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Biomedical subjects

B Dreyfus

Publications and source records attributed to B Dreyfus.

At least 19 recordsLinked to original sources

Cytogenetics and molecular analysis in chronic myelogenous leukemia patients treated by interferon and chemotherapy.

A new combination regimen including chemotherapy and interferon was started in 1986 for the treatment of 24 chronic myelogenous leukaemia patients in chronic phase. Complete cytogenetic remission were noted in 10 patients and 6 of them are in sustained remission. The overall survival was 70% at 40 months. However, a minimal residual disease was detected in the 3 patients tested with the polymerase chain reaction. In addition no correlation was detected between the site of the breakpoint and response to therapy.

Adult

Combined therapy interferon and chemotherapy in chronic myelogenous leukemia.

In view of the results achieved by others with interferons and also with low doses of Cytosine-arabinoside, we have treated 24 patients with IFN alpha 2a and Hydroxyurea for induction, with addition of low dose Ara-C during IFN maintenance therapy when the cytogenetic results were deemed insufficient. Complete hematological responses were obtained in 75% of the cases. Cytogenetic responses were noted in 83% of 12 previously untreated (complete or almost complete response in 3) and in 50% of previously treated patients (complete or almost complete response in 3 patients).

Adolescent

Karyotypes of 33 patients with clonal aberrations in chronic lymphocytic leukaemia. Review of 216 abnormal karyotypes in chronic lymphocytic leukaemia.

We report on 33 unpublished patients with clonal anomalies in chronic lymphocytic leukaemia. The literature was thoroughly reviewed in order 1) to quantify the frequency of anomalies found in chronic lymphocytic leukaemia and to give new status to the rarest, 2) to determine whether a given anomaly was an additional anomaly and/or a primary anomaly, and 3) to find out whether strong associations between different anomalies exist in this disease.

Chromosome Aberrations

[Acute leukemia with translocation (8;16)].

Three cases of M5 acute leukemia, 1 with a t(8;16)(p11;p13), 2 with variant translocations, both with a breakpoint at band 8p11, t(6;8)(q27;p11) and t(8;19)(p11;q13) are reported and 12 other published cases reviewed. This is a rare new entity, of variable but rather poor prognosis, seen from birth to 75 years of age, with several cases in infants. Leukemic cells, which often show conspicuous phagocytic activity on bone marrow smears, bear both monocytic and granulocytic markers in at least some cases. The best therapeutic regimen is not well-defined. The molecular basis of the disorder remains to be elucidated.

Adult

[Disseminated aspergillosis in acute leukemia probably chemically-induced].

One case of invasive aspergillosis in patient with myeloblastic leukemia probably induced by chimiotherapy is reported. Aspergillosis is a severe infection in immunodepressed host with 70 to 80% of mortality. Early diagnosis can be often carried by broncho-alveolar lavage, route of entry is always pulmonary one. Rapidly established antimycotic treatment can avoid death. Laminar air flow rooms and air spores numeration allow prevention of this infection in the immunodepressed host.

Adult

[Multiple myeloma with high tumoral mass. Treatment combining melphalan, cyclophosphamide, vincristine, CCNU and prednisone. 35 cases].

The results of a combination chemotherapy trial (melphalan, CCNU, vincristine, cyclophosphamide) involving 28 patients with stage III (n = 21) or stage II (n = 7) multiple myeloma suggest a high response rate, with a mean 71% malignant cell destruction in 78.5% of the patients. A longer survival in responsive stage III patients as compared with patients treated with alkylating agents seems likely, but this can only be established by a randomized trial. Despite haematological side-effects, this combination therapy may be used in high risk patients, especially those resistant to a single alkylating agent and in whom the frequency, intensity and duration of responses appears to be the same as in previously untreated patients. In contrast, only one of the 7 patients treated for relapse after previous response to a single alkylating agent responded to the combination chemotherapy.

Adult

Adult T-cell lymphoma leukemia in Western countries.

A new T-cell disorder has recently emerged: the so-called adult T-cell lymphoma leukemia (ATLL) initially described in Japan. Subsequently, ATLL cases were recognized in patients from the Caribbean. We summarize the clinical and hematological features of 19 published cases from Western countries, in addition to a new case we encountered. The leukemic cells display characteristic morphological features and a T3+T4+T8-T6- surface antigenic phenotype. Overall survival is of short duration, but remission could be obtained in our case despite a subsequent relapse in skin and CNS. Geographic clusters of ATLL cases have led to the discovery of the possible role of a new retrovirus, HTLV, in the genesis of this rare malignancy.

Adult

Reticulocytosis, hypochromia, and microcytosis: an unusual presentation of the preleukemic syndrome.

Two patients exhibiting a highly unusual preleukemic syndrome with marked reticulocytosis, hypochromia, and microcytosis are reported. This red cell phenotype has been investigated by means of HbF, HbA2, and i-antigen activity dosages, immunofluorescence labeling of F cells, reticulocyte survival, globin chain synthesis, and electron microscopy study. The marked reticulocytosis is explained by a delayed disappearance of the reticulum. Serum iron is normal, and a thalassemic syndrome is excluded because of a balanced alpha/non-alpha globin chain synthesis. Electron microscopy studies are consistent with a defect in iron uptake by erythroid cells. All the hematologic data and investigations are similar to those observed for the Belgrade laboratory rat. It is hypothesized that the low expression of HbF and i-Ag associated with microcytosis are related to a prolongation of erythroid maturation as reflected by abnormal reticulocyte survival.

ABO Blood-Group System

Respiratory distress of hyperleukocytic granulocytic leukemias.

The rapid appearance of acute respiratory distress during the course of 25 hyperleukocytic leukemias was associated with the rapid increase of the leukocytosis. The regression of the tachypnea was spectacular when treating hyperleukocytosis by exchange transfusion and chemotherapy. Blood gas studies, although blurred to some extent by in vitro blast consumption of oxygen, showed a hypoxemia with a hypo-or normocapnia. The symptoms seem to be related to the leukostasis by the mechanical obstruction of the pulmonary capillaries. This leukostasis was shown to be responsible for a septal and alveolar oedema. The high frequency of this syndrome during the course of AGL and of acute phase of CGL seems to be linked to the low deformability of the myeloblasts. In CGL at its chronic phase, CLL or even in ALL, the absence of this syndrome could be explained by the greater deformability of the circulating cells. The hyperleukocytic AGL patients which do not have this syndrome are all characterized by a stable or slowly increasing leukocytosis. Thus, this syndrome seems to characterized by hyperleukocytic granulocytic leukemias with a rapid blood leukocyte doubling rate. Treatment in such cases is an emergency.

Adolescent

Ultrastructural localization of peroxidases in 'undifferentiated' blasts during the blast crisis of chronic granulocytic leukaemia.

Twelve cases of Philadelphia chromosome positive chronic granulocytic leukaemia (CGL) in blast transformation have been investigated using ultrastructural peroxidase detection. In all cases, the leukaemic blasts were negative for myeloperoxidase on the basis of standard cytochemistry. In nine cases a variable proportion of blasts contained peroxidase activity detectable only by electron microscopy, permitting definition of their myeloid nature. By their distinct characteristics and localization, different peroxidase activities were recognized. Thus, several types of blasts were identified: megakaryoblasts (MKB), basophil promyelocytes (BPM), myeloid blasts with small granules containing peroxidase (MyB), and proerythroblasts (ProE). MKB were predominant in two cases and present in four cases, mixed with other myeloid blasts. BPM were abundant in one case and present in seven cases. MyB were identified as a majority in four cases. Three cases remained without any peroxidase. It is concluded that ultrastructural detection of peroxidases is of value for the identification of early myeloid blasts. Their high incidence and the simultaneous presence of several myeloid precursors suggest that during the blast crisis the target cell is frequently a pluripotent myeloid stem cell.

Bone Marrow

[An atypical form of Waldenstrom's macroglobulinaemia with complete remission lasting for more than two years after multiple chemotherapy (author's transl)].

An authentic case of Waldenstrom's macroglobulinaemia without a tumoural syndrome is reported, with initial signs of peripheral pancytopenia. Complete and persistent remission appears to have followed multiple sequential chemotherapy. This very satisfactory and, according to data in the published literature, very rare result suggests that this type of treatment should be prescribed for all patients with macroglobulinaemia with poor prognostic clinical and histological characteristics.

Adult