International Journal of Hematology-Oncology and Stem Cell Research
Volume:3 Issue: 1, Jan 2009

Allogeneic hematopoietic cell transplantation (HSCT) 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 HSCT has proven to be a rather challenging task for hematologists. We analyzed the outcome of 30 FA patients (median age at HSCT was 9 years age range, 2-32 years) who underwent HSCT between 1992 and 2008 in Shariati Hospital Tehran, Iran.. Patients were transplanted from either an HLA-identical sibling or matched relative (n=29), or an HLA-partially matched relative(n=1).Four different conditioning regimens without radiation were used. Graft versus host disease (GVHD)prevention consisted of cyclosporine with methotrexate or cyclosporine alone. The median follow-up duration for survivors was 2.7 years (ranged 1 month to 12 years). The median survival time was 8.5 months. The 5-year overall survival was 43.6% (SE=10.0%). All surviving patients had normal blood counts with full donor engraftment.The median survival rate for patients who did or did not receive fludarabine in preparation for the allograft wasnot statistically significant (p-value=1.0). Our study demonstrates that none of the studied variables significantly affected the survival,including sex, age, radiation-free conditioning regimens, corticosteroids before transplant, pre-transplanttransfusions, acute GVHD and congenital abnormalities.The availability of better diagnostic tools to predict clinical course of FA, and modification of the conditioningregimen should improve survival and long-term consequences of therapy for patients in the future.

This study testing the effectiveness, toxicity and tolereability of new chemotherapeutic regimen (consist of Irinotecan 5-FU and Leucovorin) in locally advanced and metastatic Gastric Cancer.Patients and Patients with locally advanced gastric cancer (at least stage 3) or metastatic gastric cancer that no treated previously with chemotherapeutic agents entered onto this study. Treatment consist of 6-12 cycles (every 2 weeks) with Irinotecan (CPT-11) 140mg/m2 iv, Lecovorin 100mg/m2 iv, followed by 5-FU 400mg/m2 (in4hours) and then 5-FU 1200m2 (ci in 48h), all cycles given every 2 weeks and repeated at least for 6 cycles(6-12 cycles). Ten (10) patients were enrolled and eligible patients received this protocol therapy. The over all response rate was 60% and 10% complete response, and 50% with partial response and 10% stable disease. Median survival was 13 months and median event free survival was 8 months. The major toxicity was Neutropenia (grade 4) in 3 cases (30%), that two cases died in neutropenic sepsis feature. Grade 4 anemia was observed in one case that needs to transfusion therapy. Other mild side effect and toxicity of this protocol therapy were: Grade 1 neutropenia (10%), mild thrombocytopenia (10%),mild diarrhea (40%)mild nausea and vomiting (60%)and lethargia and mucositis were not seen. This protocol consist of Irinotecan iv bolus that followed with Leucovorin and 5-FU in 4h and then 5-FU in 48h ci is very active in patients with locally advanced and metastatic Gastric Cancer. Diarrhea and mucositis were less than other reports. Future trials in large series need to documented effectiveness and tolereability and toxicity of this new protocol.

Acute Myelogenous Leukemia (AML) is a clonal malignant disease of hematopoietic tissue. Theearly use of Hematopoietic Stem Cell Transplantation (HSCT) for AML was for patients with advanced stagesof disease, usually while in relapse, in second or subsequent remission, or with resistant disease.Patients and From March 1991 until February 2008, 356(65%) registered allogeneic transplantation and 192(35%) of registered autologous transplantation had comprehensive data available for analysis and were included in this study and all patients have followed through January 2008. In allogeneic and autologous groups, 263 (73.9%) and 160 (88.3%) patients were in first complete remission, respectively. The stem cells sources of transplantation in allogeneic recipients were Peripheral Blood (92.4%), Bone Marrow (7%), Bone Marrow combined Peripheral Blood (0.3%) and Cord Blood (0.3%). The source of stem cell transplantation for 157 (82.3%) autologous patients was Peripheral Blood, for 33(17.2%) patients was Bone marrow and there was one recipient (0.5%) with combined Peripheral Blood and Bone marrow. Totally, 279 (78.4%) allogeneic patients and 123 (64.1%) autologous patients were alive since the end of this study. Relapse was the most common cause of death in both groups. Five years Overall Survival (OS) and Disease Free Survival (DFS) in allogeneic patients were 70.6% and 62.3% (SE=3%) and in autologous patients were 53.6% and 46.8% (SE=5%, 4%). The median follow up time for this study was 1.5 years (19 months). According to this study and our experience, the acceptable treatment for Acute Myeloblastic Leukemia, especially in first complete remission is allogeneic Hematopoietic Stem Cell Transplantation.

In 1989, the first successful umbilical cord blood transplantations (UCBTs) was reported in a boy with fanconi''s anemia, using umbilical cord (UCB) of his HLA matched sister. Cord blood transplantation is a good substitute for bone marrow or peripheral blood transplantation especially for children and small body size adults. Between 1998 and 2007, 14 children(10 boys and 4 girls) with non-malignant(9 Beta-Thalassemia Major, 3 SCID,1 Hurler) and malignant(1 AML) diseases were given an allogeneic CB transplant from sibling (10 cases) or unrelated(4 cases). In the majority of cases, busulfan and cyclophosphamide were given in various dosage in conditioning regimen GVHD prophylaxis consisted mainly of (CsA) alone (in 9 cases), the combination of CsA and methotrexate (in 4 cases) and methotrexate alone (in 1 case). In thalassemic patients 89% are alive but 55% with disease. Graft failure occurred in 4 thalassemic and 1 AML patients. Only 2 cases experienced GVHD (1 acute GVHD grade2 and 1limited chronic GVHD). All three cases of SCID that transplanted very late after many infectious complications are dead. This study demonstrated that CBT is curative in some thalassemic patients but in future, we can improve results of CBT with use of ATG, Alemtuzumab, Thiotepa, G-CSF, double cord, TNC dose of 4×107/kg and elimination of methotrexate for GVHD prophylaxis in HLA- match sibling.

This paper used Generalized Gamma (GG) distribution to find the predictive factors of overallsurvival (OS) after haematopoietic stem cell transplant (HSCT) in acute myeloid leukemia patients. Discrimination among the exponential, Weibull, GG, log-logistic, and lognormal distributions was done using maximum likelihood and Akaike information criteria. The 5-year OS in 301 patients was 65% (95%CI: 60.7-69.3). Peak mortality hazard occurred at months6-7 after HSCT then, it was U Shape. The data was fitted by GG distribution better than other distributions.Univariate analysis using GG distribution showed a positive association between OS with dose of infused WBC (P=0.018), CD3 (p=0.001), no relapse (P<0.001), cGVHD (P<0.001), and platelet recovery (P<0.001).Multivariate analysis indicated that, OS has relationship with relapse (P<0.001), platelet recovery (P=0.004),disease status at transplant (P=0.036) and aGVHD (P=0.036). We showed that GG distribution can be a useful tool for recognizing prognostic factors of OS inAML patients.

Hematology- Oncology and Stem Cell Transplantation Research Center related to Tehran University of Medical Sciences located in Shariati Hospital. These center activities have started in 1991 in order to help needful patients and augment new data to reach new aspects of therapeutic trials. Also it is one of the greatest Stem Cell Transplantation centers in world and is the second center in the world based on the transplanted Thalassemia patients. Since 1991, 2426 first Hematopoietic Stem Cell Transplantation (HSCT) has been performed in patients with different diseases. Acute Myelogenous Leukemia (647 patients), Thalassemia Major (386 patients) and Acute Lymphoblastic Leukemia (335 patients) were the most common transplanted disorders, respectively. There were 1662 cases that have received allogeneic HSCT and 764 cases that have received autologous HSCT. Number of allogeneic ant autologous HSCT is increased during the time, but the allogeneic to autologous ratio remains constant. The first peripheral blood Hematopoietic Stem Cell Transplantation was performed in 1996 and since then, there was 1988 patients were done with this method.The donor types for 1662 allogeneic first HSCT were 1577 (94.9%) Human Leukocyte Antigen (HLA) matched-identical siblings, 44 (2.6%), HLA mismatched sibling/other relative, 16 (1%) syngeneic twins, 18 (1.1%) HLA matched other relative and 7 (0.4%) unrelated. The first cord blood Hematopoietic Stem Cell Transplantation was performed in 1998 and since then there was 16 patients that have obtained cord blood transplantations. Recently, new methods have been used like Donor Lymphocyte Infusion (DLI) and Cellular Therapy. There were 147 patients with Cellular Therapy for post MI, Cirrhosis, Thalassemia major, MultipleSclerosis, Head of Femour Necrosis and GvHD treatment.

Multiple myeloma represents a malignant proliferation of plasma cells derived from a single clone and it results in bone pain or fracture, renal failure, susceptibility to infections, anemia, and hypercalcemia. The hyperviscosity syndrome is rare. Cryoglobulins are immunoglubulins that precipitate in the cold temperature less than 37°C. Monoclonal cryoglobulins are associated with a significant hematologic disorder and are often asymptomatic. We reported the second case of multiple myeloma with gangrene of all four extremities.Case: The Patient was a 77 year-old farmer who was referred due to 2-weeks history of blue, cold and painful fingers spread to middle of forearms and forelegs which was accompanied by feet skin erosions, dark points on auricle and discoloration of nose tip. In physical examination, quadrigangrene associated with auricle and tip of nose ischemia was seen. Serum proteins electrophoresis demonstrated monoclonal gammopathy and serum was positive for cryoglobulin. Bone marrow study showed neoplastic plasma cells infiltration. The patient with cryoglobulinemia, based on multiple myeloma was treated.

Burkitt’s lymphoma is one of the most hematologic malignancies that involve the central nervous system. Our patient had a rare presenting symptom of lymphoma during pregnancy. It is reasonable to search for Burkitt’s lymphoma in every patient with abdominal mass and neurologic symptom. Involvement of cranial nerves especially abducens are among this neurologic complications but are rare as presenting symptoms of lymphoma.