[Paraneoplastic sideroblastosis: disappearance after cutting away the malignant tumor].
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Biomedical subjects
Publications and source records attributed to A Broustet.
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Leukaemias which complicate myeloma under treatment are usually acute non-lymphocytic leukaemias. We report here a case of acute leukaemia with lymphoblastic features occurring 30 months after diagnosis of myeloma. The exceptional character of this association lead us to refine the cytological diagnosis by studying surface markers and ultrastructural cytochemistry. Because of the absence of T or B markers, and the absence of peroxidase activity in the nuclear envelope, the endoplasmic reticulum, and the Golgi apparatus of blastic cells, we conclude that this leukaemia is "null" lymphoblastic.
An 8-year-old girl with profound mental retardation and a neurologic syndrome associated with morphologic abnormalities was found to have a supernumerary small submetacentric chromosome. Several members of her family carried a balanced translocation t(12;18)(p12;q11), and the child's karyotype could be explained by 3:1 maternal segregation (tertiary trisomy). The proband was trisomic for 12p13 and 18p. A gene dosage effect was demonstrated for triosephosphate isomerase and glyceraldehyde-3-phosphate in erythrocytes and leukocytes allowing us to assign the corresponding loci to the tip of the chromosome 12 short arm.
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A case of complex structural rearrangement of chromosome 4 identified by R-banding as 46,XX,del(4),inv(4)(pter leads to 16::q24 leads to p16::q32 leads to qter) is reported in an infant with congenital anomalies and psychomotor retardation.
Kaposi's sarcoma was diagnosed in a 62 year old female at the last stage of an indefined malignant lymphoma. Clinically and histopathologically, some cutaneous aspects were consistent with mycosis fungoides. However, the histological examination of lymph-nodes led to the diagnosis of Hodgkin's disease, sometimes associated with features of Kaposi's sarcoma. During the past few years, the occurrence of Kaposi's sarcoma in the course of cancers, malignant lymphomas and especially during Hodgkin's disease, has been reported. It has been suggested that immunosuppressive therapy undertaken for the initial tumor could account for a viral carcinogenesis. In this respect, the vascular proliferation observed in Kaposi's sarcoma could be compared with the lymphocyte induced angiogenesis, which occurs during the experimental graft versus host reaction. Therefore, Kaposi's sarcoma could be the result of a tumoral rejection. In the case of our observation, it does not seem possible to diagnose an angio-immunoblastic lymphadenopathy; but it is of interest to note that, in this later disease, histological features of vascular neogenesis have also been reported.
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Monosomy 9p is reported in a boy with trigonocephaly and advanced bone age.
In 30 couples consulting after two or more failed pregnancies, the authors detected two women with reciprocal translocation in the heterozygous condition: t (5 ; 6) (q 35 ; q 21) in one case. t (5 ; 10) (q 15 ; q 25) in the other case. In both cases, there was, later, birth of a normal boy of karyotype 46, XY. It thus appears obvious that the balanced structural changes which occur in the fertility of these couples, reduces the latter.
The case of a sibship of 4, 2 members of which present aneuploïdy (45,X and 47,XX,21+) is reported. The paternal grandfather and grandmother are first cousins and there is a large number of centromeric associations in the father.
A 17-month old boy is partially trisomic for 7q22 and 7q31 due to a probable insertion in the paternal chromosome 13. The phenotype of the patient is similar to that of two other patients reported in the literature.
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