The authors report the observation of a 76-year-old man who since 1974 had a persistent anaemia considered as a pre-leukaemic state. The patient was hospitalized in May 1977 with fever and severe asthenia. The laboratory results indicated a probable diagnosis of acute non-differentiated leukaemia of stem cells. In spite of treatment, the anaemia grew worse, the leukocytosis accompanied by blast cells became more pronounced, a massive thrombopenia occurred and the patient died in irreversible shock. Cytogenetic examination done on a medullar culture revealed the presence in all the cells of a chromosome No. 5 with the long arms deleted : 46,XY,5q--. This rare medullar anomaly was reported for the first time in 1974-1975 by the Louvain school (Van den Berghe, Sokal, et al.) in a group of refractory anaemias. It has also been described in association with other chromosomal aberrations, in anaemias or other hemopathies which all developed into acute myeloblastic leukaemia. The clinical evolution and the cytogenetic data of the patient presented here are compared with those of other cases of 5q-- published in the literature, and the significance of this 5q-- chromosome aberration in hemopathies is discussed.

The grounds for treating or not treating chronic lymphocytic leukemias (CLL) and chronic myelocytic leukemias (CML) are discussed in the light of results in recent years. In the case of CLL, objective evaluation of treatment benefits has hitherto been difficult due to the heterogeneity of this group of diseases. A recent classification, based on hematological and clinical parameters and apparently of prognostic value, will undoubtedly help to clarify the therapeutic problem. In the case of CML, routine chemotherapy programs certainly alleviate the symptoms of the disease but have not so far achieved a significant prolongation of survival.

Monozygotic twins aged 23 underwent surgery, at an interval of 4 months from each other, for a myxomatous tumour of the right ventricle which had been completely asymptomatic until then. The authors present the clinical, electrocardiographic and angiographic findings, together with details of the operation and of the histological findings. They recall how rare a site this is, and summarise some of the observations which have been made on familial myxomas.

The clinico-pathological findings are presented in a case of Peutz-Jeghers syndrome associated with a bilateral mammary invasive ductal carcinoma, a well-differentiated mucinous adenocarcinoma of the cervix and microscopic, bilateral ovarian sex cord tumors with annular tubules. The sex cord tumor with annular tubules was described in 1970 by SCULLY, who recognized its striking association with the Peutz-Jeghers syndrome. Two cases of adenocarcinoma of the cervix and another case of uterine adenocarcinoma of unspecified localization associated with Peutz-Jeghers syndrome were found in the literature. It is possible that women with Peutz-Jeghers syndrome run an increased risk of developing adenocarcinoma of the uterine cervix.

Ten clinical observations concerning six families with familial myeloproliferative disorders are reported. Family no. 1 : two brothers, RES with myelosclerosis and ROS with chronic myeloid leukemia. Family no. 2 : PG atypical myeloproliferative syndrome and his brother polycythemia vera. Family no. 3 : DF myelosclerosis and her son (DR) polycythemia vera. Family no. 4 : DM, polycythemia vera, the mother and a sister with splenomegaly. The brother died with myelofibrosis. Family no. 5 : GA and ML, cousins with polycythemia vera. Family no. 6 : MB and ZG, a brother and sister with polycythemia vera. No consanguinity and no toxic, infections or malignant etiology were found in these families. The literature reviewed emphasises the rarity of the familial incidence of myeloproliferative disorders.

Human colon adenocarcinomas from 52 patients were investigated for the presence of the colon polymorphic antigens WZ. The patients were typed for their WZ phenotype, using the immunofluorescence method on non tumoral colon mucosa sections: 27 patients were found W+ Z+, 18 W- Z+, and 7 W- Z-. The tumors were tested for the presence of the WZ phenotypes, using the immunofluorescence method and a radio-immunoassay. The WZ phenotypes were not expressed in the non secreting tumors, whatever the patient's phenotype. They were expressed in the secreting tumors and had the same phenotype as found in the corresponding normal mucosa. The WZ phenotypes were present in human developed into "nude" Mice inoculated either with differentiated colon carcinomas, or with a human colon carcinoma cell line (HT-29).

In the treatment of familial polyposis the conservation of the rectum is essential to assure a convenient control and rhythm of defecation. Meanwhile the future of resting polyps deserves the attention of the surgeon. Certain authors are optimistic and claim that spontaneous regression of the polyps is possible. Our experience illustrates that the rectal mucosa tends to fabricate new crops of polyps. The prolonged post operative observation of more than 4 years of polyposis in one family has permitted us to follow the evolution of the rectal mucosa and to deduce that gravity of the lesion is quite variable. The polypotique character of the rectal mucosa must influence the choice of certain surgical technic specially that nine of them achieve entire satisfaction yet.

Among 2966 acute leukemia, 26 familial cases were reported. Leukemia occured mainly in the first relative individuals and particularly in the sibship. The relative risk for a sib of leukemic patient is four time more than for random people. Leukemia was often similar among patients of the same family and the onsets of the disease occured approximatly at the same age whatever the time between the dates of diagnosis. Twins with leukemia were often monozygotous. Relative risk of leukemia among twins, decreases according to the age: the probability of leukemia for a twin is: (a) 100 p. cent if the other twin is leukemic before 1 year old; (b) 15 p. cent between 1 to 4 years old and (c) similar to other sib after 4 years old. Among chronic leukemias, only chronic lymphocytic leukemia seems to have a genetic background for susceptibility. Some familial diseases (congenital aplastic anemia, Bloom's disease, Ataxia telangiectasia) or congenital diseases (Down's syndrome) increase the risk of leukemia.

Since ten years, there is an extensive search for association between antigens of the major histocompatibility system HLA and malignant diseases. Data show only weak associations with Hodgkin disease and acute lymphocyte leukemia. For studies of a variety of solid tumours the difference between patients and controls do not attain statistical significance, except for nasopharyngeal carcinoma. According to the gene frequency variations in populations and the ethnic differences in some cancers, inter-population studies are possible. Significant geographic associations between some cancers and HLA antigens have been found. They give evidence for a genetic background of susceptibility or resistance to cancer.

The role of hereditary factors in the etiology of cancer is difficult to prove. However, one may precise a hereditary relationship in those cancers which are either transmitted as a monofactorial trait, or secondary to a hereditary disease, or linked to a genetic marker. Most often, it is necessary to fall back on familial investigations, which may sometimes indicate an excess of familial cases. However, a familial concentration of certain cancers may also be due to hereditary factors, as well as to a common environment.