Long-term results of non-fludarabine versus fludarabine-based stem cell transplantation without total body irradiation in Fanconi anemia patients

Hematopoietic cell transplantation [HCT] is the only therapeutic modality capable of correcting the hematologic manifestations of Fanconi anemia [FA]. The development of well-tolerated immunosuppressive conditioning regimens for FA patients undergoing HCT has proven to be a challenging task for hematologists. Retrospective, patients referred to the hematology, oncology and stem cell transplantation research center. We analyzed the outcome of 53 FA patients who had undergone HCT between 1992 and 2010. The median age at transplantation was 9 years. Patients received transplants from an HLA-identiccal sibling [n=39] or matched relative [n=9] and one-antigen locus mismatched other relative/sibling [n=5]. All of the patients underwent transplantation with fludarabine and non-fludarabine-based conditioning regimens. No radiation therapy was given. The median follow-up period for survivors was 13.5 months [range, 3 months-13.5 years]. The 3-year overall survival [OS] was 60.6%. The 3-year OS for patients who did or did not receive fludarabine-based preparative regimens for the allograft was 36.4%, and 70%, respectively. However, there were no statistically significant differences in OS rates between these two groups [P=.112]. Graft failure occurred in 4 patients [7.5%]. All of these 4 patients had received fludarabine-based conditioning regimens. The incidence of acute GVHD after fludarabine-based regimens was 45% versus 79% in non-fludarabine-based regimens [P=.03]. Despite the high incidence of acute GVHD [78.6%] in the non-fludarabine group, which ressulted in the death of some patients, the OS rate was significantly better than in fludarabine recipients. Therefore, in spite of the fact that recent studies advocate the fludarabine-based conditioning regimens, we propose to conduct a multicenter, prospective study to evaluate the outcomes of regimens employed in FA patients

Treatment of relapsed acute promyelocytic leukemia by arsenic trioxide in Iran

Although standard first line treatment of acute promyelocytic leukemia is All trans retinoic acid [ATRA] and chemotherapy, some patients relapse and need a second line of treatment. Relapsed cases of promyelocytic leukemia can be salvaged with arsenic trioxide. Between May 1999 and Jan. 2010, we treated 31 relapsed cases of promyelocytic leukemia with arsenic trioxide. These cases relapsed after previous treatment with ATRA and chemotherapy. We applied arsenic trioxide as 0.15 mg/kg iv infusion until complete remission. After achieving complete remission patients received 2-4 consolidation therapy in the same schedule as remission induction. The median age of patients was 27 years. Complete remission rate was 77.4%. We observed four mortalities during remission induction. With a median follow up of 32 months, ten more relapses occurred. Two year disease-free survival and overall survival for the entire cohort was 54.6% and 81.1%, respectively. Our result is the same as other studies. Thus, we suggest that arsenic trioxide can be used as salvage therapy in patients who relapsed. Despite a good complete remission rate, the relapse rate during the first two years of treatment is high and hematopoietic stem cell transplantation should be considered after achieving complete remission

Hematopoietic stem cell transplantation in Iran: 1991 to 2008

Since 1991, 2042 first hematopoietic stem cell transplants [HSCT] have been performed at the Hematology-Oncology and Stem Cell Transplantation Research Center at Tehran University of Medical Sciences. Acute myelogenous leukemia [548 patients], thalassemia major [335 patients] and acute lymphoblastic leukemia [275 patients] have been the most common transplanted disorders. There were 1418 cases that received allogeneic HSCT and 624 cases that have received autologous HSCT. The numbers of allogeneic and autologous HSCT have increased, but the allogeneic to autologous ratio has remained constant. The first peripheral blood hematopoietic stem cell transplantation was performed in 1996; since then, 1671 have been done. The donor types for 1418 allogeneic first HSCT were 1367 [96.4%] human leukocyte antigen [HLA] matched-identical siblings, 29 [2%] HLA-mismatched sibling/other relative, 13 [0.9%] syngeneic twins, 5 [0.4%] HLA-matched other relatives and 4 [0.3%] unrelated. The first cord blood hematopoietic stem cell transplantation was performed in 1998 and since then there have been 14 patients that have obtained cord blood transplantations. Recently, new methods have been used like donor lymphocyte infusion [DLI] and cellular therapy. There were 111 patients with cellular therapy for post-myocardial infarction, cirrhosis, thalassemia major, multiple sclerosis, head of femur necrosis and renal cell carcinoma

[ value of Tc-99m MIBI scintigraphy in active diseases and remission phase of multiple myeloma]

Background: [99m]Tc methoxy isobutyl isonitrile [[99m]Tc MIBI] has been proposed as a tumor-seeking agent in malignant disease. The goal of this study is to evaluate the frequency distribution of the different patterns, intensity and extension of abnormal uptake identified in MIBI scan in relation with various clinical status of the patients diagnosed as multiple myeloma

Methods: forty-three patients entered the study, including six patients with no prior treatment, 22 patients who received autologous bone marrow graft, and 15 patients with history of chemotherapy and radiotherapy. Plasma protein electrophoresis for monoclonal antibody, bone marrow biopsy, and urine analysis for Bence-Jones protein has been carried out and standard criteria were used for diagnosis of active disease and remission phase for each patients. The extension of the lesions [E-score] on scintigraphy were categorized into E0-E3 by three nuclear physicians who were blinded to the patient's clinical condition. I-score was also obtained with comparing the intensity of the lesions with intensity of myocardial uptake and classified as I0-I3

Results: the sensitivity, specificity, positive predictive value and negative predictive value of [99m]Tc MIBI scan for determining active lesions and relapsed cases were 69%, 100%, 100%, and 60%, respectively. Nineteen patients were initially thought to be in remission phase, but scintigraphy was abnormal in 5 cases who were diagnosed as active myeloma later in the course of the study. There was a significant correlation between clinical status, and pattern, intensity, and extension of the abnormal uptake of [[99m]Tc-MIBI. Also a significant correlation between intensity and extension of the abnormal tracer uptake with serum monoclonal component and urine Bence-jones protein was noted, however no correlation between blood hemoglobulin and degree of extension in scintigraphy was seen

Conclusion: our study suggests the patterns, extension and intensity of [99m]Tc MIBI uptake, in addition to the hematological findings are associated with disease activity and clinical status of the patients. Hence, in addition to the standard protocol, [99m]Tc-MIBI has a very high accuracy for detection of active myeloma disease and also can detect the group of patients who might benefit from treatment