International Journal of Hematology-Oncology and Stem Cell Research
Volume:6 Issue: 3, Jul 2012

Despite all improvement in the treatment of acute myeloid leukemia (AML) patients, the management in relapsed or refractory disease is still controversial. The use of multiple chemotherapeutic agents will induce more toxicity and morbidity in patients. Fludarabin- containing therapy (FLAG and FLANG) mostly has been used in the treatment of relapsed or refractory AML patients. In this retrospective study, we evaluated the response of treatment in 40 adult leukemia patients treated with these regimens from October 2007 to December 2011 in our center. They took FLAG (fludarabine, cytarabine, GCSF) and FLANG (fludarabine, cytarabine, mitoxantrone) according to the approved protocol. They were taken packed cells or platelets as needed and antibiotics in cases of established or highly suspicious infections. After these therapies, 12 patients with suitable donor made ready for allogeneic hematopoietic stem cell transplantation (HSCT) and one patient without donor underwent autologous HSCT. The median age of patients was 29.5 (range: 16- 50) years old. The male to female ratio was 30:10. The patients were diagnosed with ALL (15, 37.5%) and AML (25, 62.5%). The most common subtype of leukemia was ALL- L2 and AML- M4. The most common disease treated was AML-M4. Two patients had secondary leukemia (breast cancer and MDS). Except two patients which treated with FLAG regimen, others received FLANG. Most patients were primary refractory (18, 45%), first relapse (17, 42.5%) or second relapse (5, 12.5%) before this treatment. Thirteen patients underwent hematopoietic stem cell transplantation after FLANG. Four patients received FLANG as relapse treatment after transplantation. Pulmonary aspergillus infection occurred in 13 patients and aspergillus sinusitis in two after FLANG/ FLAG treatment. White blood cells and platelet recovery observed in 30 patients with a median time of 20 days after treatment. Treatment resulted in complete response (n=19), partial response (n=1) and no response (n=11) in patients. Early death (before 60 days) after treatment occurred in 9 (22.5%) patients. The most common causes of death were primary disease (relapse) (24, 60%) and infection (5, 12.5%). The one- year overall survival was 21.4% (SE: 7.1%). Among the survivors, only 5 patients received transplantation and 5 patients are alive without transplantation. The study shows that refractory and relapsed leukemia patients can achieve complete remission with FLAG/ FLANG treatment. The side effects include serious fungal infections and sepsis. Moreover, high mortality during these treatments was observed.

Despite high remission rates in acute lymphoblastic leukemia (ALL) patients after induction chemotherapies, post- remission therapies needed to avoiding relapse. Autologous hematopoietic stem cell transplantation (HSCT) role in the treatment of ALL is still controversial. In this retrospective study, we assessed the outcome of auto- HSCT in the treatment of ALL patients treated in this center Patients and From March 1991 to December 2005, 25 ALL patients with no suitable donors underwent auto-HSCT. All patients received Endoxan, Cytarabin and Etoposide according to the center- approved protocol for conditioning regimen. The sources of graft were peripheral blood and bone marrow. The patients hospitalized in same special rooms and circumstances. The Kaplan-Meier method was used for the data analysis. The median age of patients was 18 years old (range: 8-54). The majority of patients were male. The mean number of WBC count at diagnosis was 48.5×103/µl. Seventy- two percent of patients received autologous HSCT in CR1. Eighty percent of ALL subtypes were B- lineage. Primary central nervous system involvement at diagnosis time was observed in 16%. The median number of harvested nucleated cells: 4.16×108/kg, MNC: 3.69×108/kg, CD3: 1.52×108/kg and CD34+ cells were 0.07×108/kg of recipient weight. The median time of neutrophil and platelet recovery was 12 (range: 9-37) and 17 (range: 10-74) days, respectively. The median follow-up period for survivors was 12 months (range: 4-110 months). Relapse occurred in 17(68%) of patients. Relapse was the only cause of death in patients. The one-year overall survival (OS) and disease-free survival (DFS) were 46% (SE: 10.2%) and 38% (SE: 9.9%), respectively. Age at transplantation and WBC count at diagnosis time had no significant effect on DFS and OS. Source of stem cells had no significant effect on survival outcome too. Transplantation in first complete remission had the best survival outcome (p =0.01) The role of autologous HSCT in ALL patients who do not have suitable donors is still inferior to chemotherapy alone. Regarding poor results of the current study, further studies on the role of auto- HSCT in specific subtypes of ALL patients is suggested.

Parenteral nutrition (PN) therapy remains an essential part of patients’ care during HSCT. There are many studies that focused on nutrition therapy in this population study, but there is not any consensus statement about nutrition therapy in patients who are hospitalized for bone marrow transplantation. The aim of the present study was to assess PN status and related complications in the patients during the course of HSCT in the HSCT wards of a referral teaching hospital. This study was conducted in the three HSCT wards of Shariati Hospital, Tehran, Iran. During a prospective study, data related to PN therapy were collected by clinical pharmacist during six-month period. PN therapy information including PN initiation, PN content, PN duration, and total daily calories intake in comparison with standard calories intake was recorded for each patient. An internal protocol for PN therapy was designed based on reliable guideline. The data related to patients’ nutrition therapy were compared with this protocol. The nutrition therapy was considered appropriate if it was compatible with the protocol regarding time of PN initiation, PN content, rout of PN administration, PN duration, and total kilocalories received. The average of duration of hospital stay and PN therapy were 27.17± 9.16 and 15 ± 6.1 days respectively.One hundred and fifty-seven of medication errors were detected during the study period in the patients. Overall rate of PN therapy errors was 2.3 numbers per patient during hospitalization. Errors in the rate of errors in the content of PN administration (100%) and incorrect total daily calories intake (55.9%), incorrect PN initiation (54%) and incorrect PN duration (25.6%) were the most common types of PN therapy. Our result showed that inappropriate PN therapy occurred commonly in the patients undergoing HSCT. Improvement in knowledge and attention of health-care workers about nutrition support for preventing of malnutrition in aspect of nutrition therapy.

The cord blood banks and cord blood transplantation community are on the constant lookout for a single objective dependable test that will indicate the functional capabilities of the cord blood units. This should ideally reflect engraftment, proliferation and differentiation capabilities. Presently in vitro Colony forming unit (CFU) assay is being performed by most cord blood centers to determine the functional efficacy of cord blood units. However, technical challenges associated with this assay have made it difficult to standardize the methodology among testing laboratories. A subjective test is associated with variability and non-uniform reporting. The aim of this study was to evaluate the usefulness of the newly introduced HALO® assay by Hemogenix® by comparing it with the information provided by the traditional colony forming assay (CFU). Repeatability and objectivity were also studied. Sixteen Cord Blood units processed in the repository were tested by both, the traditional CFU assay and the HALO® assay. Our study shows that the CFU assay provides information relating to proliferation and differentiation potential of CBUs while the HALO® assay gives quantitative output with reference to proliferative capability of CBUs. While both the assays provide valuable information on the functional efficacy of cord blood units from different stand points, one test perhaps cannot be substituted for the other. It is good to have both the tests available to bring in some additional information on its capabilities, which is much better than providing only one value with high subjectivity component.

Acute lymphoblastic leukemia (ALL) is the most common pediatric malignancy. The risk of thrombophilia increases in patients with ALL during chemotherapy. The present study aimed to investigate the frequency of factor V Leiden (FVL) mutation in children with acute lymphoblastic leukemia and its possible association with ALL.Patients and We studied 92 patients with ALL and 249 healthy individuals from Kermanshah Province of Iran. Detection of FVL mutation was performed by PCR-RFLP using restriction enzymes of Mnl I. The frequency of FVL G1691A polymorphism was 7.8% in patients compared to 3.2% in controls (p=0.052). There was a trend towards increased risk of ALL in the presence of FVL mutation [OR=2.54, 95% CI 0.9-7.2, p=0.08]. Our results indicated that the frequency of both thrombophilic mutation of FVL was higher in ALL patients from Kermanshah province compared to healthy individuals and FVL mutation tended to be associated with the increased risk of ALL. Further studies needed to evaluate the association between FVL mutation and the occurrence of thromboembolism in ALL patients.

Recurrent respiratory papillomatosis (RRP) is characterized by the recurrence of benign tumors (papillomata) in the aero digestive tract caused by Human Papilloma Virus. The burden for the patient and the society is non negligible, due to the high frequency of repetitive surgeries. The disease follows a bimodal age distribution. Usually the very first manifestation is hoarseness or voice changes but if negligee it can cause airway obstruction resulting in respiratorystridor or acute respiratory distress The treatment is challenging, includes surgery (mostly CO2 laser) and adjuvant medical therapy is almost always required (Cidofovir, interferon a, and others). The risk of malignant transformation is not negligent. Advances in immunology will lead us to understand the biology of HPV and will permit successful therapies. Prophylactic HPV vaccines are a promising area of research concerning RRP.

While thalassemia major (TM) used to be a prevalent genetic disease in the past, however, Phenylketonuria (PKU) is quite rare in spite of consanquiness marriage rate of about 40% in the region. Preventive efforts for TM started >20 years ago but neonatal screening for PKU started since 2007. This is the first report of co-existence of thalassemia and PKU in Middle East and in consideration of the prevalence of each genes, this chance association is a very unusual event. We report a case of having PKU and TM.

A 25 y/o male was admitted with iron deficiency anemia and nystagmus. Anti-TTG (IgA) and Anti-endomyosial (IgA) were high and pathologic findings in duodenal biopsy were suggestive of celiac disease. The diagnosis of celiac disease associated with nystagmus was made.