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Biomedical subjects

P Bierling

Publications and source records attributed to P Bierling.

At least 91 records · Page 5Linked to original sources
PubMed1988

Early immunization against platelet glycoprotein IIIa in a newborn Glanzmann type I patient.

Alloimmunization against platelet glycoprotein IIb and/or IIIa is a complication rarely observed during the evolution of type I Glanzmann's thrombasthenic patients. The occurrence of such alloantibodies is usually due to repeated blood transfusion and greatly complicates the treatment of these patients since they prevent effective platelet transfusion and might, theoretically, cause posttransfusion purpura. We describe the case of a newborn thrombasthenic patient who developed an IgG platelet allo-antibody 1 month after birth. The diagnosis of Glanzmann's thrombasthenia was complicated by the rare platelet phenotype (PLA1-negative PLA2-positive) of the healthy mother, which was probably heterozygous for the abnormal thrombasthenic gene. Immunofluorescence and immunoblotting techniques demonstrated that the patient antibody was principally directed against the platelet glycoprotein IIIa. Surprisingly, this patient had only received four blood transfusions (fresh frozen plasma on days 1 and 2, and standard red blood cell concentrates on days 5 and 6) before the discovery of the antibody, suggesting prior in utero sensitization. This study emphasizes the need for early diagnosis of the disease. Thrombasthenic patients should be transfused with deleukocyted platelet-free blood products.

Blood Platelet Disorders↗
PubMed1988

Anti-RH immunoglobulin therapy for human immunodeficiency virus-related immune thrombocytopenic purpura.

The potential hazards of steroids in human immunodeficiency virus (HIV)-infected patients led us to evaluate the effectiveness and safety of anti-D and anti-c Ig in 17 adults with severe HIV-related immune thrombocytopenic purpura (platelet count less than 20 x 10(9)/L). The 14 Rh+ patients received 12 to 25 micrograms/kg of anti-D IgG intravenously on two consecutive days. A significant platelet rise above 50 x 10(9)/L was obtained in nine patients. Repeated boosters were performed in six cases and were effective in all cases. The 3 Rh- patients had a good response after they were given 20 mL x 2 of plasma containing potent anti-c antibodies. Therapy was well tolerated, and only one patient had significant hemolysis. These data suggest that anti-Rh IgG can be effective and safe in HIV-related thrombocytopenic purpura and that a specific interaction between the RBC antigens and the anti-Rh antibodies is required.

Autoimmune Diseases↗
PubMed1987

[Prevalence of anti-HIV antibodies in immunoglobulins recipients].

Seven hundred and forty subjects who, between 1980 and 1985, had received intravenous or intramuscular injections of immunoglobulins (Ig) prepared by Cohn fractionation of plasma pools from more than 2000 donors were investigated for anti-HIV antibodies. Anti-HIV antibodies were detected in only one subject, a female drug addict and therefore belonging to a group at high risk of AIDS. In contrast with the huge amounts of Ig received by the remaining 739 subjects (976 litres intravenously, 128 litres intramuscularly), this young woman had only received 10 ml of anti-HBs Ig intramuscularly. The wide scattering in both space and time of these recipients of Ig and the prevalence of anti-HIV antibodies among blood donors in France (0.7 per 1000 in 1985) suggest that all the batches studied (over 100) were contaminated by HIV. The absence in these subjects of seropositive reaction ascribable to the injections of Ig confirms that Cohn fractionation inactivates the virus, as demonstrated in vitro. It is concluded that recipients of Ig prepared from plasma pools not tested for the absence of anti-HIV antibodies should not be regarded as a group at risk of AIDS.

Acquired Immunodeficiency Syndrome↗
PubMed1987

Persistent remission of adult chronic autoimmune thrombocytopenic purpura after treatment with high-dose intravenous immunoglobulin.

Transient efficacy of high-dose intravenous immunoglobulin infusions (HDI) is widely demonstrated in autoimmune thrombocytopenic purpura (AITP). A trial involving repeated injections of HDI was conducted in two adult patients with chronic AITP in order to evaluate their long-term efficacy in refractory forms and their ability to obviate splenectomy. Both patients received 2 g/kg body weight of polyvalent intact intravenous immunoglobulin followed by seven and eight boosters (0.8 to 1.2 g/kg body weight) when the platelet count decreased. It was found that the injections could be spaced progressively. The platelet count remained normal 200 and 800 days after the end of the treatment. The demonstration of the possible long-term efficacy of repeated injections of intravenous immunoglobulin encourages us to recommend this safe therapy for patients with chronic AITP.

Adult↗
PubMed1987

[Transfusion strategy for patients in therapeutic aplasia. Experience of the Henri Mondor Hospital apropos of 2 successive protocols].

Retrospective analysis of two transfusion protocols applied in our institution to the bone marrow transplanted patients was conducted. Granulocyte transfusions should be only proposed as a therapeutic treatment to patients with severe well documented bacterial infection resistant to an adapted antibiotherapy. Leukocyte-depleted blood products reduce the incidence of HLA-immunization but do not influence the frequency of CMV infections. Random single donor platelet concentrates (obtained by cytapheresis) could decrease the incidence of polyspecific HLA-antibodies in comparison with the use of random standard platelet concentrates. The best transfusion protocol should associate leukocyte-depleted blood products with transfusion of prophylactic single donor platelet concentrates. In our institution, this protocol is less expensive than the protocol with prophylactic white blood cell transfusions and has the same cost than other protocols using standard blood products.

Agranulocytosis↗
PubMed1987

Response to therapy in 37 patients with HIV-related thrombocytopenic purpura.

Thirty-seven patients belonging to the major AIDS-risk groups and with serum antibodies to the human immunodeficiency virus (HIV), were evaluated and treated between 1983 and 1986 because of profound chronic thrombocytopenia with clinical bleeding. Fifteen out of 26 patients achieved a significant response while on prednisone therapy. Six of them sustained their platelet count above 50 x 10(9)/1 following cessation of steroids. High dose intravenous immunoglobulin therapy gave a satisfactory initial response rate in 12 of 17 patients. Only two of 18 patients responded to danazol. Splenectomy was successful in 10 of 14 patients.

AIDS-Related Complex↗
PubMed1986

Pulmonary complications occurring after allogeneic bone marrow transplantation. A study of 130 consecutive transplanted patients.

This report deals with 81 pulmonary episodes occurring in 130 consecutive patients who underwent allogeneic bone marrow transplantation for hematologic malignancy in the same unit over a 5-year period. These episodes observed in 69/130 patients (53%) were mostly of infectious origin, and were investigated by bronchoalveolar lavage (BAL). The main causes of pneumonia were: cytomegalovirus (CMV) (n = 25), bacterial pneumonia (n = 17), invasive aspergillosis (n = 11) and pulmonary hemorrhage (n = 9). The overall mortality due to or associated with pneumonia was 26/130 (20%). Graft-versus-host disease clearly increased the incidence of infectious pneumonia and the mortality due to or associated with pneumonia. Granulocyte transfusions did not influence the incidence of CMV pneumonitis. The main causes and risk factors for pneumonia are discussed. The role of BAL as a noninvasive procedure is stressed.

Adolescent↗
PubMed1985

Acute promyelocytic leukemia in 57 previously untreated patients.

Fifty-seven patients in initial phase of acute promyelocytic leukemia (APL) were treated in the same department with heparin infusion, platelet transfusions, and two related induction regimens both including cytosine arabinoside and daunorubicin. Clinical and biological findings at presentation were studied. The complete remission (CR) rate was 53%. Twenty-seven patients (47%) died during the initial course of the disease, either before day 5 (early death [ED], n = 7) or after day 5 (death in aplasia [DA], n = 20). Most ED was due to intracerebral hemorrhage (6/7), especially when large hemorrhages had been seen on fundus oculi examination. Most DA was due to multivisceral failure (9/20). No correlation was found between initial disseminated intravascular coagulation (DIC) and death. However, the worsening of coagulation parameters during induction therapy, with or without initial DIC, significantly increased the occurrence of renal and respiratory failure which were particularly frequent during the first month. The median duration of survival was short (3.5 months) and the median duration of CR (11 months) was similar to that of other acute myeloid leukemias treated with the same regimens. The possible causes of the high mortality observed during the initial courses of APL and the possible benefit of a more graduate induction chemotherapy are discussed.

Acute Kidney Injury↗
PubMed1985

Acquired autoimmune thrombocytopenia after allogeneic bone marrow transplantation.

A 29-year-old man in remission from acute myeloblastic leukaemia was treated by chemoradiotherapy and transplantation of bone marrow (BMT) collected from his HLA identical brother. Engraftment was documented on D12. Transient acute GVHD (grade II) appeared from D34. No infection complicated the BMT. Nevertheless severe thrombocytopenia persisted and was unresponsive to marrow donor platelet transfusion. The platelet immunofluorescence test demonstrated the autoimmune basis of the thrombocytopenia. This study suggests that the transient immune imbalance observed in the early post graft period could facilitate the appearance of autoimmune cytopenias.

Adult↗
PubMed1984

High dose intravenous gammaglobulin and platelet transfusions in leukaemic HLA-immunized patients.

3 aplastic patients with acute leukaemia, strongly HLA-immunized and refractory to platelet transfusions, received polyvalent gammaglobulin i.v. infusions (0.4 g/kg/d for 5 or 6 d) in association with daily random platelet transfusions. Platelet recovery was obtained in 2 patients. The 3rd patient did not show any significant rise in platelet count. The ability of gammaglobulin to prolong the life-span of incompatible transfused platelets could facilitate the management of HLA-immunized patients.

Adult↗
PubMed1984

[Autoimmune thrombopenic purpura and pregnancy. Absence of thrombopenia in the newborn infant despite the presence of circulating IgG auto-antibody in the mother].

Many pregnant women with AITP bear children with transient thrombocytopenia which is a potentially life threatening complication. Previous reports have demonstrated that monitoring of IgG circulating platelet antibodies may help with the management of these women. We report the case of a pregnant woman with AITP whose sera contained an IgG auto antiplatelet demonstrated by the fluorescein labelled antiglobulin techniques. Steroids had a beneficial effect with a return to a normal of the mother's platelet count but with no disappearance of the circulating antibody. Surprisingly the neonatal platelet count was normal and studies in the baby showed that the antibody had not crossed the placenta in detectable amounts. This case demonstrates our current inability to predict fetal thrombocytopenia and plan the mode of delivery.

Autoimmune Diseases↗
PubMed1983

Early response to chemotherapy as a prognostic factor in Hodgkin's disease.

In 164 patients with Hodgkin's disease staged between 1973 and 1979 the response to the 3 initial cycles of multiagent chemotherapy was evaluated as a prognosticator of survival. Treatment of localized disease (Stages I, II, III1) consisted of 3 cycles of chemotherapy followed by subtotal nodal irradiation, including the splenic area in non splenectomized patients. Treatment of extended disease (Stage III2 and IV) consisted of 6 cycles followed by low-dosage radiotherapy of initial bulky disease. Five-year actuarial survival was 88% in Stage I, 80% in II, 100% in III1, 45% in III2 and IV. Chemotherapy-induced complete remission after 3 cycles (CH leads to CR) was associated with a favorable prognosis. Five-year survival of Stage III2 and IV patients was better in those who reached CH leads to CR than in those who did not (75% versus 25%; P less than 0.01). This relationship between CH leads to CR and five-year survival was confirmed in patients with localized disease, as shown in Stage II patients (respectively 97% versus 63%; P less than 0.05). Therefore the response to initial chemotherapy provides a new prognostic factor that may serve to delineate a "high-risk" group of patients. The latter deserve aggressive therapy while those in the favorable group would benefit from a less aggressive combined regimen that would minimize long-term complications.

Antineoplastic Agents↗