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Biomedical subjects

A Delannoy

Publications and source records attributed to A Delannoy.

At least 55 records · Page 3Linked to original sources

Biological and radiological investigations in patients with an increased red blood cell mass: Which are needed? Which are useful? which are unnecessary?

A few simple, low-priced, outpatient investigations, including an upper abdominal ultrasonography, a measurement of the arterial oxygen saturation and, in some cases, a leukocyte alkaline phosphatase score (LAP) suffice to find out the etiology of an increased red cell mass in most patients. The diagnosis of polycythemia vera (PV) could be accepted in patients with an increased red cell mass if the spleen is enlarged and if the arterial oxygen saturation is normal, although the latter measurement may not be required in typical cases. If the spleen is not enlarged, the diagnosis of PV could be accepted if, in addition, the leukocyte or thrombocyte count is increased. An elevated LAP score also points to the diagnosis of PV, provided fever or inflammation are not present. If, at that stage, an etiologic diagnosis has not been made, smoker's polycythemia should be considered and excluded. The next step should include a serum erythropoietin assay and culture of erythroid stem cells before performing investigations aimed at ruling out the many conditions associated with secondary polycythemia.

Alkaline Phosphatase↗

Hypothyroidism and arthritis during interferon therapy.

The development of autoantibodies during interferon therapy is frequent, but clinical symptoms of autoimmune disease are uncommon. We report on a female patient who developed arthritis with strongly positive antinuclear factor (ANA) and autoimmune thyroiditis while being treated with alpha 2b interferon (IFN) for chronic myelocytic leukaemia (CML). The arthritis subsided promptly after discontinuation of IFN and initiation of nonsteroidal anti-inflammatory drugs.

Antibodies, Antinuclear↗

Von Recklinghausen neurofibromatosis and hematologic malignancies: 2 case reports in adulthood.

Von Recklinghausen's neurofibromatosis is a hereditary disease predisposing to distinctive malignant hemopathies. These often develop during early childhood and are characterized by particular cytologic subtypes: juvenile chronic myeloid leukemia, monosomy 7-associated myeloproliferative syndrome and myelomonocytic leukemia. The etiopathologic mechanism underlying this association begins to be elucidated: the neurofibromatosis gene behaves like a tumor suppressor gene; its inactivation by mutation results in activation of the corresponding oncogenes. We report here the cases of two late-aged adults with neurofibromatosis: the first developed acute myelogenous leukemia, the second polycythemia vera. Based on a review of the literature, we suggest that, in opposition to childhood, the association between neurofibromatosis and malignant blood diseases is not demonstrated in adulthood.

Aged↗

Cigarette smoking and chronic polyclonal B-cell lymphocytosis.

A syndrome associating a polyclonal increase of B-cells with binucleate lymphocytes in peripheral blood and a polyclonal increase of serum IgM, accompanied or not by splenomegaly and lymphadenopathy, has been reported in young to middle-aged women who are cigarette smokers. The majority of patients were HLA-DR7 positive and their clinical course remained stable over periods of years. We report four cases of this syndrome. In all patients, DNA analysis provided additional evidence that the lymphocytosis was polyclonal, while in one patient who met all other symptomatic criteria the total lymphocyte count was normal. This would suggest that unless careful attention is paid to the morphology of the lymphocytes, the syndrome may go undiagnosed.

Adult↗

Clonal rearrangement of the T-cell receptor beta-chain gene in the pleural fluid of a patient with thymoma.

Thymoma is generally considered as an epithelial malignancy surrounded by lymphocytes not belonging to the tumour. This report documents a case of malignant thymoma associated with a lymphocytic pleural effusion. The pleural lymphocytes were mature T-cells, a small proportion of which were shown to have a monoclonal rearrangement of the beta-chain of the T-cell receptor. On the contrary, the lymphocytes of peripheral blood exhibited a germline configuration. The nature of the monoclonal population found in the pleural fluid is discussed and if the cells are of thymic origin their clonal configuration may point to a neoplastic nature of lymphocytes infiltrating malignant thymoma. Alternatively, the clonal T-cell subpopulation may result from an aberrant immunological response to the thymoma.

Clone Cells↗

High-dose cytosine arabinoside intensification for acute nonlymphocytic leukemia in patients over 56 years of age.

One hundred and nine consecutive patients with de novo acute nonlymphocytic leukemia aged over 56 years were admitted with the intention of administering high-dose cytosine arabinoside (HD Ara-C) intensification. After remission induction, the patients were consolidated with a course of daunorubicin (30 mg/m2/day, days 1-3) and Ara-C (100 mg/m2/day, days 1-7), followed by the intensification (Ara-C, 2 g/m2/12 h, days 1-4). The planned induction course was not started in 13 patients because of cardiac failure or unsatisfactory general status. Remission was achieved in 55% (53/96) of the patients. Twenty-seven patients (28%) had refractory disease, seven died early during induction therapy, five died of hemorrhage and three of infection during the hypoplasia that followed induction treatment. Thirty-nine patients started consolidation and 32 had the planned intensification. In these last patients the 3-year leukemia-free survival (LFS) probability was 29% (SE, 8%). No patient died as a consequence of intensification. The relapse rate of the intensified patients did not differ from the relapse rate of those patients who did not receive the planned intensification (p = 0.12). The only pretreatment variables significantly associated with a better LFS were younger age (p = 0.02) and a low WBC at diagnosis (p = 0.04). For the whole patient group, the 3-year survival probability was 15% (SE, 4%). This study shows that elderly patients can tolerate HD Ara-C. The patients completing consolidation-intensification have a currently acceptable LFS. To what extent HD Ara-C contributed to the length of the remissions remains unclear.

Aged↗

GM-CSF therapy for drug-induced agranulocytosis.

Five patients with drug-induced agranulocytosis were treated with recombinant granulocyte-macrophage colony-stimulating factor (GM-CSF)). With the exception of one patient who experienced a very short period of neutropenia, GM-CSF did not appear to alter the natural course of the disease. From our experience and from a critical review of the literature we conclude that the efficacy of GM-CSF in agranulocytosis is far from certain, and that the use of growth factors in this condition remains investigational.

Adult↗

Amylase-producing IgD-type multiple myeloma.

We describe the case of a 63-year-old woman with an IgD-type multiple myeloma and hyperamylasaemia. The evolution of the amylase concentration, the immunohistochemical data and the intracellular amylase contents of the plasma cell were consistent with secretion of amylase by the malignant clone. Moreover, cytogenetic analysis of the bone marrow revealed two structural rearrangements involving chromosome 1 near the amylase locus. Multiple myeloma should be added to the amylase-secreting tumours. This rare entity is not confined to Japan, where it was first recognized.

Adrenal Cortex Hormones↗

Allogeneic or autologous bone marrow transplantation for acute non-lymphocytic leukemia in first remission.

One hundred and seven consecutive patients with acute non-lymphocytic leukemia (ANL) aged less than 56 years were allocated to receive either allogeneic (allo-BMT) or autologous bone marrow transplantation (auto-BMT) when first complete remission (CR1) was achieved. CR was obtained in 96 patients. Twenty-four patients had an HLA-identical sibling donor and 20 of these (83%) had an allograft in CR1. Thirty-three patients (44% of the CR1 patients without donor) had an autograft in CR1. The reasons for not transplanting patients in CR1 were early relapse (nine patients), refusal (11 patients) or medical problems (23 patients). The 4-year leukemia-free survival (LFS) probability for all the CR1 patients was 25%. For the allo-BMT patients, the 4-year LFS was 71%, and for the auto-BMT patients 31% (log-rank p = 0.028). The relapse probabilities were 33% and 48% respectively (p = 0.40). If the results are analysed according to the intent of the protocol, patients with a donor had an LFS of 53%, and patients without a donor an LFS of 16% (p = 0.003). This study confirms the value of allo-BMT for consolidation of ANL in CR1. The attempt to autograft all CR1 patients without a compatible donor has not resulted in any marked improvement of LFS.

Adult↗

Acute lymphoblastic leukemia in the elderly.

We report our findings in 18 patients with acute lymphoblastic leukemia (ALL) aged 60 years or older. A preleukemic syndrome was observed in 2 patients. Compared to younger adults with ALL, L3 morphology was unexpectedly frequent (4/16). T-ALL was not observed. Other criteria of poor prognosis (high white blood cell count, CNS involvement, organomegaly, high serum LDH) were similar to those reported in young adults. 12 patients were treated with an OPAL-derived regimen, 4 with the MAV regimen, 1 with vincristine and prednisone, 1 with 6-mercaptopurine. Complete remission was achieved in 8 patients but proved short-lived. 5 patients died in aplasia and 5 failed to achieve remission. Median survival for the whole group was 3 months. ALL in the elderly raises the dilemma of an aggressive disease in patients with poor tolerance to intensive therapy.

Adult↗

High-dose cytosine arabinoside and amsacrine or mitoxantrone in relapsed and refractory acute myeloid leukaemia: a prospective randomized study.

52 patients with refractory or relapsed acute myeloid leukaemia (AML) were randomly assigned to receive a combination of high-dose cytosine arabinoside (HD Ara-C), 3 g/m2/d and either mitoxantrone (MTX), 7 mg/m2/d (5 mg if older than 60 yr) or m-amsacrine (AMSA), 120 mg/m2/d (90 mg if older than 60 yr) for 5 d. The overall response rate was 50% and did not differ significantly in the two groups (46% for AMSA and 56% for MTX, p = 0.415). The median survival was 11 months (8 months for AMSA and 12 months for MTX, p = 0.326) and the median duration of complete remission (CR) was 11 months for AMSA and 12 months for MTX (p = 0.643). In relapsed patients, the only significant predictive factor for obtaining a complete response was the length of first complete remission. Patients with a first CR shorter than 6 months had a CR rate of 36% while it was 65% if the first CR lasted more than 6 months (p = 0.03). Severe (WHO grade III-IV) gastro-intestinal toxicity was more frequent in the AMSA group (27% vs 4%, p = 0.021). Treatment-related death occurred in 4 patients in the AMSA group and in 2 patients in the MTX group (p = 0.097). We conclude that neither of these two treatment modalities was shown to be superior in terms of CR rate and survival, with a better tolerance for MTX.

Adult↗

Interferon alpha induced and maintained complete remission in chronic granulocytic leukemia in relapse after bone marrow transplantation.

We describe a patient with chronic granulocytic leukaemia (CGL) who relapsed after allogeneic bone marrow transplantation (BMT). Interferon alpha 2b (IFN alpha 2b) induced and maintained a complete remission. IFN alpha 2b led to full restoration of donor bone marrow. This case provides evidence to support a trial of IFN alpha in patients with relapsing CGL after BMT.

Adult↗