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Biomedical subjects

M T Caulier

Publications and source records attributed to M T Caulier.

At least 19 recordsLinked to original sources
PubMed1992

Accessory spleen in recurrent chronic immune thrombocytopenic purpura.

From 1969 to 1985 we discovered accessory spleens in 8 patients with chronic immune thrombocytopenic purpura (ITP) who relapsed or failed after splenectomy. Imaging of accessory spleen used a liver spleen scintigraphy with heat-treated RBC labeled with Tc-99m. Platelet kinetic studies with 51Cr or 111In, including sequestration index, were performed. Five patients had accessory splenectomy. Disappearance of bleeding symptoms was achieved in the 5 splenectomized patients but with only partial response of platelet counts. These results are discussed in the context of diagnosis and therapeutic management of accessory spleens in patients with chronic immune thrombocytopenic purpura who relapsed or failed after splenectomy.

Adolescent↗
PubMed1992

[Role of accessory spleen in recurrent chronic hematologic diseases].

Among 533 patients who were splenectomized between 1967 and 1981 for a chronic haemopathy, 8 were reoperated because of the reappearance of an accessory spleen, which was responsible for the relapse of the disease. Five patients were followed for idiopathic thrombopenic purpura (ITP), 2 for hereditary spherocytosis and 1 for a Hodgkin disease (this patient had been operated for an abdominal exploration and splenectomy). In all patients, a hepato-splenic scintigram with Tc 99 m permitted the discovery of the accessory spleens and the exploration was completed by the study of the half lifetime and sequestration of platelets or red blood cells. The disappearance of the haemorrhagic syndrome after removal of the accessory spleen was frank and didn't need any complementary treatment for 3 cases of ITP and 2 cases of spherocytosis and was incomplete and had to be completed by a secondary treatment for 2 cases of ITP and for the Hodgkin disease. The analysis and the interpretation of the results of this study can be helpful to establish the diagnosis and decide the treatment of accessory spleens which are discovered by a relapse of a chronic haemopathy, primarily treated by splenectomy.

Adolescent↗
PubMed1991

Usefulness of electron microscopy in the diagnosis of congenital dyserythropoietic anemia type I: report of a case.

This report describes a case of congenital dyserythropoietic anemia (CDA) type I diagnosed in a 22-year-old woman with a two year history of macrocytic anemia. Light microscopy study of bone marrow disclosed only minor and nonspecific findings, without erythroid hyperplasia. Electron microscopy of bone marrow erythroblasts surprisingly showed most of the classical features of CDA type I. Electron microscopy may be an important tool for the diagnosis of macrocytic anemia of obscure origin, especially in young patients.

Adult↗
PubMed1990

Clinical course of essential thrombocythemia in 147 cases.

The authors retrospectively analyzed the initial characteristics, treatment, and clinical course in 147 patients with essential thrombocythemia (ET). Median age was 60 years and the M:F ratio was 0.69. At diagnosis, 53 patients were asymptomatic; 50 patients had functional symptoms (mainly vasomotor disturbances); 27 patients had large vessel thrombosis; 27 patients had a bleeding diathesis; and seven patients had both bleeding and thrombosis. The platelet count ranged from 0.7 to 2.92 X 10(12)/l. Forty-five of the 61 tested patients (61%) had prolonged bleeding time and/or platelet hypoaggregation. Three patients had in vitro spontaneous aggregation. No significant correlations were found between hemostatic findings and in vivo bleeding or thrombosis. The incidence of bleeding, however, was higher in patients with more than 2 x 10(12)/l platelets. Of 87 karyotypes performed with banding techniques, only four were abnormal. One hundred twenty-nine patients received one or more cytoreductive agents at diagnosis or during follow-up. Sixty patients received an antiaggregating agent. First-line therapy was radiophosphorus (32P) in 22 patients; busulfan in 35 patients; and hydroxyurea in 72 patients. Hydroxyurea required continuous maintenance therapy and had to be changed to another treatment in 12 of the initial responders because of inadequate control of thrombocythemia. During follow-up, 14 treated patients experienced one or several major thrombotic events. Two untreated patients also had major thrombosis. Only one major bleeding event was seen during follow-up. Median actuarial survival was 73.5% at 7 years and only one patient progressed to acute non-lymphocytic leukemia (ANLL). These results suggest that large vessel thrombosis is the main complication of ET. It appears largely unpredictable in a given patient at diagnosis but can be largely prevented by the control of thrombocythemia. Because of the low incidence of side effects of treatment in this experience, the authors believe that cytoreductive therapy is indicated in most patients with ET, as long as a group of patients with very low risk of thrombosis is not defined in prospective studies.

Adolescent↗
PubMed1990

Slow infusions of vinblastine in the treatment of adult idiopathic thrombocytopenic purpura: a report on 43 cases.

Forty-three adult patients with idiopathic thrombocytopenic purpura (ITP) were treated by slow intravenous infusions of vinblastine. Nineteen had ITP of recent onset (i.e. of less than 6 months duration) and had contraindication to steroids (3 patients), refractoriness to steroids (6 patients) or to steroids and high dose intravenous immunoglobulins (IVIg, 10 patients). Of the 19 patients, 10 achieved complete response (CR), 2 achieved partial response (PR), 2 had minor response (MR) and the remaining 5 patients had no response (NR). Six of the complete responders remained in CR after 12 to 48 months, whereas all other responders relapsed within 3 months, in spite of maintenance therapy. Twenty-four patients had chronic ITP (i.e. of 6 months duration or more) and had showed no or only transient response to steroids and/or splenectomy, and in many of them, to other therapeutic approaches. Four achieved CR, 4 PR, 6 MR and 10 NR. All but 3 responses were shorter than 3 months, in spite of maintenance therapy. Most responses to slow infusions of vinblastine began after the first infusion. Main side effects included leukopenia in 9 patients (but with absolute neutropenia in only one) and peripheral neuropathy in 2 patients. Interval from diagnosis was the only prognostic factor of response to treatment. We conclude that slow infusions of vinblastine may be a useful approach in ITP of recent onset, when contraindication or refractoriness to steroids and/or IVIg exists. In our experience, this treatment has limited benefit in chronic ITP. In addition, it remains to be demonstrated that slow infusions of vinca alkaloids have any superiority over intravenous bolus injections of the same drugs.

Adult↗
PubMed1990

Re-evaluation of the role of azathioprine in the treatment of adult chronic idiopathic thrombocytopenic purpura: a report on 53 cases.

We treated 53 adults (mean age 54 years, range 17-89; 37 females and 16 males) with chronic idiopathic thrombocytopenic purpura (ITP) by azathioprine. All patients had received at least one form of therapy (including splenectomy in 40 patients) and had less than 50 x 10(9)/1 platelets. The duration of ITP before azathioprine was started ranged from 6 to 350 months (median 19). All patients initially received 150 mg/d of azathioprine. This was associated with a short initial course of prednisone (0.3-0.5 mg/kg d) in 10 of them, who were refractory to prednisone alone. 34 (64%) patients responded, including 24 (45%) complete remissions (CR), three (6%) partial remissions (PR) and seven (13%) minor responses (MR). Median time to achieve response was 4 months. 17 of the CR persisted after 7-182 months, 10 of them after discontinuation of azathioprine. Seven patients relapsed after 4-26 months, five of them after azathioprine was stopped or its dose was reduced. PR were short and the median duration of MR was 8 months. Overall, 21 patients (40%) had responses lasting 1 year or more and 17 (32%) lasting 2 years or more. Median duration of treatment was 18 months (range 3-84). Five patients died of bleeding while on treatment. No prognostic factors for response to azathioprine were found. Mild leucopenia was seen in seven patients and a moderate (x3) increase in transaminases in two patients. No opportunistic infections were seen and no malignancy has occurred since the onset of azathioprine. We conclude that azathioprine gives a relatively high incidence of durable responses and very limited side effects in chronic ITP, when splenectomy has failed or is contraindicated. This efficacy, in our experience, is superior to that obtained with other therapeutic approaches. As responses may be delayed, a course of azathioprine of 4 months is required before one can infer a failure to respond. In responding patients, however, the optimum duration of treatment remains to be established.

Adolescent↗
PubMed1990

The role of danazol in the treatment of refractory idiopathic thrombocytopenic purpura. A report of 22 cases.

We treated 22 patients with refractory idiopathic autoimmune thrombocytopenic purpura (ITP) with Danazol (generic name). Median age was 57 (1 child aged 11, and 21 adults aged 18 to 77). Patients were refractory to or had contraindication to therapy by prednisone and splenectomy. Several patients had already received other treatments. All patients received Danazol at a daily dose of 600 mg/day for at least 2 months (except the child who received 400 mg/day), and other treatments for ITP were discontinued when Danazol was started. Two patients achieved complete remissions (CR), one partial remission (PR) and four had minor responses (MR). The remaining 15 patients had no response (NR). One of the complete responses lasted 34 months, but was dependent on daily maintenance doses of Danazol of 400 mg or more. One partial responder remained so after seven months, four months following the discontinuation of Danazol. All other responders relapsed within three months, while receiving Danazol 600 mg/day. The drug was generally well tolerated, with few side effects. Danazol, when used as a single drug in refractory ITP, therefore had limited benefit in our experience.

Adolescent↗
PubMed1989

Reevaluation of the prognostic factors for splenectomy in chronic idiopathic thrombocytopenic purpura (ITP): a report on 181 cases.

From 1973 to 1986 we splenectomized 181 patients with chronic ITP after platelet kinetic studies with 51Cr or 111In. Mean age at diagnosis was 34 (range 4-79 yr). Follow-up of at least 1 yr after splenectomy was available in every patient. 141 patients (78%) achieved remission (platelets greater than 100 x 10(9)/l by 3 months after splenectomy), of whom 9 subsequently relapsed. Among the 40 non-responders at 3 months, 3 achieved a later remission spontaneously. Factors associated with response to splenectomy included a high post-operative platelet count (p = 0.0001), younger age at the time of surgery (p = 0.0077) and predominantly splenic sequestration of platelets (p = 0.0002), the two latter factors being partially correlated. In a multivariate analysis, however, only post-operative platelet count and age retained an independent prognostic significance, whereas the sequestration site of platelets had only borderline value. These results are discussed in the context of indications of platelet kinetic studies in chronic ITP, before splenectomy is considered.

Adolescent↗
PubMed1987

[Ineffective erythropoiesis of immunologic origin with free bilirubin jaundice in an elderly subject].

We report on a 74-year-old male patient who presented with jaundice, highly increased unconjugated bilirubin and slight nonregenerative anemia. Ferrokinetic studies demonstrated ineffective erythropoiesis and red cell survival, studied by 51 Cr, showed moderately increased hemolysis. Other findings showed no evidence of an underlying myelodysplastic syndrome but strongly favored the autoimmune origin of the disorder, with immunologic destruction of erythroblasts (and to a far lesser extent erythrocytes) by autoantibodies.

Aged↗
PubMed1979

[Detection of platelet antibodies in idiopathic thrombocytopenic purpura (author's transl)].

The amount of IgG present on the surface of platelets (platelet-bound IgG, or PBIgG) was measured using a quantitative antiglobulin consumption assay developed by Dixon et al. (1975). Normal platelets or platelets from patients with thrombocytopenia due to a decreased production had less than 10 X 10(-15) g of IgG/platelet. Studied were 364 blood samples drawn from 196 patients; 173 patients had idiopathic thrombocytopenic purpura; 65 (93 %) of 70 in the acute phase of their disease had elevated PBIgG (values up to 100 times greater than the normal); 98 (76.5 %) of 128 blood samples from patients during a chronic phase had abnormally high PBIgG but the levels of IgG were generally lower than those observed in the acute phase; 75 (75 %) of 100 samples from patients in remission did not have pathologically increased PBIgG. Usually when the platelet count improved (after prednisone therapy or after splenectomy), PBIgG decreased. The PBIgG determination was not useful for predicting the response to prednisone therapy or the further evolution of the disease, except when PGIgG remained elevated after high-dose prednisone therapy; this generally implied a poor prognosis. PBIgG have also been found elevated in other diseases such as lupus erythematosus and in one case of chronic lymphocytic leukemia. This assay may be of a great clinical interest, especially for the diagnosis of ITP.

Antibodies↗
PubMed1977

[Laparotomy in the therapy of Hodgkin's disease].

We have performed laparotomy and splenectomy for seventy patients with Hodgkin's disease. Among 43 stages I or II before exploration, primary laparotomy revealed ignored abdominal localizations in 16 cases (37,2%). Involvement of the spleen occurred in 13 of these 16 patients, lonely (5 cases), associated with positive nodes (4 cases) or associated with positive nodes and liver involvement (4 cases). Among 4 stages III before exploration, no regression was found. Among 9 patients with presumed stages I or II above the diaphragm and previously treated by radiotherapy, delayed laparotomy revealed abdominal relapse in 6 cases. At last, among 14 patients with obvious stages II or IV and previously treated by combination chemotherapy, laparotomy, caused by persistent evolutive signs, showed residual localizations under the diaphragm in 8 cases. According to these results, we preconise immediate laparotomy in almost all presumed stages I or II above the diaphragm. About third of these subjects have their staging advanced to stage III or IV and, according to us must be treated by combination chemotherapy while the remaining cases justify of radiotherapy. In obvious stages III and IV delayed laparotomy may be useful in some circumstances, after chemotherapy.

Cobalt Radioisotopes↗