Cross immunoelectrophoresis of serum A1AT (Pi M) shows "frequent" patterns with M2 less than M6 less than M4. Other "particular" patterns were found with M6 greater than M4 and M6 less than M2. The most often found patterns among the Negroid population in our study appeared dissimilar to that found among Caucasians. The "super-gene" concept and the variability of genetic flow may explain this difference. The "particular" patterns are often found in cord blood samples and in patients with hepatocarcinoma. They show similaritis between embryonic and cancerous processes. These different patterns also agree with the "super-gene" concept.

HLA phenotypes of 13 patients surviving in lasting first remission over 6 years after BCG immunotherapy for acute childhood lymphoblastic leukaemia (ALL) were compared to phenotypes of normal subjects and of surviving ALL patients treated exclusively with chemotherapy. Among the BCG-treated patients, the frequency of the antigen HLA-BW 17 was 46.1% vs 7.3% in healthy controls (p less than 0.001) and the frequency of the antigen HLA-AW 33 was 30.8% vs 1.2% (p less than 0.001). 9 patients possessed at least one of these two antigens (69.2% vs 8% in controls p less than 0.001). Phenotypes of the chemotherapy-treated patients did not differ significantly from controls. These results suggest the existence in humans of HLA-linked genes which are involved in the response to BCG immunotherapy in ALL.

A fifty-year old patient was treated for acute lymphoblastic leukemia. One month after a complete remission, a syndrome suggesting chronic myeloid leukemia led the authors to study the marrow karyotype which revealed the existence of a Philadelphia chromosome. A second lymphoblastic attack occurred rapidly and a second complete remission was easily obtained. A few weeks later, occurred lymphoblastic meningitis. A new cytogenetic study then showed duplication of the Philadelphia chromosome. One may imagine that the initial attack represented acute lymphoid transformation of chronic myloid leukemia. The theoretical and practical significance of this case is discussed.

An account is given of a family from the Canton of Valais, suffering from hereditary adenocarcinomatosis. The pedigree extends over four generations; the first three comprise 47 individuals (28 males, 19 females), of whom 21 (16 males and 5 females), i.e. 44.6%, are affected with malignant tumours. Of the 32 people in the fourth generation, only one individual is affected to date (a girl aged 21, IV/14). There were 27 tumours in all: 16 adenocarcinomas of the colon, two gastric adenocarcinomas, one duodenal adenocarcinoma, one rectal adenocarcinoma, one papillary carcinoma of the ovary, one osseous sarcoma, one cutaneous fibrosarcoma, a multiform glioblastoma of the basal nuclei of the brain, a basocellular epithelioma, also a cerebral metastasis from an adenocarcinoma, the origin of which has not been established, and a tumour invading the biliary tract. Three members of the family suffered from multiple tumours. In three of the patients, the colonic adenocarcinoma was accompanied by one or two polyps. The average age at the onset for all the tumours was 45 years. It was definitely lower in the third than the second generation (anticipation). The transmission was autosomal dominant, with predilection for the male sex (57.1% male and 26.3% female patients). The penetrance was about 80%. The author finally discusses the diagnostic criteria for hereditary adenocarcinoma and reviews the different familial forms of cancer.

Chromosome C variants have been analyzed in individuals with hematological disorders. The incidence of chromosome 1 gh+ was significantly enhanced in CML patients (20/24) compared with controls (8/17). The distribution of C-variants of chromosomes 9 and 16 was not different in these individuals.

Two brothers developed acute leukemia, one at the age of 7 months and the other at the age of 14 months. Both suffered from a staturoponderal retardation and the same malformation syndrome. The karyotype carried out only on the second child revealed breaks and chromatid changes. A diagnosis of Fanconi's anaemia can be discarbed since no blood cytopenia preceded the leukemia. Finally, the diagnosis of Bloom's syndrome prevailed despite the absence of telangiectatic erythema and the atypical chromosomal anomalies.

Cytogenetic analysis of 58 cells originating in 7 mammary carcinomas indicated the existence of characteristic numerical and structural modifications, concerning the long arm of chromosome 1, chromosomes 4, 5, 7, 14, 15 and X, and the short arm of chromosome 16. Establishing the alterations of chromosomal "markers" could not only depend on selective phenomena relating to modifications of the genome but also on the preferential reassociations of heterochromatic segments.

In ten cases of apparently primary acute leukaemia, the discovery of a Philadelphia chromosome at routine examination of the caryotype led a diagnosis of blastic crisis of chronic myeloid leukemia. The clinical, cytological and cytogenetic pictures varied and only routine caryotypic examination may be used in reaching the diagnosis. The prognosis appear to be less bad than in blastic crises occurring after a long course of chronic myeloid leukaemia and closer to that of primary acute myeloid leukaemia.

The François' syndrome associates disseminated firm nodular subcutaneous lesions, a deforming arthropathy and a corneal dystrophy. Inheritance seems to be recessive. Six cases of this rare syndrome have been previously published. The syndrome is reported here in two Mexican brothers. In the authors' opinion the François' syndrome is not related to the xanthomatoses. It should be considered as a genetically recessive arthropathic nodular fibromatosis.

About a spontaneously regressive case of osteo-cutaneous congenital fibromatosis, the authors describe the characteristics of the disease (32 observations). Although the majority of cases are sporadic, 3 familial observations are in favour of a dominant autosomal transmission of low penetrance. Study of the familial cases and analysis of the different localisations demonstrate the unicity of the so-called diffuse forms with visceral involvement and of the so-called generalized forms without visceral involvement congenital fibromatosis is characterized by several fibromas at birth: in two-thirds of the cases, it is a purely cutaneous or osteocutaneous form, which disappears spontaneously; in one third of the cases, it is a cutaneous or osteo-cutaneous form with lethal visceral involvement.

The authors present three cases of multiple, intra-cranial meningiomatosis with contact hyperostosis affecting the grandmother, mother and daughter, in a very stereotypic manner. No other sign of von Recklinghausen's disease was noted, except for two tiny neurofibromas on the left hand in the mother. Only the latter suffered from bilateral deafness, but it was not possible to give definite proof of the presence of an acoustic neuroma in this patient. Anatomical verification in the first case, and surgical intervention in the last, confirmed that it was in fact a meningioma. Thus, a new clinical form of expression of neuro-fibromatosis has been found.

Two cases of acute myeloblastic leukemia were observed in a young 22 year old woman, then two years later, in her brother aged 16 years. The elder brother had died previously probably from the same disease. The very rare families in which more than two members had the same type of leukemia show the possible intervention of a hereditary factor in leukemogenesis.