5-Aza-2'-deoxycytidine (Decitabine) induces trilineage response in unfavourable myelodysplastic syndromes.

The preliminary results of a disease-oriented phase I-II study aimed at evaluating the clinical activity of 5-aza-2'-deoxycytidine (Decitabine) in patients affected by advanced myelodysplastic syndromes (MDS) are reported. Two patients affected by refractory anemia with excess of blasts (RAEB) and eight with RAEB in transformation (RAEB-T) were treated with Decitabine at a daily dose of 45 mg/m2, divided into three 4 h infusions for 3 days (six patients) or as continuous infusion of 50 mg/m2 for 3 days (four patients). Treatment with Decitabine resulted in a significant increase in circulating neutrophils, platelets, and hemoglobin with respect to pretreatment values in over 50% of patients. These changes were accompanied by the improvement of the marrow myeloid relative differentiation index (median fivefold increase in the whole group of patients) and of the myeloid to erythroid cell ratio (median twofold increase) in most of the patients. In four out of ten patients a complete normalization of peripheral blood (PB) and bone marrow (BM) picture (complete hematologic response) was obtained. The evaluation of the percentage of CD34-positive BM cells showed a slow but progressive reduction of early leukemic progenitors in most of the patients. A transient slight BM hypoplasia was obtained in less than 50% of patients while a severe marrow aplasia was never observed in our group of MDS patients during treatment with Decitabine. Extra-hematological toxicity was very mild in all the patients. The preliminary results of our study indicate that Decitabine is able to induce trilineage hematological responses in advanced MDS patients along with a stable normalization of the PB and BM picture in some of the subjects. Decitabine appears an active agent in advanced MDS and this deserves careful investigation in this heterogeneous group of disorders.

A case of virus associated hemophagocytic syndrome and malignant histiocytosis: sometimes a difficult distinction.

The differential diagnosis among hemophagocytic syndromes and particularly between the virus-associated hemophagocytic syndrome (VAHS) and the malignant histiocytosis (HM) is quite easy in typical cases. Difficulties of interpretation may sometimes ensue when viral-bacteriologic work-ups indicative of VAHS are negative and/or morphologic histiocytic aberrations indicative of HM are missing. The authors present a case of hemophagocytic syndrome with the latter characteristics and discuss the differential diagnosis.

[Primary hyperparathyroidism and peptic ulcer]. / Iperparatiroidismo primitivo ed ulcera peptica.

The prevalence of peptic ulcer disease was retrospectively analysed in 35 patients affected by primary hyperparathyroidism consecutively observed from 1977 through 1987. Eight of the examined patients (22.8%) had peptic ulcer (7 duodenal and 1 gastric ulcer), that in five cases (14%) represented the first clinical manifestation of the endocrine disease. A Zollinger-Ellison syndrome was demonstrated in three cases (8.5% of the total series, 37% of the patients with ulcer). There was no difference in calcium, gastrin and parathormone serum level between patients with and without ulcer, excluding patients with Zollinger-Ellison syndrome. These data confirm the high prevalence of peptic ulcer disease in hyperparathyroidism, but the mechanism causing this association remains to be elucidated.

[Necrotizing lymphadenitis without granulocytic infiltration or Kikuchi disease. Description of 5 cases]. / Linfoadenite necrotizzante senza infiltrazione granulocitaria o malattia di Kikuchi. Descrizione di cinque casi.

The authors describe five cases of necrotizing lymphadenitis without granulocyte infiltration or Kikuchi's disease. Of the patients, all young, four were females and one of male sex. In one case serological positivity was found for the anti Toxoplasma antibodies. Histologically they are noted in the lymph node infiltration areas constituted by histiocytes, immunoblasts, lymphocytes and plasmacytoid cells with phenomena of variable degree necrosis. Granulocytes were absent in all. Cytochemistry and immunohistochemistry studies with specific antiserums for the k and lambda chains, the S100 protein and the MB1, MB2 and MT1 antigens, the Lisozyme and the alpha 1 antichymotrypsin, show a prevalent histiocyte population, associated with a modest polyclonal B component and with a T population of variable entity. The plasmacytoid component did not appear reactive to the same antibodies.

[Variability in immunologic aspects of Whipple's disease. Is a unitary interpretation possible?]. / Variabilità degli aspetti immunologici nella malattia di Whipple. E possibile una interpretazione unitaria?

A case of Whipple's disease with histological and ultrastructural studies, characterized by unusual bacteriological and immunologic findings, is reported. Alpha hemolytic Streptococcus and Candida tropicalis were isolated from the culture of the intestinal biopsy specimens. The immunological function study showed a global defect both of humoral and cellular immunity. On the basis of the literature review, the Authors debate a unitary interpretation of the various immunological dysfunctions reported in this disease.

[Aplastic anemia and myelodysplasia: a not-always-easy distinction]. / Anemia aplastica e mielodisplasia: una distinzione non sempre agevole.

The differential diagnosis between Myelodysplasia and Aplastic anaemia may be sometimes difficult, because clinical and morphological features may appear similar. Two cases, a Myelodysplastic syndrome with hypocellular and an Aplastic anaemia with hypercellular BM aspirates, are described in this report. Reciprocal connections between these two pathological entities, some biological aspects and the value of BM biopsy are also discussed.