International Journal of Pediatric Otorhinolaryngology, 2019
Introduction: Tonsillectomy and adenoidectomy (T& A) are the most common pediatric surgical proce... more Introduction: Tonsillectomy and adenoidectomy (T& A) are the most common pediatric surgical procedures performed worldwide. Bleeding remains the most common complication of these procedures with 1-5.7 % prevalence. Methods: We recruited 1269 patients who were scheduled for either tonsillectomy, adenoidectomy or both. All patients had preoperative CBC, PT, and aPTT ordered. According to the results, patients were labelled as either "abnormal group" or "normal group". Results: 35 patients had abnormal lab results 18 of these patients were diagnosed with coagulation disorders on further laboratory testing.9 of these patients had no pertinent history of bleeding. Even though an association is noted between abnormal lab tests and preoperative history of risk of bleeding, the correlation did not have high specificity (28.6%). Conclusion: This study provides evidence that preoperative history can give some information on patients with abnormal coagulation profile but may underestimate the prevalence of such diseases. In addition, patients with abnormal coagulation profile have more risk of postoperative bleeding even after adequate medical treatment perioperative. Thus, identifying these patients will help the clinician in providing the best surgical management with the least morbidity and mortality.
For 15 years, we have determined urinary vanillylmandelic acid (VMA) by a method (1) that relies ... more For 15 years, we have determined urinary vanillylmandelic acid (VMA) by a method (1) that relies on the reaction of extracted VMA with the diazo derivative of p -nitroaniline to give a pink chromophore; the chromophore undergoes a bathochromic shift in the presence of an aprotic solvent and a base to give a blue derivative with maximum absorbance at 600 nm. Hitherto, we observed neither positive nor negative interferences. Recently, however, we observed the development of a pink color in the final stage of the procedure, masking the blue color of VMA (Fig. 1⇓ ). The urine …
Expandable endoprostheses provide a limb salvage option for skeletally immature patients with bon... more Expandable endoprostheses provide a limb salvage option for skeletally immature patients with bone sarcoma of the lower extremities. Initial reports of the Repiphysis prosthesis were encouraging; however, medium-term follow-up revealed high complication rates. We report on the long-term follow-up of a cohort of patients treated with the Repiphysis prosthesis. Eleven patients were included in the study. Data collected included sex, age at surgery, duration of follow-up, site of disease, histologic diagnosis, number of lengthening sessions, amount lengthened, postoperative complications, endoprosthetic failure, mode of endoprosthetic failure, duration from index surgery to failure and to revision, type of revision surgery and final limb-length discrepancy. The average duration of follow-up from the time of surgery was 180 months (range, 144–215 months). Fifteen Repiphysis implants were used in 11 patients. All implants failed with an average time from surgery to failure of 36 months (range, 3–72 months). Twenty-four complications were observed: one wound dehiscence, two deep infections, 18 mechanical failures, implant collapse with destruction of proximal tibia epiphysis in two and one periprosthetic proximal femur fracture with dislodgement of the stem. Despite being an option for limb salvage, the Repiphysis prosthesis has a high rate of mechanical failure and need for revision, similar to other expandable implants. The authors, therefore, recommend full disclosure of the potential short- and long-term complications and need for revision, as well as alternative treatment options if their use is considered. Level of evidence: IV (Therapeutic).
Background: Interindividual variability in thiopurine-related toxicity could not be completely ex... more Background: Interindividual variability in thiopurine-related toxicity could not be completely explained by thiopurine S-methyltransferase (TPMT) polymorphisms, as a number of patients who are homozygous wild type or normal for TPMT still develop toxicity that necessitates 6mercaptopurine (MP) dose reduction or protocol interruption. Recently, few studies reported on an inherited nucleoside diphosphate-linked moiety X motif 15 (NUDT15) c.415C>T low-function variant that is associated with decreased thiopurine metabolism and leukopenia in childhood acute lymphoblastic leukemia (ALL) and other diseases. Procedures: The aim of this study is to measure the frequency of TPMT and NUDT15 polymorphisms and assess whether they are predictors of MP intolerance in children treated for ALL. One hundred thirty-seven patients with ALL of whom 121 were Lebanese were evaluated. MP dose intensity was calculated as the ratio of the tolerated MP dose to planned dose during continuation phase to maintain an absolute neutrophil count (ANC) dose above 300 per l. Results: One patient was NUDT15 heterozygous TC and tolerated only 33.33% of the planned MP dose, which was statistically significantly different from the median-tolerated MP dose intensity of the rest of the cohort (76.00%). Three patients had the TPMT*3A haplotype and tolerated 40.00-66.66% of the planned MP dose, which was also statistically significantly different from the rest of the cohort.
The aim of this work was to determine the role of MRI in interpreting abnormal signals within bon... more The aim of this work was to determine the role of MRI in interpreting abnormal signals within bones and soft tissues adjacent to tumor bulk of osteosarcoma and Ewing's sarcoma in a pediatric population by correlating MR findings with histopathology. Thirty patients met the inclusion criteria, which included (1) osteosarcoma or Ewing's sarcoma, (2) MR studies no more than 2 months prior to surgery, (3) presence of abnormal MR signal surrounding the tumor bulk, (4) pathological material from resected tumor. The patients received standard neoadjuvant chemotherapy. Using grid maps on gross pathology specimens, the abnormal MR areas around the tumor were matched with the corresponding grid sections. Histopathology slides of these sections were then analyzed to determine the nature of the regions of interest. The MR/pathological correlation was evaluated using Mann-Whitney U test and Fisher's exact test. Twenty-seven patients had osteosarcoma and three patients had Ewing's sarcoma. Of the studied areas, 17.4% were positive for tumor (viable or necrotic). There was no statistically significant correlation between areas positive for tumor and age, gender, signal extent and intensity on MRI, or tissue type. There was, however, a statistically significant correlation between presence of tumor and the appearance of abnormal soft tissue signals. A feathery appearance correlated with tumor-negative areas whereas a bulky appearance correlated with tumor-positive regions. MR imaging is helpful in identifying the nature of abnormal signal areas surrounding bone sarcomas that are more likely to be tumor-free, particularly when the signal in the soft tissues surrounding the tumor is feathery and edema-like in appearance.
Background Viral infections in children and adolescents with malignancy are commonly encountered ... more Background Viral infections in children and adolescents with malignancy are commonly encountered and have a significant impact on morbidity and mortality. Studies and epidemiological data regarding viral infections in children with cancer in developing countries are lacking. This retrospective cohort study aims to assess the burden of viral infections in children and adolescents with cancer, by assessing prevalence, risk factors, as well as morbidity and mortality of common viruses over a period of 8 years. Methods and findings Medical records of cancer patients treated at the Children Cancer Center of Lebanon were reviewed and 155 participants under the age of 21 were identified with at least one documented viral infection during the period from July 2009 to November 2017. This subset included 136 participants with active malignancy and 19 participants with a history of cancer who underwent hematopoietic stem cell transplantation [HSCT] and were in remission; the latter group was analyzed separately. Information regarding participant characteristics, hospital course, and complications were obtained. Associations between viral infections and certain factors were assessed. In the cohort, 64% were male, 81% were Lebanese. In participants with active malignancy, 90% received chemotherapy in the 6 months preceding the viral infection episode, 11% received radiotherapy. 51% of participants were neutropenic at the time of viral detection, and 77% were lymphopenic. 17% experienced a bacterial coinfection, and 3 experienced a viral co-infection. Among 162 viral infection episodes,
Outcome of severe acquired aplastic anemia (SAA) has improved dramatically over the past two deca... more Outcome of severe acquired aplastic anemia (SAA) has improved dramatically over the past two decades due to better regimens for allogeneic hematopoietic stem cell transplantation (allo-HSCT) [1,2]. Different conditioning regimens have been evaluated in matched-related donor (MRD) allografts with varying results. Before 2008, we encountered low incidence of day-30 engraftment in 3 patients and one death due to graft failure when using the standard conditioning regimen of high dose cyclophosphamide (200 mg/kg) and antithymocyte globulin (ATG) [3]. Srinivasan et al described outcomes following fludarabine in combination with cyclophosphamide (120 mg/kg) with encouraging engraftment results; however, a high incidence of acute and chronic GvHD was reported [4]. Fludarabine, very low dose cyclophosphamide (1200 mg/m 2) and ATG was pioneered by Bacigalupo et al. for alternative donor transplants with resulting graft failure rate of up to 18% and a 2-year overall survival of 73% [5]. Since 2008, we adopted this regimen in addition to low dose total-body irradiation (TBI) for patients older than 15 years for MRD transplants aiming at improving engraftment and ultimately achieving better survival. 2. Methods This is a retrospective review of all consecutive patients diagnosed with acquired SAA who underwent allo-HSCT between August 2008 and December 2015 at the American University of Beirut Medical Center (AUBMC). Patients transplanted for other forms of marrow failure (Fanconi anemia, pure red cell aplasia, dyskeratosis congenita) were excluded. This study was approved by our Institutional Review Board and was conducted in accordance with the declaration of Helsinki.
Optimal treatment for Hodgkin's disease during childhood is unknown. We report the treatment outc... more Optimal treatment for Hodgkin's disease during childhood is unknown. We report the treatment outcome of patients with Hodgkin's disease ≤13 years of age seen at the American University of Beirut Medical Center (AUBMC) between 1980 and 1996. A retrospective review of the medical records of 24 children treated for HD at AUBMC was performed. Treatment consisted of chemotherapy alone (n = 15) or chemotherapy plus involved field radiotherapy (n = 9). Chemotherapy consisted of COPP, ABVD, or alternating cycles of each for a total of 6 to 12 cycles, depending on clinical and radiological response; three patients received MOPP. Five patients in the chemotherapy group had clinical stage (CS) I and II and 10 had CS III disease. In the combined modality group, eight patients had CS I and II and one had CS IV disease. At a median follow-up of 5 years, the event-free survival (EFS) for the combined modality group was 100% and the overall survival (OS) 100%. For the chemotherapy alone group, the EFS was 56% and the OS was 79%. Four patients (27%) in the chemotherapy alone group who had Stage IIIB disease relapsed. Mean time to relapse was 4.3 years. In our experience, six cycles of COPP or (COPP plus ABVD) alone were suboptimal for the treatment of Stage IIIB Hodgkin's disease patients, especially those with involvement of lower abdominal nodes (III2B), extensive pulmonary disease, or mixed cellularity histology. Radiation therapy or additional chemotherapy courses are required for these patients.
Translocations involving chromosomes 1 and 15 are uncommon in hematologic malignancies. So far, o... more Translocations involving chromosomes 1 and 15 are uncommon in hematologic malignancies. So far, only 42 cases have been reported with t(1;15) as a reciprocal or complex chromosomal abnormalities. We herein report the first case in the literature, to our knowledge, of a 44-year-old female with essential thrombocythemia and severe myelofibrosis who developed acute myeloid leukemia (AML-M4) with der(1;15)(q10;q10) after 13 years of treatment. In addition, we reviewed the literature for all up-todate published cases with t(1;15).
Pediatric Hematology Oncology Journal, Mar 1, 2020
The Pediatric Oncology East and Mediterranean (POEM) group was established in 2013 as a cooperati... more The Pediatric Oncology East and Mediterranean (POEM) group was established in 2013 as a cooperative platform for physicians, scientists and healthcare professionals from institutions in the East and Mediterranean area with the goal of sharing experiences, initiating cooperative trials and establishing common strategies to achieve optimization of the care of pediatric oncology patients in the region. The mission of POEM group is to continuously improve pediatric oncology care, research, training and advocacy in the East and Mediterranean region by means of integrated teamwork. Our Vision is to be the leading pediatric oncology collaborative group in the East and Mediterranean Region. POEM group is currently at more than 550 registrants from 119 centers in 28 countries regionally. This review summarizes the structure and activities of POEM group since its inception, and highlights identified challenges and future plans.
Purpose of review Acute lymphoblastic leukemia is the most common pediatric cancer. The cure rate... more Purpose of review Acute lymphoblastic leukemia is the most common pediatric cancer. The cure rate of this disease is over 80% in developed countries utilizing protocols with very tolerable toxicity. Several factors contributed to this success, including the implementation of large collaborative clinical trials and the better understanding of disease biology allowing for risk-stratified treatment. We will review the current state of the treatment of acute lymphoblastic leukemia in low-income and medium-income countries. Recent findings The picture differs from country to country, but recent advances have been made in many countries and the cure rates have improved significantly. Tackling problems such as access to care, abandonment of treatment and toxicity of protocols and the development of centers of excellence are critical for continued improvement. Twinning with programs in developed countries and the creation of regional collaborative groups will allow the implementation of risk-directed therapy and better supportive care.
Resource‐limited settings often have financial barriers to genetic testing for heritable cancer. ... more Resource‐limited settings often have financial barriers to genetic testing for heritable cancer. This retrospective study investigated the pattern of heritable cancer predisposition testing in a middle‐income country over the period 2014–2021, excluding retinoblastoma. After establishing a specific fund in 2019, rate of tests increased from 1.1% to 10.9% of new diagnoses. Most common testing was for constitutional mismatch repair deficiency (CMMRD), rhabdoid predisposition syndrome, TP53 (tumor protein 53) mutation, and hereditary cancer panel. Of 33 patients, 13 (39%) tested positive, 12 (36%) negative, and eight (24%) had variants of unknown significance. Positivity rate was 43% for a clinical phenotype and 44% for a tumor type indication.
Hematology/Oncology and Stem Cell Therapy, Jun 1, 2017
A 5-year-old boy with intermediate-risk acute lymphoblastic leukemia (ALL) presented to our Emerg... more A 5-year-old boy with intermediate-risk acute lymphoblastic leukemia (ALL) presented to our Emergency Department 3 weeks after finishing chemotherapy with dysuria, fecaluria, suprapubic abdominal pain, high-grade fever, vomiting, and diarrhea. Vital signs were stable. There were no specific findings on physical examination, except for suprapubic tenderness. Workup showed positive Clostridium difficile toxin in the stool and hyperchloremic normal anion-gap metabolic acidosis with a bicarbonate of 11 mEq/L. The complete blood count revealed white blood cells of 7.8 Â 10 9 /L with 88% segmented neutrophils and an absolute neutrophil count of 6.8 Â 10 9 /L. Urine analysis showed 3-6 white blood cells per high-power field, 2-4 red blood cells per high-power field, negative nitrate, and negative leukocyte esterase. Computed tomography with contrast scan of the abdomen showed a vesicoileal fistula with enteroenteric fistula at the level of the anterior dome of the bladder associated with ileal bowel wall thickening, in keeping with inflammatory reaction (Figs. 1 and 2). Intravenous alkalinization was started. Antibiotics, including
The triad of thiamine‐responsive anaemia, diabetes mellitus and deafness has been reported in 15 ... more The triad of thiamine‐responsive anaemia, diabetes mellitus and deafness has been reported in 15 patients with macrocytic anaemia, sometimes associated with moderate thrombocytopenia. The bone marrow aspirate usually shows megaloblastic changes and ringed sideroblasts. However, tri‐lineage myelodysplasia has never been reported. We describe two patients who presented with diabetes, deafness and thiamine‐responsive pancytopenia. Bone marrow aspirate and biopsy were typical of tri‐lineage myelodysplasia. These findings suggest that thiamine may have a role in the regulation of haemopoiesis at the stem cell level. We propose the term ‘thiamine‐responsive myelodysplasia’ rather than that of thiamine‐responsive anaemia.
Melorheostosis, also known as Leri's disease, is a rare benign form of mesodermal mixed sclerosin... more Melorheostosis, also known as Leri's disease, is a rare benign form of mesodermal mixed sclerosing bone dysplasia. We report the unusual case of a 14-year-old boy with melorheostosis in the lower extremity that went undiagnosed due to concurrent Ewing sarcoma in the opposite limb, confounding the findings for metastatic disease. The diagnosis was made on FDG PET/CT when the patient presented for post Ewing sarcoma treatment follow-up. The different types of melorheostosis as well as the challenge of diagnosing this rare entity are discussed in this report.
International Journal of Pediatric Otorhinolaryngology, 2019
Introduction: Tonsillectomy and adenoidectomy (T& A) are the most common pediatric surgical proce... more Introduction: Tonsillectomy and adenoidectomy (T& A) are the most common pediatric surgical procedures performed worldwide. Bleeding remains the most common complication of these procedures with 1-5.7 % prevalence. Methods: We recruited 1269 patients who were scheduled for either tonsillectomy, adenoidectomy or both. All patients had preoperative CBC, PT, and aPTT ordered. According to the results, patients were labelled as either "abnormal group" or "normal group". Results: 35 patients had abnormal lab results 18 of these patients were diagnosed with coagulation disorders on further laboratory testing.9 of these patients had no pertinent history of bleeding. Even though an association is noted between abnormal lab tests and preoperative history of risk of bleeding, the correlation did not have high specificity (28.6%). Conclusion: This study provides evidence that preoperative history can give some information on patients with abnormal coagulation profile but may underestimate the prevalence of such diseases. In addition, patients with abnormal coagulation profile have more risk of postoperative bleeding even after adequate medical treatment perioperative. Thus, identifying these patients will help the clinician in providing the best surgical management with the least morbidity and mortality.
For 15 years, we have determined urinary vanillylmandelic acid (VMA) by a method (1) that relies ... more For 15 years, we have determined urinary vanillylmandelic acid (VMA) by a method (1) that relies on the reaction of extracted VMA with the diazo derivative of p -nitroaniline to give a pink chromophore; the chromophore undergoes a bathochromic shift in the presence of an aprotic solvent and a base to give a blue derivative with maximum absorbance at 600 nm. Hitherto, we observed neither positive nor negative interferences. Recently, however, we observed the development of a pink color in the final stage of the procedure, masking the blue color of VMA (Fig. 1⇓ ). The urine …
Expandable endoprostheses provide a limb salvage option for skeletally immature patients with bon... more Expandable endoprostheses provide a limb salvage option for skeletally immature patients with bone sarcoma of the lower extremities. Initial reports of the Repiphysis prosthesis were encouraging; however, medium-term follow-up revealed high complication rates. We report on the long-term follow-up of a cohort of patients treated with the Repiphysis prosthesis. Eleven patients were included in the study. Data collected included sex, age at surgery, duration of follow-up, site of disease, histologic diagnosis, number of lengthening sessions, amount lengthened, postoperative complications, endoprosthetic failure, mode of endoprosthetic failure, duration from index surgery to failure and to revision, type of revision surgery and final limb-length discrepancy. The average duration of follow-up from the time of surgery was 180 months (range, 144–215 months). Fifteen Repiphysis implants were used in 11 patients. All implants failed with an average time from surgery to failure of 36 months (range, 3–72 months). Twenty-four complications were observed: one wound dehiscence, two deep infections, 18 mechanical failures, implant collapse with destruction of proximal tibia epiphysis in two and one periprosthetic proximal femur fracture with dislodgement of the stem. Despite being an option for limb salvage, the Repiphysis prosthesis has a high rate of mechanical failure and need for revision, similar to other expandable implants. The authors, therefore, recommend full disclosure of the potential short- and long-term complications and need for revision, as well as alternative treatment options if their use is considered. Level of evidence: IV (Therapeutic).
Background: Interindividual variability in thiopurine-related toxicity could not be completely ex... more Background: Interindividual variability in thiopurine-related toxicity could not be completely explained by thiopurine S-methyltransferase (TPMT) polymorphisms, as a number of patients who are homozygous wild type or normal for TPMT still develop toxicity that necessitates 6mercaptopurine (MP) dose reduction or protocol interruption. Recently, few studies reported on an inherited nucleoside diphosphate-linked moiety X motif 15 (NUDT15) c.415C>T low-function variant that is associated with decreased thiopurine metabolism and leukopenia in childhood acute lymphoblastic leukemia (ALL) and other diseases. Procedures: The aim of this study is to measure the frequency of TPMT and NUDT15 polymorphisms and assess whether they are predictors of MP intolerance in children treated for ALL. One hundred thirty-seven patients with ALL of whom 121 were Lebanese were evaluated. MP dose intensity was calculated as the ratio of the tolerated MP dose to planned dose during continuation phase to maintain an absolute neutrophil count (ANC) dose above 300 per l. Results: One patient was NUDT15 heterozygous TC and tolerated only 33.33% of the planned MP dose, which was statistically significantly different from the median-tolerated MP dose intensity of the rest of the cohort (76.00%). Three patients had the TPMT*3A haplotype and tolerated 40.00-66.66% of the planned MP dose, which was also statistically significantly different from the rest of the cohort.
The aim of this work was to determine the role of MRI in interpreting abnormal signals within bon... more The aim of this work was to determine the role of MRI in interpreting abnormal signals within bones and soft tissues adjacent to tumor bulk of osteosarcoma and Ewing's sarcoma in a pediatric population by correlating MR findings with histopathology. Thirty patients met the inclusion criteria, which included (1) osteosarcoma or Ewing's sarcoma, (2) MR studies no more than 2 months prior to surgery, (3) presence of abnormal MR signal surrounding the tumor bulk, (4) pathological material from resected tumor. The patients received standard neoadjuvant chemotherapy. Using grid maps on gross pathology specimens, the abnormal MR areas around the tumor were matched with the corresponding grid sections. Histopathology slides of these sections were then analyzed to determine the nature of the regions of interest. The MR/pathological correlation was evaluated using Mann-Whitney U test and Fisher's exact test. Twenty-seven patients had osteosarcoma and three patients had Ewing's sarcoma. Of the studied areas, 17.4% were positive for tumor (viable or necrotic). There was no statistically significant correlation between areas positive for tumor and age, gender, signal extent and intensity on MRI, or tissue type. There was, however, a statistically significant correlation between presence of tumor and the appearance of abnormal soft tissue signals. A feathery appearance correlated with tumor-negative areas whereas a bulky appearance correlated with tumor-positive regions. MR imaging is helpful in identifying the nature of abnormal signal areas surrounding bone sarcomas that are more likely to be tumor-free, particularly when the signal in the soft tissues surrounding the tumor is feathery and edema-like in appearance.
Background Viral infections in children and adolescents with malignancy are commonly encountered ... more Background Viral infections in children and adolescents with malignancy are commonly encountered and have a significant impact on morbidity and mortality. Studies and epidemiological data regarding viral infections in children with cancer in developing countries are lacking. This retrospective cohort study aims to assess the burden of viral infections in children and adolescents with cancer, by assessing prevalence, risk factors, as well as morbidity and mortality of common viruses over a period of 8 years. Methods and findings Medical records of cancer patients treated at the Children Cancer Center of Lebanon were reviewed and 155 participants under the age of 21 were identified with at least one documented viral infection during the period from July 2009 to November 2017. This subset included 136 participants with active malignancy and 19 participants with a history of cancer who underwent hematopoietic stem cell transplantation [HSCT] and were in remission; the latter group was analyzed separately. Information regarding participant characteristics, hospital course, and complications were obtained. Associations between viral infections and certain factors were assessed. In the cohort, 64% were male, 81% were Lebanese. In participants with active malignancy, 90% received chemotherapy in the 6 months preceding the viral infection episode, 11% received radiotherapy. 51% of participants were neutropenic at the time of viral detection, and 77% were lymphopenic. 17% experienced a bacterial coinfection, and 3 experienced a viral co-infection. Among 162 viral infection episodes,
Outcome of severe acquired aplastic anemia (SAA) has improved dramatically over the past two deca... more Outcome of severe acquired aplastic anemia (SAA) has improved dramatically over the past two decades due to better regimens for allogeneic hematopoietic stem cell transplantation (allo-HSCT) [1,2]. Different conditioning regimens have been evaluated in matched-related donor (MRD) allografts with varying results. Before 2008, we encountered low incidence of day-30 engraftment in 3 patients and one death due to graft failure when using the standard conditioning regimen of high dose cyclophosphamide (200 mg/kg) and antithymocyte globulin (ATG) [3]. Srinivasan et al described outcomes following fludarabine in combination with cyclophosphamide (120 mg/kg) with encouraging engraftment results; however, a high incidence of acute and chronic GvHD was reported [4]. Fludarabine, very low dose cyclophosphamide (1200 mg/m 2) and ATG was pioneered by Bacigalupo et al. for alternative donor transplants with resulting graft failure rate of up to 18% and a 2-year overall survival of 73% [5]. Since 2008, we adopted this regimen in addition to low dose total-body irradiation (TBI) for patients older than 15 years for MRD transplants aiming at improving engraftment and ultimately achieving better survival. 2. Methods This is a retrospective review of all consecutive patients diagnosed with acquired SAA who underwent allo-HSCT between August 2008 and December 2015 at the American University of Beirut Medical Center (AUBMC). Patients transplanted for other forms of marrow failure (Fanconi anemia, pure red cell aplasia, dyskeratosis congenita) were excluded. This study was approved by our Institutional Review Board and was conducted in accordance with the declaration of Helsinki.
Optimal treatment for Hodgkin's disease during childhood is unknown. We report the treatment outc... more Optimal treatment for Hodgkin's disease during childhood is unknown. We report the treatment outcome of patients with Hodgkin's disease ≤13 years of age seen at the American University of Beirut Medical Center (AUBMC) between 1980 and 1996. A retrospective review of the medical records of 24 children treated for HD at AUBMC was performed. Treatment consisted of chemotherapy alone (n = 15) or chemotherapy plus involved field radiotherapy (n = 9). Chemotherapy consisted of COPP, ABVD, or alternating cycles of each for a total of 6 to 12 cycles, depending on clinical and radiological response; three patients received MOPP. Five patients in the chemotherapy group had clinical stage (CS) I and II and 10 had CS III disease. In the combined modality group, eight patients had CS I and II and one had CS IV disease. At a median follow-up of 5 years, the event-free survival (EFS) for the combined modality group was 100% and the overall survival (OS) 100%. For the chemotherapy alone group, the EFS was 56% and the OS was 79%. Four patients (27%) in the chemotherapy alone group who had Stage IIIB disease relapsed. Mean time to relapse was 4.3 years. In our experience, six cycles of COPP or (COPP plus ABVD) alone were suboptimal for the treatment of Stage IIIB Hodgkin's disease patients, especially those with involvement of lower abdominal nodes (III2B), extensive pulmonary disease, or mixed cellularity histology. Radiation therapy or additional chemotherapy courses are required for these patients.
Translocations involving chromosomes 1 and 15 are uncommon in hematologic malignancies. So far, o... more Translocations involving chromosomes 1 and 15 are uncommon in hematologic malignancies. So far, only 42 cases have been reported with t(1;15) as a reciprocal or complex chromosomal abnormalities. We herein report the first case in the literature, to our knowledge, of a 44-year-old female with essential thrombocythemia and severe myelofibrosis who developed acute myeloid leukemia (AML-M4) with der(1;15)(q10;q10) after 13 years of treatment. In addition, we reviewed the literature for all up-todate published cases with t(1;15).
Pediatric Hematology Oncology Journal, Mar 1, 2020
The Pediatric Oncology East and Mediterranean (POEM) group was established in 2013 as a cooperati... more The Pediatric Oncology East and Mediterranean (POEM) group was established in 2013 as a cooperative platform for physicians, scientists and healthcare professionals from institutions in the East and Mediterranean area with the goal of sharing experiences, initiating cooperative trials and establishing common strategies to achieve optimization of the care of pediatric oncology patients in the region. The mission of POEM group is to continuously improve pediatric oncology care, research, training and advocacy in the East and Mediterranean region by means of integrated teamwork. Our Vision is to be the leading pediatric oncology collaborative group in the East and Mediterranean Region. POEM group is currently at more than 550 registrants from 119 centers in 28 countries regionally. This review summarizes the structure and activities of POEM group since its inception, and highlights identified challenges and future plans.
Purpose of review Acute lymphoblastic leukemia is the most common pediatric cancer. The cure rate... more Purpose of review Acute lymphoblastic leukemia is the most common pediatric cancer. The cure rate of this disease is over 80% in developed countries utilizing protocols with very tolerable toxicity. Several factors contributed to this success, including the implementation of large collaborative clinical trials and the better understanding of disease biology allowing for risk-stratified treatment. We will review the current state of the treatment of acute lymphoblastic leukemia in low-income and medium-income countries. Recent findings The picture differs from country to country, but recent advances have been made in many countries and the cure rates have improved significantly. Tackling problems such as access to care, abandonment of treatment and toxicity of protocols and the development of centers of excellence are critical for continued improvement. Twinning with programs in developed countries and the creation of regional collaborative groups will allow the implementation of risk-directed therapy and better supportive care.
Resource‐limited settings often have financial barriers to genetic testing for heritable cancer. ... more Resource‐limited settings often have financial barriers to genetic testing for heritable cancer. This retrospective study investigated the pattern of heritable cancer predisposition testing in a middle‐income country over the period 2014–2021, excluding retinoblastoma. After establishing a specific fund in 2019, rate of tests increased from 1.1% to 10.9% of new diagnoses. Most common testing was for constitutional mismatch repair deficiency (CMMRD), rhabdoid predisposition syndrome, TP53 (tumor protein 53) mutation, and hereditary cancer panel. Of 33 patients, 13 (39%) tested positive, 12 (36%) negative, and eight (24%) had variants of unknown significance. Positivity rate was 43% for a clinical phenotype and 44% for a tumor type indication.
Hematology/Oncology and Stem Cell Therapy, Jun 1, 2017
A 5-year-old boy with intermediate-risk acute lymphoblastic leukemia (ALL) presented to our Emerg... more A 5-year-old boy with intermediate-risk acute lymphoblastic leukemia (ALL) presented to our Emergency Department 3 weeks after finishing chemotherapy with dysuria, fecaluria, suprapubic abdominal pain, high-grade fever, vomiting, and diarrhea. Vital signs were stable. There were no specific findings on physical examination, except for suprapubic tenderness. Workup showed positive Clostridium difficile toxin in the stool and hyperchloremic normal anion-gap metabolic acidosis with a bicarbonate of 11 mEq/L. The complete blood count revealed white blood cells of 7.8 Â 10 9 /L with 88% segmented neutrophils and an absolute neutrophil count of 6.8 Â 10 9 /L. Urine analysis showed 3-6 white blood cells per high-power field, 2-4 red blood cells per high-power field, negative nitrate, and negative leukocyte esterase. Computed tomography with contrast scan of the abdomen showed a vesicoileal fistula with enteroenteric fistula at the level of the anterior dome of the bladder associated with ileal bowel wall thickening, in keeping with inflammatory reaction (Figs. 1 and 2). Intravenous alkalinization was started. Antibiotics, including
The triad of thiamine‐responsive anaemia, diabetes mellitus and deafness has been reported in 15 ... more The triad of thiamine‐responsive anaemia, diabetes mellitus and deafness has been reported in 15 patients with macrocytic anaemia, sometimes associated with moderate thrombocytopenia. The bone marrow aspirate usually shows megaloblastic changes and ringed sideroblasts. However, tri‐lineage myelodysplasia has never been reported. We describe two patients who presented with diabetes, deafness and thiamine‐responsive pancytopenia. Bone marrow aspirate and biopsy were typical of tri‐lineage myelodysplasia. These findings suggest that thiamine may have a role in the regulation of haemopoiesis at the stem cell level. We propose the term ‘thiamine‐responsive myelodysplasia’ rather than that of thiamine‐responsive anaemia.
Melorheostosis, also known as Leri's disease, is a rare benign form of mesodermal mixed sclerosin... more Melorheostosis, also known as Leri's disease, is a rare benign form of mesodermal mixed sclerosing bone dysplasia. We report the unusual case of a 14-year-old boy with melorheostosis in the lower extremity that went undiagnosed due to concurrent Ewing sarcoma in the opposite limb, confounding the findings for metastatic disease. The diagnosis was made on FDG PET/CT when the patient presented for post Ewing sarcoma treatment follow-up. The different types of melorheostosis as well as the challenge of diagnosing this rare entity are discussed in this report.
Uploads
Papers by Samar Muwakkit