International Journal of Hematology-Oncology and Stem Cell Research
Volume:1 Issue: 2, Apr 2004

From 1996 to 2002, fifty three patients with major beta-thalassemia received allogeneic peripheral blood stem cell trans plantation (PBSCT). Median age was 6 years. Twenty two were class I, 17 class II and 14 class III. All of the donors were HLA-identical. Conditioning regimen for class I and II patients consisted of Cyclophosphamide (CY) 50 mg/kg/day for 4 days + Busulfan (Bu) 3.5 mg/kg for 4 days, while class III patients received 4 mg/kg/day Busulfan for 4 days and 40mg/kg/day Cyclophosphomide for 4 days. G-CSF (Neopogen) 5µ/kg IV was given to donors. Graft Versus Host Disease (GVHD) prophylaxis regimen consisted of Cyclosporin-A (CsA) 3 mg/kg/day plus Methotrexate (MTX) 10 mg/m2 on day+1 and 6 mg/m2 on days +3 and +6. The median time for neutrophil and platelet engraftment was day +16 and day +23 post transplantation, respectively. Chronic GVHD (cGVHD) was observed in 30 patients (56%). Ten patients (18.8%) died. Forty patients are well and transfusion independent. Median time of follow-up was 23 months. Recurrences have been seen at 3 pts, one patient 21 months, the other one 6 months and the last one 8 months after transplantation, who received Donor Lymphocyte infusion (DLI). Event free survival was 72% and overall survival was 80%. In conclusion, we suggest that PBSCT can be considered a safe and effective treatment for children with Beta-thalassemia major and cGVHD is tolerable and manageable in these patients.

: Myeloablative-allogeneic stem cell transplantation is a common way of treating various malignant and nonma-lignant diseases; but, it is associated with hazardous immediate and late complications. The majority of patients are not good candidates for high dose therapy because of old age, medical co-morbidities or previous heavy treatments. The donor stem cells can engraft in the recipient and induce mixed chimerism when we use a less intensive, but sufficiently immunosup-pressive, conditioning regimen, known as mini-transplantation or non-Myeloablative allogeneic Stem Cell Transplantation (NM-allo-SCT). The conditioning regimens were the combination of Fludarabine and Cyclophosphamide or Busulfan and ATG. Prophylaxis against graft versus host disease (GVHD) included Cyclosporine A (CSA) +/- Methotrexate. A multiplex-PCR using short tandem repeats (VNTR) was used for chimerism analysis. We report the results of NM-allo-SCT from the HLA-identical siblings in 20 patients with AML (N=7), CML (N=6), NHL (N=2), MDS (N=2), ALL (N=1) and Fanconi anemia (N=2). Fourteen males and 6 females with median age of 43 years (range 8-55) underwent NM-allo-SCT and were followed up 4-870 days (median 420 days). Typical side effect of conventional HSCT, such as severe mucositis, vomiting and VOD were absent. Most of the patients did not become se verely pancytopenic and had relatively short hospitalization. Hematological recovery was rapid, a median of 8.5 days. Acute GVHD (grade ≥II) and extensive chronic GVHD was observed in three patients. Most of the patients initially had mixed-chimerism, progressing to full-donor-chimerism in 11 patients, after the interruption of the CSA therapy, and, in one patient, after DLI. Nine patients died, six from relapse or disease progression and three from transplantation-related complications (GVHD, infection or secondary malignancy). 14 month overall survival and disease free survival of 55% and 50%, respec tively, was observed. Our results confirm that NM-allo-SCT is safe and minimally toxic and is a potential new approach for a safer treatment of a large variety of hematologic diseases, especially in patients with AML and CML in remission.

History and Goals: Now a days, the main purpose of treatment in patients with Acute Myeloid Leukemia (AML), is achiev ing a "definite cure", and this goal is impossible unless stem cell transplantation (bone marrow transplantation) or the ad ministration of high-dose Cytarabine is performed.As these therapies are very expensive and money consuming, and due to restricted application of these procedures in Iran, most hematological clinics in Iran, prefer to have a classical approach in such instances. These approaches consist of 4 cycle performing a 4 months period of hospitalization.In patients who can not pay the cost of treatment and have a low socioeconomic and cultural status, maintaining therapy is very difficult and usually unsuccessful.Out patient consolidation therapy is an alternative to cope with such problems. As a clinical trial, all cases of AML, admitted to Taleghani Hospital (Kermanshah-Iran) from De cember, 1998, after achieving complete remission and receiving first consolidation therapy were selected to be evaluated. For patients who could not accept long time hospitalization because of their socioeconomic, financial status, consolidation therapy was arranged for them as outpatients, for 6 continuous cycles, every 3-4 weeks. Other patients received 2 runs of classic consolidation therapy in hospital. Till October 2002, of the 57 patients admitted with AML diagnosis, 7 cases were excluded because of advanced age (age>65) and functional weakness and 3 patients died as a result of early complications. Of the 47 remaining patients, 39 cases achieved complete remission with induction therapy. 23 of the treated patients received out-patient consolidation therapy and 16 cases underwent classical therapy.During the follow-up period which lasted between 7 to 47 months, in the classical therapy group, 8 cases of relapse occurred leading to the death of 5 patients, whereas in the out-patient group, 12 relapses occurred in which 7 persons died. From an overall survival (OS) and disease free survival (DFS) point of view, no notable and meaningful difference was seen. But, complications of therapy necessitated blood product administration and increased amount of hospitalization, which was significantly more frequent in patients assigned to classical therapy compared to the out-patient group. According to the results of this study and a few identical studies performed previously in Imam Khomeini and Dr Shariati Hospitals (Tehran-Iran), although, out-patient consolidation therapy takes more time, but, because of shortened hospitalization time, ease of performance and tolerance by patients, this therapy can be applied to all patients with a low so-cioeconomic status. Moreover, from an ethical point of view, physicians can be assured that there is no difference in relapse and mortality rate compared with the classical approaches.Obviously, this therapeutic approach can be generalized and extended to other regions of Iran, and in underdeveloped coun tries with the same limitations and problems.

Acute Promyelocytic Leukemia is a sub-type of acute myelogenous leukemia that occurs in about 10-15% of patients with AML. Approximately 20%-30% of these patients, who are treated with the current standard All Trans Retinoic Acid (ATRA) and Anthracyclin-based chemotherapy regimen, suffer relapse in less than a year. Arsenic trioxide (ATO) as a single agent can induce complete remission even in refractory and relapsed patients with few adverse effects. The investiga tors efforts regarding elucidation of the mechanisms of action underlying these clinical responses has shown that Arsenic apparently affects numerous intracellular signal transduction pathways and causes many alterations in cellular function, among which the most prominent ones are the induction of differentiation & apoptosis with low & high doses of arsenic, respectively. In vivo apoptosis on these patients has not been evaluated yet and despite previous In vitro studies, which mostly reveal Fas/Apo1 is not expressed during ATO treatment, its in vivo expression has not been evaluated yet. Materials & methodes: In order to study the apoptotic pattern in leukemic cells of APL patients, we conducted a single-laser, triple-color flowcytometric experiment, to detect leukemic apoptotic cells in a heterogeneous population of bone mar row samples with the Annexin V & 7AAD technique. The Fas expression was also evaluated in promyelocyte population cells in a dual color panel.Results & A substantial Apoptosis was selectively detected in Promyelocytic cells during the early and middle stages of treatment and the concurrent Fas expression indicates its involvent in Apoptosis induced by Arsenic Trioxide.

Vertebra plana is a radiological diagnosis that indicates complete compression of the vertebral body.(1,2) This condition was reported in Langerhans cell histiocytosis including eosino-philic granuloma,(3, 12) Ewing''s sarcoma,(2,13,14) tuberculosis,(15,16) vertebral osteomyelitis,(17) aseptic necrosis,(18) aneurysmal bone cyst,(19) osteosarcoma,(20) neuroblastoma,(21) acute leu-kemia,(22) rhabdomyosarcoma(23) and some other diseases.(25-30)There have been rare reports of vertebra plana due to malignant lymphoma.(23,24) Herein, we report a case of a 13 year-old boy with vertebra plana due to malignant lymphoma and describe his clinical manifestations, histological features, diagnosis, managment and clinical course