside or outside the thyroid area. This scintigraphic method enables the surgeon to define more precisely details about the location of the enlarged gland and contributes to improved parathyroid surgery.

Choristoma↗
PubMed1994

The very-long-term course of polycythaemia: a complement to the previously published data of the Polycythaemia Vera Study Group.

The very-long-term follow-up of patients initially included in the PVSG protocols provides useful information. The excess risk of cancer after chlorambucil appears to persist for 5 years after stopping this treatment. The risk of leukaemia induced by marrow suppression (32P or chemotherapy) was marked before the 10th year, but low thereafter. Phlebotomy is unacceptable as permanent treatment because of the poor clinical tolerance and the frequency of vascular complications. This treatment is also associated with a risk of early progression towards myelofibrosis with myeloid splenomegaly. In the very long term, 15 years or more after the diagnosis, this complication is the major clinical risk, affecting almost 50% of our patients surviving at this time. The prevention of this type of complication could constitute one of the objectives of future protocols dealing with this disease.

Aged↗