[Atypical urticaria disclosing acute basophilic leukemia. Apropos of a case associated with hyperhistaminemia]. / Urticaire atypique révélatrice d'une leucémie aiguë à basophiles. A propos d'un cas associé à une hyperhistaminémie.

Acute basophilic leukaemia (ABL) is a rare disease characterized by high fever, anaemia and haemorrhagic diathesis. Its prognosis is somber and its response to therapy mediocre. Death is mainly due to cerebral or digestive hemorrhage and coronary disease. In a very small number of cases ABL, like all diseases accompanied by an increase in basophils, may be associated with hyperhistaminemia responsible for cutaneous and gastric symptoms.

Impaired phagocytic activity of neutrophils in patients receiving haemodialysis: the critical role of iron overload.

The metabolic burst (as measured by the spontaneous and stimulated nitroblue tetrazolium tests), the phagocytosis of heat inactivated bakers' yeast and of Staphylococcus aureus, the killing of Staph aureus, and the myeloperoxidase activity of polymorphonuclear neutrophils were studied in 11 patients receiving maintenance haemodialysis. Of these patients, six were polytransfused and had high serum ferritin concentrations (mean 5940 (SD 2925) micrograms/l; group 1), and five had normal serum ferritin values (mean 171 (116) micrograms/l; group 2). Patients in group 1 had a history of more infectious episodes (0.167 v 0.025 per patient per month) and significantly more genitourinary infections (p = 0.015) than those in group 2. Phagocytosis and myeloperoxidase activity were severely reduced in group 1 but normal in group 2. Percentages of neutrophils ingesting one or more particles together with the index of phagocytosis in patients' serum were inversely correlated with serum ferritin concentrations. Four patients in group 1 were treated with desferrioxamine, and after six to 18 weeks of treatment phagocytosis and myeloperoxidase activity had returned to normal in three of them. These data suggest that in patients receiving haemodialysis iron overload due to multiple transfusions plays an important part in the mechanisms underlying the susceptibility to bacterial infections, mediated at least partially through impaired neutrophil function.

Fanconi's anaemia. Simultaneous onset in 2 siblings and unusual cytological findings.

Fanconi's anaemia is reported in 2 siblings. The simultaneous onset of pancytopenia after possible exposure to common external agents suggest that both a frail genotype and environmental factors may be etiologically involved in the disorder. One of the children died after having developed a monoblastic leukaemia. This patient disclosed very striking cytological abnormalities, including a dyserythropoietic pattern and ropalocytosis. The physiopathology of the latter abnormality, which has hitherto not been noticed in Fanconi's anaemia, is uncertain.

[Myeloma and hypereosinophilic leukemoid reaction (author's transl)]. / Myélome et hyperéosinophilie. Réaction leucémoïde à éosinophiles associée à un myélome.

In a 56-year-old man with a myeloma marked eosinophilia was discovered. The diagnosis of myeloma was made on the discovery of plasmocyte infiltration in the mandible. Despite surgery and chemotherapy, general bone infiltration by plasma cells occurred over 15 months. The leukocyte count rose to 226,000/mm3 with 162,720 mature eosinophils. Pathogenic problems suggested by such an unusual association (myeloma-eosinophilic leukemoid reaction) are considered.

[Iron metabolism during proteoenergetic malnutrition in children]. / Aspects du métabolisme du fer dans la malnutrition protéo-énergétique de l'enfant.

The iron stores are normal in marasmic kwashiorkor in kivu, but they notably decrease on re-feeding. On admission, the total iron binding capacity depends not only on the severity of the protein depletion but also on the iron stores. There exists an abnormality of the iron release from the stores; this cannot be attributed to the hypotransferrinemia or to the hypoceruloplasminemia, which are too moderate, nor to an ascorbic acid deficiency, but possibly to the inflammatory state which is a feature of kwashiorkor. The iron deficiency which occurs on re-feeding is not explained by abnormalities of iron absorption or by increased iron loss, but by the raised requirement for iron. Parental iron treatment alone is required to normalise the packed cell volume and the red cell volume after re-feeding.