A case report of Down syndrome and centroblastic lymphoma.

We describe a case of left cervical stage I centroblastic lymphoma in a 29-year old male patient with Down's syndrome due to a (14; 21) Robertsonian translocation. The disease presented as extensive lymph node necrosis leaving rare areas of tumor cells, accounting for the diagnostic difficulties. According to our review of the literature, lymphoma is one of the most common neoplasms in DS patients and may represent the second most common malignancy in this condition, far behind leukemia.

Assessment of liver iron content in 271 patients: a reevaluation of direct and indirect methods.

Two direct methods for hepatic iron assessment (liver iron concentration and stainable liver iron--judged by a new histologic grading) and three indirect methods (serum iron and transferrin saturation, deferoxamine-chelation test, and ferritinemia) were reevaluated in 271 patients. These patients consisted of: 103 with idiopathic hemochromatosis, 39 with alcoholic cirrhosis, 54 with noncirrhotic alcoholic liver diseases, 13 with nonalcoholic liver diseases, and 62 with miscellaneous disorders. The results indicate that: (a) liver iron concentration, well correlated with mobilized excess iron (r = 0.88; p < 0.01), is the method of reference and validates the proposed histologic grading; (b) serum ferritin, which is in good correlation with liver iron concentration (r = 0.80; p < 0.01), is a valuable indirect method for hepatic iron evaluation; (c) regarding the other indirect methods a "boundary zone" may be delimited, thus corresponding to liver iron concentration values of 10.7 mumol/100 mg dry liver weight, beyond which values of serum iron less than 28.6 micrometer or transferrin saturation less than 45% or chelatable iron less than 45 mumol/24 h are rare; and (d) using the various indirect methods, there is a marked risk in idiopathic hemochromatosis to underestimate and in alcoholic liver diseases to overestimate hepatic iron content.

[Pyoderma gangrenosum and chronic myeloïd leukemia. Two new cases (author's transl)]. / Pyoderma gangrenosum et leucose myéloïde chronique. A propos de deux cas.

Two new cases of pyoderma gangrenosum with chronic myeloid leukemia are described and added to three others of the literature. Pyoderma gangrenosum is often bullous, painful and progresses quickly. It seems to have a bad prognosis in myeloid leukemia as in polycythemia vera, in which it appears lately as an evolution to myelofibrosis or acute leukemia transformation. Chemotactic disorders of polynuclear may be the main etiological factor in these cases.

[Lymphadenopathy caused by hemophagocytic sinus histiocytosis (Destombes-Rosai-Dorfman syndrome). Immunologic and histopathologic study of a new case]. / Lymphadénopathie par histiocytose sinusale hémophagocytaire (syndrome de Destombes-Rosai-Dorfman). Etude immunologique et histo-pathologique d'un nouveau cas

The case reported is one of lymphadenopathy due to haemophagocytic sinusal histiocytosis which had progressed for more than 10 years before resulting in death at the age of 58 years. In this advanced form, all superficial and deep nodes were involved. In addition to histiocytic hyperplasia, with haemophagocytosis and lipid overload, there was plasmocytic hyperplasia, i.e. B-lymphocytes, with polyclonal hypergamma-globulinaemia and an increase in the levels of many antibodies, in particular against rubella and the Epstein-Barr virus. The aetiopathological significance is discussed, in particular the role of an infection due to the EB virus.