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Objective Ankle arthropathy is a frequent and invalidating manifestation of hemophilia. Arthrodesis is the gold standard surgical procedure in end-stage disease, with many drawbacks in young patients. Recent literature has shown increase interest in regenerative procedures in hemophilic arthropathy, which may be desirable to delay or even avoid arthrodesis. The aim of this article is to present five cases of osteochondral lesions in ankle hemophilic arthropathy treated with a regenerative procedure: bone marrow–derived cells transplantation (BMDCT).Design We report five hemophilic patients (four cases with hemophilia type A; one case with hemophilia type B) who have undergone BMDCT treatment, synovectomy, and arthroscopic debridement, with the use of autologous platelet-rich fibrin, to treat osteochondral lesions in hemophilic ankle arthropathy. The patients, included within this retrospective study, were clinically and radiologically evaluated with serial follow-ups, using the American Orthopaedic Foot and Ankle Society (AOFAS) scores, radiographs, and magnetic resonance imaging (MRI).Results The mean preoperative AOFAS score was 35. After a mean follow-up of 2 years, the mean postoperative AOFAS score was 81, which included three patients returning back to sporting activities. The MRI Mocart score demonstrated signs of regeneration of chondral and bony tissue. No progression of joint degeneration was shown radiographically.Conclusion BMDCT is a promising regenerative treatment for osteochondral lesions in mild ankle hemophilic arthropathy, which may be useful to delay or even avoid ankle arthrodesis. Nevertheless, longer follow-ups and a larger case series are required.

All-trans retinoic acid (ATRA) is currently being used as remission induction treatment for acute promyelocytic leukemia (APL). Conventional chemotherapy is added both during and after ATRA treatment, in order to avoid the occurrence of hyperleukocytosis, and to improve the duration of complete remission. In this study we analysed the hematopoietic recovery of 18 consecutive APL patients after standard Idarubicin or Daunorubicin ± Cytosine-Arabinoside regimens. 9 of the patients were at the onset of the disease, (CHT group) while 9 had been pre-treated with ATRA 45 mg/sqm/day for at least 3 months (ATRA group). 500 PMN/mmc were reached after 20.8 day from the end of treatment in CHT group and after 12.0 days in ATRA group (p = 0.007). Platelets recovery was faster, even though not significantly, in ATRA group. Interestingly, PMN recovery in ATRA group was even shorter (p = 0.004) than that obtained in CHT group, after the first course of chemotherapy (treatment in CR vs treatment in CR). If these results are confirmed in a larger study, a protective effect of ATRA on normal residual hemopoiesis should be postulated.

All-trans retinoic acid (ATRA) represents a highly effective treatment for acute promyelocytic leukemia (M3-ANLL). This compound induces the leukemic promyelocytes to differentiate into morphologically and phenotypically mature myeloid cells. The mechanism of action of ATRA is far from fully understood. It has recently been reported that, along with its differentiation activity, ATRA causes apoptosis in the acute promyelocytic leukemia cell line HL-60. In this study we attempted to test whether ATRA is also able to induce apoptosis in fresh leukemic cells from M3-ANLL patients. Our results indicated that although morphological differentiation was detectable in 9/9 M3-ANLL samples after in vitro exposure to ATRA 1CT6 M., the percentage of apoptotic cells in the treated samples did not significantly differ from that obtained in controls (13.1% vs 9.4% respectively, after 8 days exposure). These data suggest that apoptosis does not seem to be the key mechanism by which ATRA exerts its action in M3-ANLL, at least at the blast cell level.

We retrospectively studied a consecutive series of 100 patients with acute leukemia and aspergillosis to evaluate the clinical findings and risk factors for colonization of the central nervous system (CNS) by Aspergillus species. The diagnosis of CNS aspergillosis was made in 14 patients on the basis of the following criteria: neurological signs of CNS involvement (13 of 14 patients); cerebral CT scan findings (9 of 12); microbiological findings (6 of 12); and histological findings at autopsy (11 of 11). The majority of patients had severe neurological complications (i,e., hemiparesis or seizures), due mainly to brain abscesses or mycetomas. Autopsies were performed on 11 of 14 patients and provided evidence that CNS localization was secondary to invasive aspergillosis; in each case, the most likely primary focus of infection was the lung. Although all patients had received oral antimycotic prophylaxis and had received timely empirical antifungal treatment, they all died within a median time of 5 days from the onset of neurological symptoms. Analysis of the characteristics of patients with invasive aspergillosis did not reveal any difference between those with CNS localization and those without eNS localization.