Combination of minimal residual disease on day 15 and copy number alterations results in BCR-ABL1-negative pediatric B-ALL: A powerful tool for prediction of induction failure.

The current genomic abnormalities provide prognostic value in pediatric Acute Lymphoblastic Leukemia (ALL). Furthermore, Copy Number Alteration (CNA) has recently been used to improve the genetic risk stratification of patients. This study aimed to evaluate CNA profiles in BCR-ABL1-negative pediatric B-ALL patients and correlate the data with Minimal Residual Disease (MRD) results after induction therapy. We examined 82 bone marrow samples from pediatric BCR-ABL1-negative B-ALL using the MLPA method for the most common CNAs, including IKZF1, CDKN2A/B, PAX5, RB1, BTG1, ETV6, EBF1, JAK2, and PAR1 region. Subsequently, patients were followed-up by multiparameter Flow Cytometry for MRD (MFC-MRD) assessment on days 15 and 33 after induction. Data showed that 58.5 % of patients carried at least one gene deletion, whereas 41.7 % of them carried more than one gene deletion simultaneously. The most frequent gene deletions were CDKN2A/B, ETV6, and IKZF1 (30.5 %, 14.6 %, and 14.6 %, respectively), while the PAR1 region showed predominantly duplication (30.5 %). CDKN2A/B and IKZF1 were related to positive MRD results on day 15 (p = 0.003 and p = 0.007, respectively). The simultaneous presence of more than one deletion was significantly associated with high induction failure (p = 0.001). Also, according to the CNA profile criteria, the CNA with poor risk (CNA-PR) profile was statistically associated with older age and positive MRD results on day 15 (p = 0.014 and p = 0.013, respectively). According to our results, the combined use of CNAs with MRD results on day 15 can predict induction failure and be helpful in ameliorating B-ALL risk stratification and treatment approaches.

Can N-acetylcysteine reduce red blood cell transfusion burden in patients with transfusion-dependent ß-thalassemia?

Patients with beta-thalassemia major require lifelong and frequent red blood cell transfusions for survival, impacting their quality of life and life expectancy. This treatment approach poses risks of organ damage, iron overload, and increased transfusion-transmitted diseases. N-acetylcysteine (NAC) has been studied for its potential antioxidant effects on hemoglobin stability, aiming to reduce the burden of red blood cell transfusions. To explore this possibility further, we conducted a quasi-experimental study involving 35 individuals with thalassemia major over six months All subjects were already receiving iron chelators and blood transfusions. They were given a daily oral dose of 10 mg/kg NAC for three months. After three months of treatment with NAC, the serum levels of ferritin and liver enzymes (SGOT and SGPT) did not show significant changes (p = 0.35, p = 0.352, and p = 0.686, respectively). However, the red blood cell transfusion burden was significantly reduced in all patients after NAC therapy (p = 0.029), with no corresponding decrease in serum hemoglobin levels (p = 0.931), indicating maintained hemoglobin concentration despite reduced transfusion volume. The study indicates that NAC can effectively decrease the burden of red blood cell transfusions without significant toxicity in these patients. This finding suggests the potential for NAC as a cost-effective and manageable treatment option for these patients. A larger clinical trial with more robust statistical methods could further confirm these results and pave the way for using NAC as a valuable therapeutic agent for managing beta-thalassemia major patients.

Corrigendum to "Bilateral Botryoid Wilms tumor presented with acute renal failure" [Radiology Case Reports 16 (2021) 166-170].

[This corrects the article DOI: 10.1016/j.radcr.2020.10.055.].

Erratum to "Bilateral Botryoid Wilms Tumor presented with acute renal failure" [Radiology Case Reports 16 (2021) 166-170].

[This corrects the article DOI: 10.1016/j.radcr.2020.10.055.].

A Clinical Decision Support System for Increasing Compliance with Protocols in Chemotherapy of Children with Acute Lymphoblastic Leukemia.

Objective: In this survey, a protocol-based Chemotherapy Prescription Decision Support System (CPDSS) was designed and evaluated to reduce medication errors in the chemotherapy process of children with ALL. Methods: The CPDSS algorithm was extracted by the software development team based on the protocol used by doctors to treat children with ALL. The ASP.Net MVC and SQL Server 2016 programming languages were used to develop the system. A 3-step evaluation (technical, retrospective, and user satisfaction) was performed on CPDSS designed at 2 children's hospitals in Tehran. The data were analyzed using descriptive statistics. At the technical evaluation step, users provided recommendations included in the system. Results: In the retrospective CPDSS evaluation step, 1281 prescribed doses of the drugs related to 30 patients were entered into the system. CPDSS detected 735 cases of protocol deviations and 57 (95%, CI = 1.25-2.55) errors in prescribed chemotherapy for children with ALL. In the user satisfaction evaluation, the users approved two dimensions of the user interface and functionality of the system. Conclusions: With the provision of alerts, the CPDSS can help increase compliance with chemotherapy protocols and decrease the chemotherapy prescribing errors that can improve patient safety.

Outcome of Patients Treated with Hematopoietic Stem Cell Transplantation: Results from A Single Center.

OBJECTIVE: Hematopoietic stem cell transplantation (HSCT) is known as one of the most advanced and modern treatments in the world for various diseases which do not respond well to other therapies. Evaluating outcomes of these patients, especially in newly developed centers, can crucially help in developing and improving the quality of these centers. METHODS: In a retrospective analytical cohort study, we statistically analyzed all patients treated with HSCT in the Bone Marrow Transplant Unit of the Ali-Asghar Pediatric Hospital affiliated to Iran University of Medical Sciences. The demographic information as well as all information concerning each patient's transplant process was extracted and statistically analyzed using SPSS Version 23. RESULTS: The mean neutrophilic and platelet engraftment days were, respectively, 16 (range = 12-21) and 22 (range = 15-34) days after HSCT, while the neutrophilic engraftment occurred significantly earlier in allogeneic transplants compared to the autologous ones (P = 0.020). The total event-free survival (EFS) rate of the patients based on the median follow-up of 12 months was 11.50% ± 53.60%. Based on the total follow-up period, the estimated total EFS rate of the patients was calculated as 35.20% ± 13.50%. The estimated EFS rate was found to be better in patients who had undergone allogeneic transplantation than those who received an autologous transplant (P = 0.780). CONCLUSIONS: The HSCT results at our center are comparable to those at other centers in Iran. We argue that the facility can provide adequate therapy to patients requiring HSCT, on the proviso that some organizational limitations are addressed.

Bilateral Botryoid Wilms Tumor presented with acute renal failure.

Botryoid Wilms tumors are intrapelvic polypoid masses that are uncommon feature of Wilms tumor. The authors reported a rare case of bilateral botryoid Wilms tumor in a 19-month-old boy who admitted to hospital with acute renal failure secondary to hydronephrosis caused by bilateral botryoid Wilms tumor with ureteral extension. The patient was successfully treated with chemotherapy and bilateral nephron sparing surgery in 2 separate operations. During 15 months follow-up, no evidence of recurrence or metastasis was found.

New translocation in acute myeloid leukemia M4 eos.

The most common childhood malignancy is acute leukemia. Approximately 15- 20% of it, is Acute myeloid leukemia (AML). The general symptoms of this malignancy include fatigue, weakness, fever, paleness and bleeding disorders. There are two methods of classifying for AML: The French-American-British (FAB) and the World Health Organization (WHO) classification.The M4 eos subtype, also called myelomonocytic leukemia, is one subtype of AML with eosinophilia. The most common cytogenetic variations in this leukemia include inv (16) (p13q22) or the variant t (16; 16) (p13; q22). In this report, we present the first AML-M4 eos case with a new translocation that has not yet been reported.

Study of baseline echocardiography and treatment endpoint in patients with acute lymphoblastic leukemia.

INTRODUCTION: Acute lymphoblastic leukemia (ALL) is the most common malignancy in children. Anthracyclines are among the most common and effective drugs for the treatment of children ALL. However, long-term consumption and higher doses of these drugs may lead to toxic effects on the heart of children. For this purpose, in the present study, the baseline and posttreatment echocardiography status was evaluated in children with ALL during 7 years. MATERIALS AND METHODS: This retrospective cross-sectional study was performed on 53 children with ALL who were under the age of 18 years. Different factors including risk groups, age, gender, white blood cells, dosage, and duration of treatment, as well as baseline and posttreatment echocardiography findings including EF, E/E/, E//A/, MVE/A were evaluated in all patients. RESULTS: All enrolled patients had not any abnormalities in the baseline echocardiography preventing the beginning of chemotherapy. The results of this study did not show a significant difference in mean baseline echocardiography parameters after treatment including EF̨E/E/˛E//A/˛, MVE/A. In addition, there was no significant difference in mean EF, E//A/˛, MVE/A, and ˛E/E/ before and after treatment among SR, IR, and HR groups. Although there was no significant difference in the mean EF, E//A/, and MVE/A before and after treatment between male and female sex, the mean E/E/ after chemotherapy in girls (8.5 ± 0.7) was significantly higher than the mean before treatment (6.85 ± 1.5) (P < 0.001). It was also higher than the mean in boys (7.04 ± 0.99) (P = 0.019). Although there was no significant relationship between the duration of chemotherapy and the mean of EF̨E/E/˛E//A/˛, MVE/A after treatment, high dose of the drug was found to be significantly associated with a gradual decrease of EF or systolic function of the heart (P < 0.001). There was no significant relationship between drug dose and other parameters of echocardiography including E/ A/, MVE/A, and E/E/ after treatment. CONCLUSION: It appears that chemotherapy should not be delayed by echocardiography. Based on the findings presented herein, increasing the dose of anthracycline can be considered as an effective factor in reducing the systolic function of the heart (EF reduction). On the other hand, chemotherapy in the period of 1 to 5.3 years does not seem to have a significant effect on the mean parameters of EF, E/E/, E//, and MVE/A although another study with higher sample size and follow-up is needed to confirm these results.

Association between body mass index and insulin resistance in survivors of pediatric acute lymphoblastic leukemia.

In this retrospective cohort study, oral glucose tolerance testing (OGTT) was performed for all enrolled survivors of patients who suffered from acute lymphoblastic leukemia (ALL) at least 9 months after ending chemotherapy, as well as for control group. All demographic, treatment and anthropomorphic data were obtained from medical records. Furthermore, insulin resistance was evaluated according to insulin level, Matsuda and HOMA-IR index. Of 53 subjects, 28 exhibited B-cell ALL, and 25 were healthy children (F/M = 1.12/1). Mean age (±SD) was determined to be 10.77 ± 2.83 years, and meantime off for therapy was also recorded as 3.0 ± 1.6 years. ALL patients were diagnosed at age 1-15 years; none of them received the cranial radiotherapy, and demographic characteristics (age, sex, and sexual maturity) were found to be the same in both groups. Seven patients showed transient hyperglycemia during ALL treatment, however, no significant differences were found in terms of body mass index (BMI) means between ALL patients with and without transient hyperglycemia. Based on the data presented herein, insulin resistance could not be predicted by glucose levels during therapy, cumulative asparaginase, and steroid. Our findings demonstrated that elevated BMI could be a valuable predictor of insulin resistance probability in ALL patients. The presence of transient hyperglycemia does not appear to be associated with the increased risk of glucose intolerance or insulin resistance in the first few years after completion of therapy.

Outcome of Children with Wilms' Tumor in Developing Countries.

Wilms' tumor is the most common kidney tumor of childhood. The outcome of this malignant tumor has improved due to the improvement of therapeutic strategies. The most important factor in determining the prognosis of these patients is the histopathology subtype of the tumor; unfavorable histopathology is seen in only 11.5% of the patients, which accounts for 52% of deaths. Therefore, the aim of this study was to determine the outcome of children with Wilms' tumor referred to our hospital over a period of 10 years. This is a retrospective cohort study, and the target population included all patients with Wilms' tumor referred to Ali Asghar Hospital and were treated according to the National Wilms tumor study 4 (NWTS-4) protocol. All patients' data were extracted from the medical records of the department. Overall survival and event-free survival (EFS) were analyzed by the Kaplan Mayer method in the SPSS software, version 23. Fifty-two patients (24 male and 28 female patients) with Wilms' tumor were included. The mean age of the subjects was 40 months. The most common stage among boys and girls was stage II (23.08% and 28.85%, respectively). Our findings revealed that the overall five-year survival of patients was 87±5%; this figure was determined as 100% for boys and 76.8% ± 1.6 for girls (P = 0.018). Our findings show a dramatic improvement in the outcome of children with Wilms's tumor, and our results are comparable with other results from developed countries. Gender may be an independent prognostic factor of children with Wilms' tumor.

Evaluation of Efficacy, Safety, and Satisfaction Taking Deferasirox Twice Daily Versus Once Daily in Patients With Transfusion-Dependent Thalassemia.

OBJECTIVE: Deferasirox is a once-daily oral iron-chelation agent approved by the US Food and Drug Administration in November 2005. The authors aimed to evaluate efficacy, safety, and satisfaction of patients regarding twice-daily dose of deferasirox in patients with thalassemia who are resistant to once-daily regimen. METHODS: In this historical cohort multicenter study, 34 patients with beta-thalassemia major resistant or intolerant to once-daily dose of deferasirox (35 mg/kg/d) were investigated in 2016. Patients were registered at 3 thalassemia referral centers in Shiraz, southern Iran and Tehran, the capital of Iran. All patients were followed for 1 year and monitored by regular physical examination, laboratory data, serum ferritin levels, and heart and liver T2 magnetic resonance imaging. RESULTS: Mean age of thalassemia patients was 25.6±8.1 (8 to 40) years, including 22 female individuals and 12 male individuals. Serum ferritin levels significantly decreased during the study period (2021±955 at baseline vs. 1228±894 at the end of the study, P<0.001). Liver T2 magnetic resonance imaging of the patients demonstrated a significant improvement during the study. 73.3% of patients showed normal values at the end of study compared with 28.1% at the baseline (P<0.001). Drug side effects were reported only in 2 patients (5.8%) including 1 patient with abdominal pain and 1 with leukopenia and thrombocytopenia. CONCLUSIONS: It seems that deferasirox can be used with increased dose and twice daily with acceptable efficacy in unresponsive or intolerant thalassemia patients to once-daily dose. Close monitoring of the patients is necessary to detect and manage any possible adverse events.

Frequency of Cytogenetic Findings and its Effect on the Outcome of Pediatric Acute Lymphoblastic Leukemia.

INTRODUCTION: Acute lymphoblastic leukemia (ALL) is one of the most common cancers in children and accounts for about ⅓ of cancers in children. The annual incidence of ALL is 4 patients per 100,000 children. Their peak age is between 2-5 Year. One of the most important prognostic factors is cytogenetic abnormalities which are very effective in determining treatment policy. AIM: To determine the frequency of cytogenetic findings and its effect on the outcome of children with ALL. MATERIALS AND METHODS: This retrospective cross-sectional analytical study was conducted on children with ALL who their disease was diagnosed between 2001and 2009. Furthermore, 206 patients with ALL were examined by referring to Clinic of Ali Asghar Hospital in Tehran. Data was collected from medical records and analyzed by SPSS16 software. RESULTS: 206 children with ALL were enrolled in the study. The estimated event-free survival rate of all enrolled patients was more than 70%. There was a significant relationship between type of cytogenetic disorder and clinical outcome of patients (P˂0.0001), where the highest mortality was observed in patients with t (9;22) and t (4;11). There was no significant correlation between the sex and age with the clinical outcome of the patient (P = 0.064; p=0.322). There was a statistically significant relationship between mediastinal mass and clinical outcome (P = 0.002), indicating that the presence of cells growth in an involuntary way can be cause of the cancer. A significant association was found between the clinical outcome of patients and radiotherapy (P = 0.043), indicating that radiotherapy is effective in improving cancer. CONCLUSION: The findings demonstrated that the average survival rate without recurrence in children was at level of the European countries. However, the strong chemotherapy weakened the role of many prognostic factors in ALL patients, but some translocations are prognostic factors in predicting death in patients with ALL. Therefore, patients with this factor need to receive more confident treatment policy. Comprehensive studies are required by focusing on more samples because of low number of relapses and deaths in the present study.

Effects of chemotherapy prescription clinical decision-support systems on the chemotherapy process: A systematic review.

OBJECTIVE: To carry out a systematic review of studies assessing the effects of chemotherapy prescription clinical decision-support systems (CDSSs) on the chemotherapy process. METHODS: Articles published in English before May 1, 2017 and indexed in the PubMed and Embase databases were reviewed systematically. Studies that focused on the effects of chemotherapy prescription CDSSs on the chemotherapy process were included in this research and reviewed. RESULTS: 2283 articles were retrieved, of which 37 met the inclusion criteria. Twenty-seven of the included studies reported the effect of chemotherapy prescription CDSSs on medication errors, 18 studies focused on user satisfaction and system acceptance, 10 articles studied the effect of CDSSs on costs and care time and only 3 studies examined the impact on compliance with chemotherapy protocols. DISCUSSION AND CONCLUSION: In most of the studies, the use of CDSSs in chemotherapy prescription has reduced medication errors, especially dosage errors and has also reduced the time of chemotherapy process takes. However, in a few studies, the system has not been effective in reducing medication errors, has increased certain type of errors or has introduced new errors. Most of the software used has been specifically designed for the chemotherapy process and is intended to increase user satisfaction and system acceptance. There was not sufficient evidence on the effects of these systems on compliance with protocols and chemotherapy costs to draw firm conclusion. Higher quality studies are required to provide more evidence on the effects of CDSSs on medication errors, user satisfaction and system acceptance, costs, care time and compliance with protocols.

Peripheral primitive neuroectodermal tumor associated with paraneoplastic Cushing's syndrome: The rare case.

INTRODUCTION: Primitive neuroectodermal tumors (PNET) form a group of tumors defined by their appearance that are thought to develop from primitive (undifferentiated) nerve cells in the brain. They are rare tumors and their incidence is not well defined. CASE PRESENTATION: An 18-month-old male presenting with typical Cushingoid appearance (moon face, central obesity, hirsutism and growth arrest) was admitted for evaluation of endocrine problems. Subsequent laboratory studies revealed markedly elevated adrenocorticotropic (ACTH) and cortisol levels, as well as a hypokalemic metabolic alkalosis, these data are consistent with the diagnosis of Cushing's disease. He was treated with metyrapone to control hypercortisolemia. One month and a half later, a mass was detected in the abdomen by ultrasonography. An abdominal Computed tomography confirmed a large heterogeneous retroperitoneal mass with a significant amount of extension into surrounding structures which was removed by laparoscopic abdominal surgery. The patient's symptoms completely resolved and the ACTH and cortisol levels were also greatly reduced. Histologically, the tumor tissue consistent with the diagnosis of the retroperitoneal primitive neuroectodermal tumor which was confirmed immunohistochemically. This case demonstrates the successful diagnosis and treatment of a rare neoplasm. CONCLUSION: This is the first rare case with ectopic ACTH syndrome caused by the peripheral primitive neuroectodermal tumor.

Specifications of Computerized Provider Order Entry and Clinical Decision Support Systems for Cancer Patients Undergoing Chemotherapy: A Systematic Review.

OBJECTIVE: Chemotherapy medication errors are catastrophic. The prescription phase in the chemotherapy process plays a key role in the creation of medication errors, and therefore the use of computerized physician order entry (CPOE) and clinical decision support system (CDS) systems is recommended to reduce chemotherapy medication errors. The purpose of this study was to carry out a systematic review on the specifications of the CPOE and CDS systems for chemotherapy prescription. MATERIALS AND METHODS: A systematic review on articles published in English up to September 22, 2017, using the 3 databases PubMed, Embase, and Medline was conducted. Those articles that focused on the specifications of CPOE and CDSS in chemotherapy prescription were included in this review. FINDINGS: Of the 2,471 articles identified, 58 articles met the inclusion criteria and were included in this study. Specifications related to chemotherapy CPOE systems were categorized into the following 6 groups: automation and facilitation of the chemotherapy prescription phase, hospital workflow support, documentation and reporting, drug safety, information security, and system communications. The specifications of chemotherapy CDSS were also divided into 4 categories: embedding chemotherapy protocols, automated dose calculations and adjustment, providing alerts/reminders at the time of prescribing, and guiding or asking the user to complete the important prescription parameters. In 12 articles, the chemotherapy prescription CDSS were designed and evaluated independently of the CPOE; 45 articles provided prescription chemotherapy CDSS as part of the CPOE system, and in 1 article CPOE was introduced with no CDSS. CONCLUSION: In complicated settings such as chemotherapy, simplification of the processes is more imperative. The use of chemotherapy CPOE, which includes specifications for helping the medical staff with their workload, encourages the professionals to use such systems and increases the likelihood for success of these systems.

COMPARE CPM-RMI Trial: Intramyocardial Transplantation of Autologous Bone Marrow-Derived CD133+ Cells and MNCs during CABG in Patients with Recent MI: A Phase II/III, Multicenter, Placebo-Controlled, Randomized, Double-Blind Clinical Trial.

This article published in Cell J (Yakhteh), Vol 20, No 2, Jul-Sep 2018, on pages 267-277, four affiliations (1, 4, 5, and 10) were changed based on authors request.

COMPARE CPM-RMI Trial: Intramyocardial Transplantation of Autologous Bone Marrow-Derived CD133+ Cells and MNCs during CABG in Patients with Recent MI: A Phase II/III, Multicenter, Placebo-Controlled, Randomized, Double-Blind Clinical Trial.

OBJECTIVES: The regenerative potential of bone marrow-derived mononuclear cells (MNCs) and CD133+ stem cells in the heart varies in terms of their pro-angiogenic effects. This phase II/III, multicenter and double-blind trial is designed to compare the functional effects of intramyocardial autologous transplantation of both cell types and placebo in patients with recent myocardial infarction (RMI) post-coronary artery bypass graft. MATERIALS AND METHODS: This was a phase II/III, randomized, double-blind, placebo-controlled trial COMPARE CPM-RMI (CD133, Placebo, MNCs - recent myocardial infarction) conducted in accordance with the Declaration of Helsinki that assessed the safety and efficacy of CD133 and MNCs compared to placebo in patients with RMI. We randomly assigned 77 eligible RMI patients selected from 5 hospitals to receive CD133+ cells, MNC, or a placebo. Patients underwent gated single photon emission computed tomography assessments at 6 and 18 months post-intramyocardial transplantation. We tested the normally distributed efficacy outcomes with a mixed analysis of variance model that used the entire data set of baseline and between-group comparisons as well as within subject (time) and group×time interaction terms. RESULTS: There were no related serious adverse events reported. The intramyocardial transplantation of both cell types increased left ventricular ejection fraction by 9% [95% confidence intervals (CI): 2.14% to 15.78%, P=0.01] and improved decreased systolic wall thickening by -3.7 (95% CI: -7.07 to -0.42, P=0.03). The CD133 group showed significantly decreased non-viable segments by 75% (P=0.001) compared to the placebo and 60% (P=0.01) compared to the MNC group. We observed this improvement at both the 6- and 18-month time points. CONCLUSIONS: Intramyocardial injections of CD133+ cells or MNCs appeared to be safe and efficient with superiority of CD133+ cells for patients with RMI. Although the sample size precluded a definitive statement about clinical outcomes, these results have provided the basis for larger studies to confirm definitive evidence about the efficacy of these cell types (Registration Number: NCT01167751).

Bone Mineral Density in Survivors of Childhood Acute Lymphoblastic Leukemia

Background: The objective of this study was to evaluate bone mineral density (BMD) after completion of treatment for childhood acute lymphoblastic leukemia (ALL). Methods: In this cross-sectional study, 103 survivors of ALL aged 13.5 ± 0.45 who completed their treatment at least one year earlier were enrolled. Among these, 49.5% and 51.5% received chemotherapy alone and chemotherapy plus cranial radiotherapy, respectively. Bone mineral content, BMD, and bone mineral apparent density in the lumbar spine (LS), femoral neck (FN) and forearm were assessed using dual-energy X-ray absorptiometry (DEXA). BMD Z-scores were classified according to International Society for Clinical Densitometry (ISCD) criteria. Results: The mean BMD Z-scores ± SD forLS, FN and forearm were -1.60 ± 0.12, -1.21 ± 0.9 and -2.43 ± 0.14 respectively with significant differences (P<0.001). Considering the lowest BMD Z-score in LS and FN areas (at any site) and according to the ISCD classification, 62.1%, 33% and 4.9% of the patients had normal BMD, low BMD and osteoporosis, respectively. Also, 8.7% of patients had developed fractures after completion of the treatment period, 4.9% having BMD Z-Scores <-2 SD at any site. A direct relationship was apparent between BMD Z-scores at LS and FN at any sites and risk of fracture (P<0.001). Conclusions: ALL patients are at risk for low BMD and fracture. Therefore, applying DEXA scanning is recommended after completion of therapy for prevention of BMD reduction and osteoporosis.

Bilateral Maxillary, Sphenoid Sinuses and Lumbosacral Spinal Cord Extramedullary Relapse of CML Following Allogeneic Stem Cell Transplant.

Isolated extramedullary relapse of chronic myelogenous leukemia (CML) after allogeneic stem cell transplant is rare. There is a case report of a child who developed a granulocytic sarcoma of the maxillary and sphenoid sinuses and lumbosacral spinal cord mass 18 months after allogeneic bone marrow transplant for CML. He was presented with per orbital edema and neurological deficit of lower extremities and a mass lesion was found on spinal cord imaging. No evidence of hematologic relapse was identified at that time by bone marrow histology or cytogenetic. The patient died 1 month later with a picture of pneumonia, left ventricular dysfunction and a cardiopulmonary arrest on a presumed underlying sepsis with infectious etiology. Granulocytic sarcoma should be considered in the differential diagnosis of mass lesions presenting after allogeneic bone marrow transplantation for CML, even if there is no evidence of bone marrow involvement.