[Fulminating hepatitis by type 5 adenoviruses during Hodgkin's disease].
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Biomedical subjects
Publications and source records attributed to J Pris.
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Some imperfectly known clinical aspects of polychondritis chronica atrophicans (relapsing polychondritis), as extracted from a series of 12 cases, are presented. The osteoarticular lesions are sometimes unusual, involving the temporomandibular or cervical articulations, and the renal lesions may be severe. Pericarditis seems to be more frequent than usually mentioned in the literature. Pulmonary complications are not always those which are classically associated with lesions of the tracheo-bronchial cartilages. Haematological manifestations are not uncommon and must be taken into account when evaluating the prognosis of the disease.
Idarubicin (IDR) is a new anthracycline that can be administered orally. Oral IDR was given at a dose of 30 mg/m2 daily for 3 d in 20 patients aged 65 to 79 yr with previously untreated acute myeloid leukemia (AML). 5 patients whose marrow remained blastic at d 14 received a second course. 8 patients achieved complete remission (6 after one single course). There were: 1 early death, 4 deaths in aplasia, 7 failures. The hematologic toxicity was high. All but 1 patient had to stay in hospital and the duration of neutropenia was 12 to 34 d (median 19). Oral IDR is an effective therapy for AML in elderly patients but the total dose of 90 mg/m2 is too aggressive to be administered safely outside the hospital.
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In 22 consecutive patients treated by allogenic bone marrow transplantation, the authors report their experience in complete gastrointestinal decontamination and prophylactic systemic vancomycin. Neither septicemia from the low digestive tract nor with Gram positive is noticed. A child developed septicemia with Capnocytophaga ochracea, resistant to vancomycin. There is no infectious death in this study and no significative toxicity is reported.
Immunohistochemistry using monoclonal antibodies has been proved of diagnostic value in bone marrow pathology. Several monoclonal antibodies identifying antigens not denatured by fixation are now available. In anaplastic tumors infiltrating bone marrow these antibodies allow the distinction of large cell lymphomas which express the leucocyte common antigen (Dako-LC+) from undifferentiated carcinomas which are reactive with anti-cytokeratin antibodies (KL-1+, Dako-EMA+), neuroblastoma (NSE+) and rhabdomyosarcoma (desmin+). KL-1 and Dako-EMA antibodies are also considered to be powerful tools to disclose micrometastases from carcinoma, particularly breast carcinoma, which otherwise may be undiagnosed. The detection of residual or minimal bone marrow involvement in malignant lymphomas (particularly Burkitt type) remains difficult. However, in some cases, Dako-LC and MB2 (anti-B) antibodies are useful to detect bone marrow involvement in some lymphoproliferative disorders composed of small or medium-sized cells scattered among hematopoietic cells, and in some large cell lymphomas (i.e. centroblastic or immunoblastic types). These antibodies allow the detection of minimal or residual involvement by malignant cells, which can be confused with immature hematopoietic cells, on routine stainings. No anti-T antibody was found to react satisfactorily with T cell on decalcified biopsy specimens. In anaplastic large cell lymphomas (so-called malignant histiocytosis), malignant cells coexpress Ki-1 and EMA antigens. Antibodies recognizing these two antigens were found to be of value to demonstrate scarce neoplastic cell in bone marrow.
We have studied the plasma pharmacokinetics of idarubicin (4-demethoxy-daunorubicin) in eight leukemia patients receiving five daily i.v. injections (7-9 mg/m2 per day) of this new anthracycline. For unchanged idarubicin, similar pharmacokinetic parameters were exhibited after the 1st and the 5th injection. Idarubicinol (13-dihydroidarubicin) was identified as the only detectable metabolite of idarubicin in plasma. Due to a protracted half-life (40 h) this compound progressively accumulated in plasma without the occurrence of peaks after the injections. This administration schedule provides therefore an interesting method of dose fractionation of a new anthracycline.
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The frequency and importance of prolonged bleeding time were studied in patients affected by a severe anemia (haemoglobin less than 8 g/dl). A Simplate bleeding time test was performed in 25 patients suffering from various haematological disorders, with a platelet count greater than 100,000/cu mm and a normal or increased factor VIII complex. Patients with acute leukaemia, myeloproliferative disorders or chronic renal impairment were excluded from the study. Bleeding time was prolonged in 12 patients; their mean haematocrit was not different from that of the 13 other patients whose bleeding time was in the normal range. Bleeding time was less prolonged than in patients with chronic renal insufficiency in spite of a lower mean haematocrit (previous study). Fifteen patients were investigated a second time after partial or full correction of the haematocrit; in all but one, the bleeding time was reduced and/or normalized. This study suggests that severe anaemia may be an additional hemorrhagic risk factor in patients with another cause of bleeding.
Two leukemia patients, refractory to chemotherapy, were treated with T101-ricin A-chain immunotoxin (T101 IT). Patient 1 (T-ALL) received a single 13.5 mg dose of T101 IT IV (12-hour infusion). Patient 2 (B-CLL) was treated with a daily 25 mg dose of T101 IT IV (two-hour infusion) over three consecutive days. Patient 2 also received 300 mg of chloroquine IM on days two and three as enhancer. In vivo binding of T101 IT was demonstrated by FACS analysis using either an antimouse Ig-FITC or anti-A-chain-FITC antibodies. Following IT therapy, the expression of T65 antigen on target cells dropped to 50% and 20% of pretreatment levels, respectively. In patient 1, circulating blast cells remained unsaturated during therapy while in patient 2, cells were fully saturated for four to six hours following each infusion. Pharmacokinetic studies showed a rapid clearance of T101 IT after IV administration. Antimouse and anti-A-chain antibodies could not be detected. There were no treatment-related adverse effects. In patient 1 a rapid but transient decrease of target cells was observed, possibly related to the administration of the antibody part of T101 IT. In contrast, patient 2 showed a 40% reduction of the lymphocyte count, which remained stable over a period of 2 weeks. Such a clinical benefit following IT therapy in patient 2 could be ascribed to the absence of circulating free antigen and the complete saturation of target cells.
A blastic crisis of chronic myeloid leukemia without a detectable chronic phase is reported. At diagnosis, blast cells present t(9;22)(q34;q11),t(14;14)(q11;q32) translocations and early B cell phenotype (DR +, TdT +, B4 +, BA1 +, J5 +). At relapse, the malignant clone evolves to a biphenotypic expression, the initial markers remain unchanged, and two myeloid antigens (My 7, My 9) appear. The wide overlap in percentages of blast cells displaying lymphoid and myeloid markers shows that a single clone bears antigens of both lineages. Simultaneous occurrence of a t(14;14)(q11;q32) translocation, usually found in T cell malignancies, and of a B cell phenotype raises the question of the relationship between chromosomal changes and surface marker expression. The malignant cell is assumed to be a progenitor cell, already committed to lymphoid lineage and retaining the potential to switch to myeloid lineage.
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A simultaneous investigation of the kinetics of serotonin (5 HT) uptake and of binding sites was carried out in the platelets of normal subjects and of 10 patients affected with various types of myeloproliferative disorders (MD). The 5 HT uptake was analysed according to the Lineweaver-Burk and the Eadie-Hofstee methods. With the two methods, the patient's platelets exhibited a dramatic reduction of the Vi max and of the Km; in some patients the Eadie-Hofstee analysis revealed that a passive diffusion phenomenon is superimposed on the active 5 HT uptake at least for the higher concentration used. The binding data were analysed with the Scatchard method. Two classes of binding sites (high affinity - low capacity, low affinity - high capacity) were found in normal subjects and patients. Pharmacological studies with imipramine, a specific inhibitor of 5 HT uptake, suggested that both the sites are involved in 5 HT uptake. The number of both binding sites was significantly decreased in patient's platelets while the affinity constants of these binding sites were not significantly reduced in comparison with those of the control subjects. No correlations were found between Vi max, Km and the number of binding sites. These results suggest that a reduction in the number of platelet membrane acceptors for 5 HT commonly occurs in myeloproliferative disorders but does not provide a full explanation of the uptake defect.
With reference to a case of malignant toxoplasmosis in a patient with angioimmunoblastic lymphadenopathy, the following features are recalled: in compromised hosts, clinical manifestations of malignant toxoplasmosis are highly variable, with neurologic signs and fever being predominant; the occurrence of lesions of the fundus, such as chorioretinitis, is an essential clue to the diagnosis; serologic tests for toxoplasmosis should be performed routinely at each stage of the disease in order to ascertain seroconversion through comparison with baseline results.
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A 31-year-old man presented with rapid onset of intractable congestive heart failure during the course of chemotherapy for eosinophilic leukemia. Patients with a hypereosinophilic syndrome usually die from complications of eosinophilic infiltration and fibrosis in target organs. The resulting cardiac lesions are a cause of death among these patients. Surgical intervention enabled our patient to survive the immediate medical crisis and has prolonged his life.
Mouse erythrocyte-rosette-forming cells (MRFC) were determined in 76 cases of chronic lymphocytic leukaemia (CLL), 19 cases of centroblastic-centrocytic follicular non-Hodgkin's lymphoma (NHL), five cases of hairy cell leukaemia (HCL) and in 62 control patients. The mean percentage of MRFC in B-CLL was 54.8% +/- 24.4%, in nodular centro-follicular NHL 2.3% +/- 3%, in hairy cell leukaemia 14.6% +/-14.7% and among the controls 3.2% +/- 1.9%. Thus, MRFC appear to be an excellent marker of B-CLL and sometimes the only B cell marker as in 11 cases of SIg negative CLL. There is no correlation between the percentage of MRFC and clinical staging according to Rai classification whereas patients with stage 2 in Binet's classification (isolated splenomegaly) have a significantly lower MRFC percentage (23.4% +/- 27.7%; P less than 0.05). There is no correlation with prognosis, nor with serum Ig levels. We found no correlation with surface phenotype IgM, IgM-IgD or IgG. Thus, this study does not confirm the hypothesis that MRFC might characterize and immature stage in the B lymphocyte differentiation. However, these results are consistent with another hypothesis which states that lymphocytes in CLL possibly originate from a clonal expansion of a normal B lymphocyte subset, characterized by a low concentration of SIg and a receptor for mouse erythrocytes.