Acute promyelocytic leukemia: an experience on 95 greek patients treated in the all-trans-retinoic Acid era.

Acute promyelocytic leukemia (APL) is highly curable with the combination of all-transretinoic acid (ATRA) and anthracycline based chemotherapy, but the percentage of early deaths remains high. In the present study, we report the clinical, immunophenotypic, cytogenetic and molecular characteristics and outcome of APL patients diagnosed and treated in various Hospitals of Greece and Cyprus.We describe the data of ninety-five APL patients who were diagnosed during the last 15 years. Seven (7.4%) newly diagnosed APL patients died due to intracranial hemorrhage within 72 hours of presentation. All but two patients were induced with ATRA alone or ATRA plus chemotherapy. The early death rate was 14.9%. After induction all 80 evaluable patients achieved complete hematologic remission. The cumulative incidence of relapse was 18.3%. Eight of the ten relapsed patients were successfully salvaged, while both patients with molecularly resistant disease died during salvage treatment. Overall survival (OS) at 5 years was 78.4% and disease free survival (DFS) 73.6%. In multivariate analysis of OS age over 60 years, DIC at diagnosis and marginally major hemorrhage at presentation were identified as adverse prognostic factors. In the subgroup of patients with available data on FLT3 mutation status (49 out of 94), ITD positivity also remained as an independent prognostic factor in the final model of OS, together with major hemorrhage and marginally high Sanz score. We found a close correlation between the CD2 expression and the development of the differentiation syndrome (DS). In conclusion, the main problem in managing patients with APL is still the high early death rate.

Familial multiple endocrine neoplasia type I: the urologist is first on the scene.

OBJECTIVE: To study the urological manifestations of familial multiple endocrine neoplasia type 1 (MEN-1). PATIENTS AND METHODS: The study included 26 adults (median age 38.5 years, range 18-80) from two unrelated MEN-1 pedigrees. In 15 of the patients the diagnosis was confirmed by genetic analysis, while in the rest the diagnosis was based on clinical criteria combined with genealogy data. RESULTS: Urolithiasis associated with primary hyperparathyroidism was present in 65% of MEN-1 patients and in 77% of those who were symptomatic. In 68% of patients complications of urolithiasis (renal/ureteric colic, urinary tract infection) were the presenting clinical manifestations of MEN-1, whereas in 50% they constituted the only clinical manifestation of the syndrome. The mean time from the onset of symptoms of urolithiasis to the diagnosis of the polyendocrinopathy was 17.2 years. Initial failure to recognize the presence of MEN-1 in patients with primary hyperparathyroidism led to conservative parathyroid surgery, with subsequent relapse of the hyperparathyroidism, requiring re-operation. Serious renal morbidity included one case of pyonephrosis necessitating nephrectomy. While urolithiasis was a cardinal clinical manifestation of MEN-1, there was otherwise considerable phenotypic polymorphism, even among patients bearing the same MEN1 gene mutation. CONCLUSION: In patients with familial MEN-1 the complications of urolithiasis are the commonest presenting clinical manifestations and the cause of significant morbidity. In the presence of a family history of renal stones, appropriate investigations may lead to the timely diagnosis of this important, albeit rare, disorder.