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M Leporrier

Publications and source records attributed to M Leporrier.

At least 37 records · Page 2Linked to original sources
PubMed1997

[Autoimmune thrombopenic purpura. Clinical practices during diagnosis. A French survey and recommendations. French College of Hemotologists Evaluation Commission].

THE SURVEY: To better ascertain diagnostic and treatment strategies used by physicians for idiopathic thrombocytopenic purpura, a questionnaire was addressed to 298 French hematologists and pediatricians. One hundred and ten responses were analyzed. DIAGNOSTIC APPROACH: Thrombocytopenia was determined on capillary blood samples by 50% of the physicians and platelet-associated antibody by 44%. Bone marrow aspirates were obtained more frequently in adults (85%) than in children (47%) and immune disorders were more often investigated in 95% of adults and in 68% of children. THERAPEUTICS: Treatment threshold was lower in children (20.10(9)/l) than in adults (50.10(9)/l). Corticoidsteroids was the treatment of choice in adults (98%) and children received either IV immunoglobulins (61%) and/or corticosteroids (63%); higher doses were used in children (> or = 2 mg/kg versus 1 mg/kg) for shorter periods (> or = 2 weeks versus > 3 weeks). Treatment failure was evaluated earlier in children (< 10 days) than in adults (> 20 days). RECOMMENDATIONS: 1. Diagnosis. Based on repeatedly low platelet counts and verification that only platelets are involved. Clinical examination is normal excepting rare cases of severe hemorrhage. Search for antiplatelet antibodies is non-contributive 2. Treatment Short-term corticosteroids, both in children and adults. Intravenous gammaglobulins should be limited to cases with signs of severe hemorrhage. 3. Consult the complete guidelines for each individual clinical situation.

Humans↗
PubMed1997

[Autoimmune thrombopenic purpura. Therapeutic modalities].

TOP PRIORITY: Preventing hemorrhage is the number one priority in the treatment of idiopathic thrombocytopenic purpura. There are three main treatments: corticosteroids, intravenous immunoglobulins and splenectomy. CORTICOSTEROIDS: The main effects of corticosteroids are to reduce platelet phagocytosis, counteract mechanisms leading to inhibited thrombopoiesis, limit vascular fenestration and endothelial fragility, and immuno-depression. Corticosteroids are usually started at 1 to 2 mg/kg/day for 2 or 3 weeks before rapidly tapering off to final withdrawal after 3 or 4 weeks. IV IMMUNOGLOBULINS: Administered by intravenous injections, immunoglobulins saturate the Fc fragment of the IgGs on the surface of the macrophages, neutralize antiplatelet antibodies, and inhibits synthesis of antiplatelet autoantibodies. Treatment cost is high as is the risk of adverse effects. SPLENECTOMY: The most effective long-term treatment, splenectomy is particularly useful when platelet sequestration, as identified by radiolabeling occurs mainly in the spleen. OTHER THERAPIES: Anti-D immunoglobulins (limited availability), danazol or immuno-suppressors have a beneficial effect which disappears after withdrawal. INDICATIONS: Essential elements are the risk of hemorrhage (low or nil above 30000 platelets/mm3), its severity, patients status (childhood cases usually resolve spontaneously) and prognosis factors (idiopathic thrombocytopenic purpura is a benign disorder).

Adult↗
PubMed1996

Paroxysmal nocturnal haemoglobinuria: long-term follow-up and prognostic factors. French Society of Haematology.

BACKGROUND: Paroxysmal nocturnal haemoglobinuria (PNH) is a rare acquired disorder of haematopoietic stem cells. Although knowledge about the pathophysiology of the disease is increasing, no multivariate analysis of factors influencing survival has been undertaken, mainly because the disease is rare. We undertook such an investigation. METHODS: Data were collected on 220 patients with PNH diagnosed over a 46-year period (1950-1995) from participating French centres. Diagnosis of the disease required, at least, an unequivocally positive Ham's test. FINDINGS: The Kaplan-Meier survival estimate was 65% (SE 4) at 10 years and 48% (6) at 15 years after diagnosis. 8-year cumulative incidence rates of the main complications (pancytopenia, thrombosis, and myelodysplastic syndrome) were 15% (3), 28% (4), and 5% (2), respectively. Demographic data, presenting features, initial treatment, complications, and causes of death were similar to those previously reported. In multivariate analysis, seven factors were significantly associated with survival in patients with PNH. Poor survival was associated with the occurrence of thrombosis as a complication (relative risk 10.2 [95% CI 6-17], p < 0.0001), evolution to pancytopenia (5.5 [2.8-11], p < 0.0001), myelodysplastic syndrome or acute leukaemia (19.1 [7.3-50], p < 0.001), age over 55 years at diagnosis (4 [2.4-6.9], p < 0.0001), need for additional treatment (2.1 [1.3-3.6], p < 0.003), and thrombocytopenia at diagnosis (2.2 [1.3-3.8, p < 0.003). Better survival was shown for patients in whom aplastic anaemia antedated PNH (0.32 [0.14-0.72], p < 0.02). Factors associated in multivariate analysis with a high risk of thrombosis during the disease course were thrombosis at diagnosis (5.1 [2.5-10.6], p = 0.0002), age over 54 years (2.6 [1.5-4.6, p = 0.0014), and infection at diagnosis (2.6 [1.3-5.2], p = 0.0099). The risk factors for progression to pancytopenia were absence at diagnosis of anaemia (4.03 [1.3-12.2], p = 0.03) and neutropenia (2.45 [1.1-5.7], p = 0.03). The risk factors for development of myelodysplastic syndrome or acute leukaemia were abdominal pain crisis at presentation (10.5 [2.5-44.0], p = 0.004) and year of diagnosis after 1983 (8.45 [1.8-40.7], p = 0.004). INTERPRETATION: This large number of cases permitted a detailed analysis of prognostic factors for the first time, in this rare disease. Estimates of PNH prognostic factors may serve as baseline data in the assessment of current and future treatments for this disease.

Adolescent↗
PubMed1996

Phase II trial of fludarabine monophosphate as first-line treatment in patients with advanced follicular lymphoma: a multicenter study by the Groupe d'Etude des Lymphomes de l'Adulte.

PURPOSE: Fludarabine monophosphate (FAMP) is a major drug in the treatment of chronic lymphocytic leukemia and showed efficacy in selected groups of patients with low-grade lymphomas, most of them pretreated. The aim of this trial was to assess the efficacy and the toxicity of FAMP in untreated patients with follicular lymphoma. PATIENTS AND METHODS: Fifty-four untreated patients with advanced follicular lymphoma were treated with intravenous (i.v.) fludarabine at a dose of 25 mg/m2/d during 5 days every 4 weeks, to a maximum of nine cycles. RESULTS: The toxicity of the drug was mild, mainly granulocytic. Granulocytopenia > or = 3 (World Health Organization [WHO]) was observed during 48 of 328 cycles (14.6%) and in 22 of 53 (41%) patients assessable for toxicity. Fludarabine had to be stopped prematurely because of toxicity in nine patients: marrow toxicity in five, peripheral neuropathy in two, and interstitial pneumonitis and hepatitis in one patient each. Among 49 patients assessable for response, the overall response rate was 65% and the complete response (CR) rate 37%. The median progression-free survival interval for all patients was 13.6 months. CONCLUSION: These results confirm that fludarabine is active when used as first-line treatment in patients with follicular lymphoma and has a low toxicity rate. It may be used as single treatment in elderly patients. Associations of fludarabine with other drugs active against follicular lymphoma need to be determined.

Adult↗
PubMed1996

Late psychosocial sequelae in Hodgkin's disease survivors: a French population-based case-control study.

PURPOSE: To evaluate late psychosocial sequelae in long-term survivors of Hodgkin's disease (HD) in the population of Calvados, France. PATIENTS AND METHODS: Ninety-three patients issued from the Calvados General Tumor Registry, treated from 1978 to 1990, free of relapse and second malignancy since January 1991, were enrolled onto cross-sectional case-control study. One hundred eighty-six healthy controls, matched for sex, age, and residency, were selected at random from electoral rolls. Two self-administered questionnaires were mailed in the spring of 1995. RESULTS: Compared with controls, HD patients reported (1) more physical (P < .001), role (P < .001), and cognitive (P = .015) functioning impairments, as well as dyspnea (P < .001) and chronic fatigue (P = .025), while no statistical difference was found in global health status; (2) to be more often childless (P = .04), fewer divorces or separations (P = .013), fewer changes in relationships with friends (P = .012), similar proportions at work but less ambitious professional plans (P < .001), and greater difficulties in borrowing from banks (P < .001); (3) a slight increase in the number of visits to a general practitioner (P = .05) and greater consumption of medical resources (mainly thyroid extracts, P = .05). CONCLUSION: The study demonstrated that French long-term HD survivors have good global health status and good psychologic, familial, and professional status, although difficulties in borrowing from banks remain a major limitation in daily life. Although physical, role, and cognitive functioning impairments persist that might limit their activities, HD survivors seem to have learned to cope with problems related to their disease and its treatment.

Activities of Daily Living↗
PubMed1996

[Comparison of chemiluminescence and polynuclear neutrophil count after aplasia in hematologic malignancies].

Polymorphonuclear neutrophils (PNN) chemiluminescence results from the luminescence emission in oxidative reactions during phagocytosis. This activity is easily detectable and measurable using a luminometer. PNN luminescence allows precociously the evaluation of the end of the aplasia. We have analysed PNN luminescence emission stimulated by latex beads in 69 patients with hemopathies after 72 aplasias following autologous bone marrow transplantation (9 pts), allogeneic bone marrow transplantation (26 pts) and acute myeloblastic leukemia (AML) induction chemotherapies (37 pts). Luminescence emission was measured in whole blood using a luminometer before, during and after aplasia and was compared to PNN manual count. Chemiluminescence measurement is a simple and reproductable method. Its allows more earlier than the PNN blood count the detection of PNN recovery: chemiluminescence nadir is reached on average on the fourth day of aplasia and correspond to the value procured by sample tubes without PNN. The onset of the chemiluminescence increasing is definite by the doubling of the nadir value. It is reached on average on the fourteenth day of aplasia. It precedes 3.5 days and 10.5 days a total PNN count > or = 0.1 x 10 9/1 and > or = 0.5 x 10 9/1 respectively, in the 72 aplasias. In autologous and allogeneic bone marrow transplantation, chemiluminescence increasing precedes at least PNN > or = 0.1 x 10 9/1 of 4.6 days whereas in AML induction chemotherapies, the advantage is only 1.5 day (p = 0.0078). The chemiluminescence could be considered as an additional tool in daily management of sustained aplasia.

Adolescent↗
PubMed1995

[Nephropathies caused by interferon alpha: apropos of 2 cases].

Two cases reports of interferon alpha-associated nephropathy are reported. The first observation is a membranoproliferative glomerulonephritis and the second a renal microangiopathy. The different cases in the literature are reviewed and the pathophysiology is discussed.

Adult↗
PubMed1995

Human recombinant granulocyte-macrophage colony stimulating factor (hrGM-CSF) improves double hemibody irradiation (DHBI) tolerance in patients with stage III multiple myeloma: a pilot study.

Double hemibody irradiation (DHBI) is an alternative treatment of stage III multiple myeloma (MM) in patients aged over 55 years. Toxic side-effects such as myelosuppression are a severe limiting factor to its use. We performed DHBI associated with human recombinant granulocyte-macrophage colony stimulating factor (hrGM-CSF) as support therapy in 10 patients with stage III MM to improve the tolerance to this treatment. Ten patients received subcutaneously 5 micrograms/kg/d of hrGM-CSF during 2 weeks after each course of hemibody irradiation. All these patients had stage III MM: eight previously received chemotherapy, six of them were regarded as patients with refractory MM and two with relapse. Two patients received DHBI as first-line treatment. hrGM-CSF increased safety and tolerance of DHBI. GM-CSF support reduced the mean time between upper body irradiation (UBI) and lower body irradiation (LBI): 41 v 108 d in a cohort of 32 patients previously treated without growth factor support. Overall there was no lethal infection with hrGM-CSF or granulocytopenia (5.0 x 10(9)/l v 0.4 x 10(9)/l at day 15 in patients without growth factor). hrGM-CSF also reduced stomatitis grading and thrombocytopenia (90 x 10(9)/l v 45 x 10(9)/l at day 15). Furthermore, hrGM-CSF increased blood colony forming unit-granulocyte macrophage (CFU-GM) and was well tolerated in all but one patient. hrGM-CSF reduces toxic side-effects of DHBI, thus providing an effective treatment in patients with advanced and resistant MM.

Aged↗
PubMed1995

[Place of celio-video-surgery in splenectomy for hematologic diseases].

In order to evaluate the place of the laparoscopic approach in splenectomy for haematological disease, the authors prospectively studied a series of 25 consecutive patients requiring splenectomy. There were 11 cases of thrombocytopenic purpura, 9 lymphomas, 2 cases of herediary spherocytosis, 1 Felty syndrome, 1 idiopathic myelofibrosis and 1 Hodgkin disease. Twelve patients (48%) underwent an immediate conventional procedure for huge splenomegaly (10), obesity (1), unavailability of video-equipment. Thirteen patients (52%) underwent a laparoscopic approach. Five of these operations were converted into a conventional approach for various reasons. In the other 8 patients, the spleen was completely released laparoscopically. In two of these 8 patients, the spleen was removed via a sub-pubic Pfannenstiel incision due toits volume. The last 6 spleens (24%) were removed in a plastic bag, corresponding to 5 cases of one thrombocytopenic purpura and one Hodgkin disease. None of these patients were obese. These results suggest that the laparoscopic approach is indicated in case of moderate splenemegaly in non-obese patients.

Adult↗
PubMed1994

[Primary adrenal lymphoma. Report of a case].

The adrenal gland is affected in about 25% of patients with diffuse lymphoma. Conversely, primary lymphoma is a rare cause of non-secreting adrenal tumour. We observed a recent case with exclusive right adrenal localization. An 84-year-old woman was hospitalized for asthenia, anorexia and a 3-kg weight loss. The past history included coronary heart disease. Physical examination was normal and the hemogram revealed pancytopenia. No lymphomatous infiltration was seen on the myelogram or the bone marrow biopsy. Echography of the abdomen showed a well-limited 10 cm mass above the right adrenal gland, distinct from the liver; the left gland had a normal aspect. Hormone assays were within normal limits. The right adrenal gland was removed surgically and histology revealed diffuse proliferation of medium to large sized lymphoid cells invading the entire gland. Immunohistochemistry suggested lymphoid B origin. No curative treatment was begun, but 2 months after surgery local invasion required polychemotherapy. The patient died of septic shock 5 days later. Serum cortisol measured during the septic shock eliminated adrenal gland failure. Malignant primitive non-Hodgkinien lymphomas are extremely rare and usually discovered incidentally. The tumour is always large and sometimes accompanied by adrenal insufficiency when the lesion is bilateral. In spite of suggestive imagery the diagnosis is based on the pathology examination. The lesion is usually a high-grade malignant phenotype B lymphoma. Treatment relies on chemotherapy but is disappointing.

Adrenal Gland Neoplasms↗
PubMed1994

[Clostridium perfringens septicemia in drug-induced aplasia].

Septicemia due to Clostridium perfringens during the course of acute leukemia is rare and often lethal particularly in childhood. Antibiotherapy is necessary but polymorphonuclear activity recovery is helpful. This can be done through transfusion or administration of colony stimulating factors. Here is a new case of such a septicemia in a 12 year-old female treated for acute lymphoblastic leukemia. Of particular interest is the favourable outcome despite a high risk situation.

Anemia, Aplastic↗
PubMed1994

Double hemibody irradiation with GM-CSF as salvage therapy for refractory chronic lymphocytic leukemia.

We describe a new and original therapy with total body irradiation in two separate 4 gy single courses (double hemibody irradiation) combined with GM-CSF support, 5ug/day on days 1-15 after each hemibody irradiation, for refractory patients with B-chronic lymphocytic leukemia (CLL). A complete response was observed in a patient with a B-CLL resistant to CAP and FAMP therapy. Overall tolerance was good. The major points of interest in this technique are the combination of the antitumor effect of irradiation, limited bone marrow toxicity and a potential specific anti-leukemia effect of GM-CSF.

Aged↗
PubMed1994

[Extramedullary hematopoiesis of thoracic and vertebral intraductal localization. Apropos of a case. Review of the literature].

The authors report a case of extramedullary haematopoiesis, presenting in the form of several tumor masses in the posterior mediastinum and in the vertebral canal. The patient presented with oedema in his lower limbs due to compression of the inferior vena cava by the mediastinal masses. The asymptomatic mass in the vertebral canal was detected by magnetic resonance imaging. The patient was first operated on to avoid spinal cord compression by the tumor and subsequently to relieve compression of the inferior vena cava. The masses removed from the vertebral canal and the mediastinum were found on histologic examination to be extramedullary haematopoiesis. The cause of the disease was unknown in this case. Such disease is often disclosed in the course of hemolytic anaemias or other medullar deficiency diseases as a compensatory phenomenon when the bone marrow fails. An other explanation is that it is a manifestation of a myeloproliferative disorder in which heterotopic pluripotential cells are transformed into haematopoietic cells. The authors review 88 cases reported in the world literature.

Aged↗