A 15 years old boy diagnosed as acute myeloid leukemia (AML) with Myelodysplastic syndrome (MDS) ... more A 15 years old boy diagnosed as acute myeloid leukemia (AML) with Myelodysplastic syndrome (MDS) related disorder with history of epistaxis since childhood. The only significant family history of epistaxis of his grandmother only. He presented as pancytopenia in ER with prolonged bleeding from his nose. No family history of malignancy. Additional genetic analysis was performed and identified two heterozygous different gene variant, one is RUNX1 gene for rare germline variant designated c.611G>A, which is predicted to result in the amino acid substitution p. Arg204Gln, another is heterozygous in the GATA2 gene for a variant designated c.554_628delins 16, which is predicted to result in frameshift and premature protein termination (p.Pro185Leufs*77). To our knowledge this variant has not been reported previously in any patient with autosomal dominant GATA2-related disorder but expected to be pathogenic. Germline GATA2 mutations are involved in a group of complex syndromes with overlapping clinical features of immune deficiency, lymphedema and propensity to acute myeloid leukemia or myelodysplastic syndrome (AML-MDS). This case illustrates the importance of recognizing the clinical features for this rare category of AML-MDS and performing the appropriate molecular testing. The diagnosis of heritable gene mutations associated familial AML-MDS has significant clinical implication for the patients and affected families.
Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation, Dec 22, 2016
Fanconi anemia is a congenital bone marrow failure syndrome that is associated with congenital an... more Fanconi anemia is a congenital bone marrow failure syndrome that is associated with congenital anomalies and increased risk of cancer. Hematopoietic stem cell transplant is a potentially curative modality for bone marrow failure in Fanconi anemia patients. Here, we report our center's experience regarding adolescent and young adult patients with Fanconi anemia and hematopoietic stem cell transplant. We conducted a retrospective patient record analyses of patients who presented at our center from 1988 to 2014. We included patients greater than 14 years old with confirmed Fanconi anemia based on positive chromosome breakage study and who underwent hematopoietic stem cell transplant at our institution. Our study group comprised 12 patients with Fanconi anemia who underwent hematopoietic stem cell transplant at our institution. The median age was 20 years (range, 14-31 y) with a female predominance of 83%. Low-dose cyclophosphamide (20-80 mg/kg)-based conditioning regimens were used...
Background A significant proportion of patients with acute myeloid leukemia (AML) will either be ... more Background A significant proportion of patients with acute myeloid leukemia (AML) will either be refractory to initial chemotherapy or will suffer refractory relapse. The role of allogeneic transplantation (SCT) in active disease is contentious. There is a growing body of literature that sequential chemotherapy, pioneered by the German FLAMSA regimen, followed by RIC SCT is a safe and efficacious modality in these patients, and there have been numerous modifications of this regimen, especially as amsacrineis not widely available. Fludarabine, cytarabineand and etoposide (VP16) (FLAV) have been reported as an effective salvage regimen. Here we report on single center outcomes of a variation of the FLAMSA regimen, substituting amsacrine for etoposide with mainly myeloablative conditioning. Methods Patients were offered this regimen if fit for allogenic HSCT and had AML which is refractory to two cycles of chemotherapy or refractory to one cycle and considered at high risk for complica...
Levofloxacin is one of the most commonly prescribed antibiotics for both inpatient and outpatient... more Levofloxacin is one of the most commonly prescribed antibiotics for both inpatient and outpatient care of respiratory tract infection. It is generally well tolerated, and it has an excellent safety profile. We report a case of severe acute liver toxicity that apparently complicated intravenous administration of levofloxacin, which resolved after discontinuation of the drug.
Lung volume reduction surgery (LVRS) has recently been introduced as a palliative treatment for p... more Lung volume reduction surgery (LVRS) has recently been introduced as a palliative treatment for patients with severe emphysema. The most common postoperative complication is persistent air leak requiring prolonged tube thoracostomy. We describe a unique case of a patient with severe emphysema who underwent LVRS and presented, about a year later, with the repeated expectoration of surgical staples.
Background: Inhaled medications are the cornerstone of asthma therapy. Metered dose inhaler techn... more Background: Inhaled medications are the cornerstone of asthma therapy. Metered dose inhaler technique is a widely used technique to administer medications like corticosteroids. Meanwhile, the health professionals and patients knowledge and practice towards this metered dose inhaler is quite deficient but arguably understood by policy makers or education expertise. Objective: This study tried to assess the pharmacists and druggists competency on MDI who are the professionals at the front line to demonstrate and teach the technique for patients. Method: A cross sectional study was conducted among registered pharmacists and druggists from different public and private pharmacies and drug stores in Mekelle Town, Ethiopia from March to June, 2013. Evaluation tool was adapted from the National Asthma Education and Prevention Programmes of America (NAEPP) step criteria for the administration of a metered dose inhaler to score the knowledge/proficiency of use of MDIs by the subjects using two evaluators. Result: The mean score given by evaluators was 4.34 and 4.28 by evaluator I and II respectively. Of the 106 professionals took part in this research, based on the competency on essential steps for optimum therapeutic value of MDI, only 2 (1.9%) and 1 (0.9%) study participants had adequate competency in metered dose inhaler according to evaluator I and evaluator II respectively. The rest, irrespective of their age, sex, educational status and experience, did not achieve adequate score on MDI technique. Of the essential steps, only 25 (23.6%) and 16 (15.1%) participants breathed in and actuating the canister together according to evaluators I and II respectively. Conclusion: Very poor MDI technique was very common in this sample of healthcare providers. Despite involvement of all participants in patient counselling on inhalers, none of them were able to perform all steps correctly, which shows that patient may not have adequate instruction.
Thirty-eight patients who met the diagnostic criteria for severe aplastic anemia underwent alloge... more Thirty-eight patients who met the diagnostic criteria for severe aplastic anemia underwent allogeneic hematopoietic stem cell transplantation (HSCT). The median patient age was 20 years (range, 14-36 years). Twenty-four patients were treatment-naïve, 11 had failed one or more previous courses of immunosuppressive therapy, and 3 had failed a previous HSCT. The conditioning regimen included fludarabine 30 mg/m 2 /day for 3 days (days 29, 28, and 27) and cyclophosphamide 50 mg/kg/day for 4 days (days 25, 24, 23, and 22). Graft-versus-host disease (GVHD) prophylaxis consisted of cyclosporine and short-course methotrexate. All patients underwent transplantation with unmanipulated bone marrow as the stem cell source. The median total nucleated cell (TNC) dose was 2.43 Â 10 8 /kg (range, 0.60-6.7 Â 10 8 / kg). The conditioning regimen was well tolerated, with minimal treatment-related mortality. Engraftment was observed in all patients after transplantation; the median time to engraftment of neutrophils and platelets was 18 and 23 days, respectively. Twenty-five of the 27 patients with available chimeric studies at day 180 maintained donor chimerism. Acute GVHD grade $II was diagnosed in 4 patients (11%). Extensive chronic GVHD was observed in 8 patients (25%) who survived beyond day 1100, at a median observation time of 43 months. Graft rejection with relapse of aplais was observed in one patient. The overall survival (OS) for the whole group was 79%. A trend toward improved OS was observed in the treatment-naïve patients (83% vs 71%), but this was statistically insignificant (P 5 .384). The fludarabine-based conditioning regimen used in this study with relatively young cohort of patients was well tolerated, with a low rate of rejection and treatment outcomes comparable to those seen in other, more intense and potentially more toxic conditioning regimens. Our results await validation in a larger study, optimally in a randomized controlled manner.
Thrombocytopenia is a common complication in human immunodeficiency virus (HIV)-infected hemophil... more Thrombocytopenia is a common complication in human immunodeficiency virus (HIV)-infected hemophiliacs. The etiology is multifactorial and a majority of the patients with hemophilia exhibit a decreased platelet count within 10 years of seroconversion. Thrombocytopenia in these patients is associated with a high risk of bleeding and death. Thrombotic microangiopathy causing thrombocytopenia in HIV-infected hemophiliacs is extremely rare. We describe an HIV-infected hemophilic patient who presented with bleeding, renal insufficiency, and thrombocytopenia. Platelet transfusion resulted in deterioration of clinical condition. Examination of blood smears demonstrated a microangiopathic process. The patient responded well to plasmapheresis with normalization of platelet and renal function. Thrombotic thrombocytopenic purpura should be suspected in HIV-infected hemophiliacs who present with a new onset of thrombocytopenia and anemia as delay in treatment may result in fatal sequelae.
Secondary acute myeloid leukemia (sAML) includes AML as a complication of an antecedent hematolog... more Secondary acute myeloid leukemia (sAML) includes AML as a complication of an antecedent hematological disorder or a therapy‐related AML. Large registry‐based data identified sAML as an independent poor‐outcome type of AML post allogeneic hematopoietic cell transplantation (allo‐HCT). In our study, we tried to define factors affecting outcomes of sAML post allo‐HCT, and identify patients with sAML who may truly benefit from allo‐HCT. We retrospectively analyzed the data of 64 patients aged (14‐61 years) with sAML who received allo‐HCT between September 2010 and February 2018 at our institute. Most of the patients were transplanted from matched related donors (MRD; 54, 84.4%). Our results showed that poor‐risk cytogenetics were identified in 31 patients (48.4%), and their presence was an indicator of poor overall survival (OS) and disease‐free survival (DFS; P‐value = .009, and .004, respectively). The cumulative incidence of chronic graft‐versus‐host disease (cGVHD) was significantly...
Background: The coronavirus disease‐2019 (COVID‐19) caused by SARS Coronavirus 2 (SARS‐CoV‐2) is ... more Background: The coronavirus disease‐2019 (COVID‐19) caused by SARS Coronavirus 2 (SARS‐CoV‐2) is a potentially lethal infection. Cancer patients, and specifically hematopoietic cell transplant (HCT) recipients are severely immunocompromised and may be at a higher risk of a complicated course with this infection. We aimed to study the COVID-19 outcomes and severity in post HCT patients. Methods: We retrospectively reviewed post-HCT patients diagnosed with COVID-19 between March 15, 2020, and December 1, 2020 at 10 transplant centers across the Middle East. Results: We identified 91 patients with confirmed SARS-CoV-2 infection across 10 transplant centers. 52 patients were post allo-HCT while the remaining 39 patients were post auto-HCT. The median time from transplant was 14.9 months. Mortality rate was 4.4%. Hospital admission rate was 53%. ICU admission rate was 14%. Mechanical ventilation rate was 10%. Oxygen supplementation rate was 18%. Time from HCT to COVID-19 > 6 months w...
ethemoglobinemia results from the oxidation of ferrous iron to ferric iron within the hemoglobin ... more ethemoglobinemia results from the oxidation of ferrous iron to ferric iron within the hemoglobin molecule (1). Although there are many causes of this disorder, use of the local anesthetics benzocaine and prilocaine have been described as a rare cause of methemoglobinemia (2-4). We describe three cases of methemoglobinemia apparently due to topically applied lidocaine used before endoscopy. CASE 1 A 26-year-old woman with a history of human immunodeficiency virus infection presented with intermittent fever, cough, and dyspnea, which had persisted for several weeks. Chest roentgenography showed bilateral infiltrates. She was started on trimethoprim-sulfamethoxazole for presumed Pneumocystis carinii pneumonia. She was not taking antiretroviral medications. Bronchoscopy was performed, during which she received 10 cc of 2% lidocaine solution (Baxter Health Care Corporation, Glendale, CA) instilled into the trachea, two sprays of 2% lidocaine solution to the throat, and 2 cc of 2% lidocaine jelly (Astra USA, Westborough, MA) to each nostril. She also received 5 mg intravenous diazepam, 75 mg meperidine, and 0.6 mg atropine intramuscularly. After the procedure, she developed worsening dyspnea and cyanosis. On 100% oxygen, her arterial oxygen tension was 398 mm Hg, her oxygen saturation was 85%, and her methemoglobin level was 14%. Trimethoprim-sulfamethoxazole was discontinued, and the patient was observed in the intensive care unit. The next day, the patient's clinical condition improved. She was no longer in respiratory distress, and the cyanosis resolved (Table 1). The bronchoalveolar lavage did not reveal any opportunistic infection. She was subsequently discharged.
Introduction: The use of a Myeloablative (MA) regimen followed bypost-transplantation high dose C... more Introduction: The use of a Myeloablative (MA) regimen followed bypost-transplantation high dose Cyclophosphamide (PT-CY) has been adopted to overcome the increased relapse risk following nonmyeloablative conditioning regimen and unmanipulated Haploidentical Bone Marrow Transplantation, in patients with high-risk hematological malignancies, with acceptable TRM and risk of GVHD. We added ATG to our Myeloablative regimen with PT-CY after noticing significant incidence of grade II-IV aGVHD with the first few cases. Here we compare the outcomes of the 12 patients who received ATG to the outcomes of the first 11 patients who were transplanted without ATG. Patients and Methods: our haploidentical program was started in 2013 as a phase I/II prospective clinical trial. After reviewing the first 11 cases enrolled on the trial (MA regimen with PT-CY, without ATG), we noticed significant incidence of high grade aGVHD (54.55%). We amended our protocol in early 2015. Rabbit ATG was added at a dos...
Introduction: Fanconi Anemia (FA) is an autosomal recessive disorder characterized by bone marrow... more Introduction: Fanconi Anemia (FA) is an autosomal recessive disorder characterized by bone marrow failure (BMF), constitutional anomalies and high risk of developing cancer. Distinguishing FA from severe aplastic anemia (SAA) can be challenging especially in asyndromic patients. We undertook a clinical and laboratory cohort study of adolescent and young adult (AYA) patients with a diagnosis of FA treated at our institution to characterize the clinical features in our population, and conducted a prospective translational study to explore integration of a genomic and proteomic approach for improved diagnosis and molecular characterization of FA. Methods: Data on FA patients was obtained from an institutionally approved BMF database and hematopoietic stem cell transplant (HSCT) database. Further data was obtained from a register of chromosomal breakage (CB) analysis results. Index cases were identified if they were older than 14 years of age at the time of diagnosis or under the care o...
Introduction: Induction with 3+7 has been standard practice in acute myeloid leukemia (AML) for o... more Introduction: Induction with 3+7 has been standard practice in acute myeloid leukemia (AML) for over 40 years. Addition of a 3rd agent or use of high dose Ara-C has been reported to show better CR rates, but this has not demonstrated a consistent improvement in overall survival. We have consistently used induction with idarubicin, cytarabine 100mg/m2 /day for 7 days and etoposide at 100mg/m2 in young patients, omitting etoposide for suspected secondary AML or AML with myelodysplasia related features, or in those in whom excess toxicity is suspected. Methods: All patients wholly treated for newly diagnosed AML were identified from the prospective institutional AML database. Patients were treated with either ICE (Idarubicin 12mg/m2 day 1-3, Ara-C 100mg/m2 day 1-7, Etoposide 100mg/m2 d1-3) or the same doses of idarubicin and Ara-C without etoposide (3+7). The latter was given in patients with dysplastic features or secondary AML or where there were concerns of toxicity. Results: We Ide...
A Significant Proportion of Young Adult Patients with Post -HCT Relapse Of AML Benefit From Aggre... more A Significant Proportion of Young Adult Patients with Post -HCT Relapse Of AML Benefit From Aggressive Salvage And 2nd Cellular Therapy. INTRODUCTION: There is currently no standard of care for patients with AML who relapse following hematopoietic cell transplantation (HCT), and outcomes in these patients are generally poor. Given this fact, there is great variability in practice, and many patients may be palliated in the absence of suitable clinical trials, especially following early relapses. We sought to analyse long-term survival of young adults with AML based on whether or not they received a second cellular therapy (CT) (second transplant or donor lymphocyte infusion [DLI]) following post-HCT relapse. METHODS: We retrospectively analysed data on patients who had received a HCT between 2000 and 2012 and had a post HCT relapse. The patients were stratified by whether or not they had 2nd CT with or without prior chemotherapy. Baseline characteristics and outcomes were compared. R...
4894 Background: Pretreatment karyotypes of leukemic cells provide the core for risk-stratificati... more 4894 Background: Pretreatment karyotypes of leukemic cells provide the core for risk-stratification schemes in acute myeloid leukemia (AML) and cytogenetic abnormalities are well established as the strongest prognostic factor for response to therapy and for survival in AML patients. Several cytogenetic risk classifications have been built to stratify AML patients. Only the recent European Leukemia Net (ELN) recommendations by an international panel proposed a new standardized cytogenetic reporting system for AML. More importantly, few studies attempted so far to validate the ELN requirements for risk assessment in AML patients treated with allogeneic hematopoietic stem cell transplantation (HSCT). We investigated whether our series of adult AML patients treated with HSCT could validate the ELN classification criteria. We also assessed the prognostic value of this new risk stratification scheme. Methods: ELN cytogenetic reporting criteria were applied to a series of 110 adult AML pat...
A 15 years old boy diagnosed as acute myeloid leukemia (AML) with Myelodysplastic syndrome (MDS) ... more A 15 years old boy diagnosed as acute myeloid leukemia (AML) with Myelodysplastic syndrome (MDS) related disorder with history of epistaxis since childhood. The only significant family history of epistaxis of his grandmother only. He presented as pancytopenia in ER with prolonged bleeding from his nose. No family history of malignancy. Additional genetic analysis was performed and identified two heterozygous different gene variant, one is RUNX1 gene for rare germline variant designated c.611G>A, which is predicted to result in the amino acid substitution p. Arg204Gln, another is heterozygous in the GATA2 gene for a variant designated c.554_628delins 16, which is predicted to result in frameshift and premature protein termination (p.Pro185Leufs*77). To our knowledge this variant has not been reported previously in any patient with autosomal dominant GATA2-related disorder but expected to be pathogenic. Germline GATA2 mutations are involved in a group of complex syndromes with overlapping clinical features of immune deficiency, lymphedema and propensity to acute myeloid leukemia or myelodysplastic syndrome (AML-MDS). This case illustrates the importance of recognizing the clinical features for this rare category of AML-MDS and performing the appropriate molecular testing. The diagnosis of heritable gene mutations associated familial AML-MDS has significant clinical implication for the patients and affected families.
Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation, Dec 22, 2016
Fanconi anemia is a congenital bone marrow failure syndrome that is associated with congenital an... more Fanconi anemia is a congenital bone marrow failure syndrome that is associated with congenital anomalies and increased risk of cancer. Hematopoietic stem cell transplant is a potentially curative modality for bone marrow failure in Fanconi anemia patients. Here, we report our center's experience regarding adolescent and young adult patients with Fanconi anemia and hematopoietic stem cell transplant. We conducted a retrospective patient record analyses of patients who presented at our center from 1988 to 2014. We included patients greater than 14 years old with confirmed Fanconi anemia based on positive chromosome breakage study and who underwent hematopoietic stem cell transplant at our institution. Our study group comprised 12 patients with Fanconi anemia who underwent hematopoietic stem cell transplant at our institution. The median age was 20 years (range, 14-31 y) with a female predominance of 83%. Low-dose cyclophosphamide (20-80 mg/kg)-based conditioning regimens were used...
Background A significant proportion of patients with acute myeloid leukemia (AML) will either be ... more Background A significant proportion of patients with acute myeloid leukemia (AML) will either be refractory to initial chemotherapy or will suffer refractory relapse. The role of allogeneic transplantation (SCT) in active disease is contentious. There is a growing body of literature that sequential chemotherapy, pioneered by the German FLAMSA regimen, followed by RIC SCT is a safe and efficacious modality in these patients, and there have been numerous modifications of this regimen, especially as amsacrineis not widely available. Fludarabine, cytarabineand and etoposide (VP16) (FLAV) have been reported as an effective salvage regimen. Here we report on single center outcomes of a variation of the FLAMSA regimen, substituting amsacrine for etoposide with mainly myeloablative conditioning. Methods Patients were offered this regimen if fit for allogenic HSCT and had AML which is refractory to two cycles of chemotherapy or refractory to one cycle and considered at high risk for complica...
Levofloxacin is one of the most commonly prescribed antibiotics for both inpatient and outpatient... more Levofloxacin is one of the most commonly prescribed antibiotics for both inpatient and outpatient care of respiratory tract infection. It is generally well tolerated, and it has an excellent safety profile. We report a case of severe acute liver toxicity that apparently complicated intravenous administration of levofloxacin, which resolved after discontinuation of the drug.
Lung volume reduction surgery (LVRS) has recently been introduced as a palliative treatment for p... more Lung volume reduction surgery (LVRS) has recently been introduced as a palliative treatment for patients with severe emphysema. The most common postoperative complication is persistent air leak requiring prolonged tube thoracostomy. We describe a unique case of a patient with severe emphysema who underwent LVRS and presented, about a year later, with the repeated expectoration of surgical staples.
Background: Inhaled medications are the cornerstone of asthma therapy. Metered dose inhaler techn... more Background: Inhaled medications are the cornerstone of asthma therapy. Metered dose inhaler technique is a widely used technique to administer medications like corticosteroids. Meanwhile, the health professionals and patients knowledge and practice towards this metered dose inhaler is quite deficient but arguably understood by policy makers or education expertise. Objective: This study tried to assess the pharmacists and druggists competency on MDI who are the professionals at the front line to demonstrate and teach the technique for patients. Method: A cross sectional study was conducted among registered pharmacists and druggists from different public and private pharmacies and drug stores in Mekelle Town, Ethiopia from March to June, 2013. Evaluation tool was adapted from the National Asthma Education and Prevention Programmes of America (NAEPP) step criteria for the administration of a metered dose inhaler to score the knowledge/proficiency of use of MDIs by the subjects using two evaluators. Result: The mean score given by evaluators was 4.34 and 4.28 by evaluator I and II respectively. Of the 106 professionals took part in this research, based on the competency on essential steps for optimum therapeutic value of MDI, only 2 (1.9%) and 1 (0.9%) study participants had adequate competency in metered dose inhaler according to evaluator I and evaluator II respectively. The rest, irrespective of their age, sex, educational status and experience, did not achieve adequate score on MDI technique. Of the essential steps, only 25 (23.6%) and 16 (15.1%) participants breathed in and actuating the canister together according to evaluators I and II respectively. Conclusion: Very poor MDI technique was very common in this sample of healthcare providers. Despite involvement of all participants in patient counselling on inhalers, none of them were able to perform all steps correctly, which shows that patient may not have adequate instruction.
Thirty-eight patients who met the diagnostic criteria for severe aplastic anemia underwent alloge... more Thirty-eight patients who met the diagnostic criteria for severe aplastic anemia underwent allogeneic hematopoietic stem cell transplantation (HSCT). The median patient age was 20 years (range, 14-36 years). Twenty-four patients were treatment-naïve, 11 had failed one or more previous courses of immunosuppressive therapy, and 3 had failed a previous HSCT. The conditioning regimen included fludarabine 30 mg/m 2 /day for 3 days (days 29, 28, and 27) and cyclophosphamide 50 mg/kg/day for 4 days (days 25, 24, 23, and 22). Graft-versus-host disease (GVHD) prophylaxis consisted of cyclosporine and short-course methotrexate. All patients underwent transplantation with unmanipulated bone marrow as the stem cell source. The median total nucleated cell (TNC) dose was 2.43 Â 10 8 /kg (range, 0.60-6.7 Â 10 8 / kg). The conditioning regimen was well tolerated, with minimal treatment-related mortality. Engraftment was observed in all patients after transplantation; the median time to engraftment of neutrophils and platelets was 18 and 23 days, respectively. Twenty-five of the 27 patients with available chimeric studies at day 180 maintained donor chimerism. Acute GVHD grade $II was diagnosed in 4 patients (11%). Extensive chronic GVHD was observed in 8 patients (25%) who survived beyond day 1100, at a median observation time of 43 months. Graft rejection with relapse of aplais was observed in one patient. The overall survival (OS) for the whole group was 79%. A trend toward improved OS was observed in the treatment-naïve patients (83% vs 71%), but this was statistically insignificant (P 5 .384). The fludarabine-based conditioning regimen used in this study with relatively young cohort of patients was well tolerated, with a low rate of rejection and treatment outcomes comparable to those seen in other, more intense and potentially more toxic conditioning regimens. Our results await validation in a larger study, optimally in a randomized controlled manner.
Thrombocytopenia is a common complication in human immunodeficiency virus (HIV)-infected hemophil... more Thrombocytopenia is a common complication in human immunodeficiency virus (HIV)-infected hemophiliacs. The etiology is multifactorial and a majority of the patients with hemophilia exhibit a decreased platelet count within 10 years of seroconversion. Thrombocytopenia in these patients is associated with a high risk of bleeding and death. Thrombotic microangiopathy causing thrombocytopenia in HIV-infected hemophiliacs is extremely rare. We describe an HIV-infected hemophilic patient who presented with bleeding, renal insufficiency, and thrombocytopenia. Platelet transfusion resulted in deterioration of clinical condition. Examination of blood smears demonstrated a microangiopathic process. The patient responded well to plasmapheresis with normalization of platelet and renal function. Thrombotic thrombocytopenic purpura should be suspected in HIV-infected hemophiliacs who present with a new onset of thrombocytopenia and anemia as delay in treatment may result in fatal sequelae.
Secondary acute myeloid leukemia (sAML) includes AML as a complication of an antecedent hematolog... more Secondary acute myeloid leukemia (sAML) includes AML as a complication of an antecedent hematological disorder or a therapy‐related AML. Large registry‐based data identified sAML as an independent poor‐outcome type of AML post allogeneic hematopoietic cell transplantation (allo‐HCT). In our study, we tried to define factors affecting outcomes of sAML post allo‐HCT, and identify patients with sAML who may truly benefit from allo‐HCT. We retrospectively analyzed the data of 64 patients aged (14‐61 years) with sAML who received allo‐HCT between September 2010 and February 2018 at our institute. Most of the patients were transplanted from matched related donors (MRD; 54, 84.4%). Our results showed that poor‐risk cytogenetics were identified in 31 patients (48.4%), and their presence was an indicator of poor overall survival (OS) and disease‐free survival (DFS; P‐value = .009, and .004, respectively). The cumulative incidence of chronic graft‐versus‐host disease (cGVHD) was significantly...
Background: The coronavirus disease‐2019 (COVID‐19) caused by SARS Coronavirus 2 (SARS‐CoV‐2) is ... more Background: The coronavirus disease‐2019 (COVID‐19) caused by SARS Coronavirus 2 (SARS‐CoV‐2) is a potentially lethal infection. Cancer patients, and specifically hematopoietic cell transplant (HCT) recipients are severely immunocompromised and may be at a higher risk of a complicated course with this infection. We aimed to study the COVID-19 outcomes and severity in post HCT patients. Methods: We retrospectively reviewed post-HCT patients diagnosed with COVID-19 between March 15, 2020, and December 1, 2020 at 10 transplant centers across the Middle East. Results: We identified 91 patients with confirmed SARS-CoV-2 infection across 10 transplant centers. 52 patients were post allo-HCT while the remaining 39 patients were post auto-HCT. The median time from transplant was 14.9 months. Mortality rate was 4.4%. Hospital admission rate was 53%. ICU admission rate was 14%. Mechanical ventilation rate was 10%. Oxygen supplementation rate was 18%. Time from HCT to COVID-19 > 6 months w...
ethemoglobinemia results from the oxidation of ferrous iron to ferric iron within the hemoglobin ... more ethemoglobinemia results from the oxidation of ferrous iron to ferric iron within the hemoglobin molecule (1). Although there are many causes of this disorder, use of the local anesthetics benzocaine and prilocaine have been described as a rare cause of methemoglobinemia (2-4). We describe three cases of methemoglobinemia apparently due to topically applied lidocaine used before endoscopy. CASE 1 A 26-year-old woman with a history of human immunodeficiency virus infection presented with intermittent fever, cough, and dyspnea, which had persisted for several weeks. Chest roentgenography showed bilateral infiltrates. She was started on trimethoprim-sulfamethoxazole for presumed Pneumocystis carinii pneumonia. She was not taking antiretroviral medications. Bronchoscopy was performed, during which she received 10 cc of 2% lidocaine solution (Baxter Health Care Corporation, Glendale, CA) instilled into the trachea, two sprays of 2% lidocaine solution to the throat, and 2 cc of 2% lidocaine jelly (Astra USA, Westborough, MA) to each nostril. She also received 5 mg intravenous diazepam, 75 mg meperidine, and 0.6 mg atropine intramuscularly. After the procedure, she developed worsening dyspnea and cyanosis. On 100% oxygen, her arterial oxygen tension was 398 mm Hg, her oxygen saturation was 85%, and her methemoglobin level was 14%. Trimethoprim-sulfamethoxazole was discontinued, and the patient was observed in the intensive care unit. The next day, the patient's clinical condition improved. She was no longer in respiratory distress, and the cyanosis resolved (Table 1). The bronchoalveolar lavage did not reveal any opportunistic infection. She was subsequently discharged.
Introduction: The use of a Myeloablative (MA) regimen followed bypost-transplantation high dose C... more Introduction: The use of a Myeloablative (MA) regimen followed bypost-transplantation high dose Cyclophosphamide (PT-CY) has been adopted to overcome the increased relapse risk following nonmyeloablative conditioning regimen and unmanipulated Haploidentical Bone Marrow Transplantation, in patients with high-risk hematological malignancies, with acceptable TRM and risk of GVHD. We added ATG to our Myeloablative regimen with PT-CY after noticing significant incidence of grade II-IV aGVHD with the first few cases. Here we compare the outcomes of the 12 patients who received ATG to the outcomes of the first 11 patients who were transplanted without ATG. Patients and Methods: our haploidentical program was started in 2013 as a phase I/II prospective clinical trial. After reviewing the first 11 cases enrolled on the trial (MA regimen with PT-CY, without ATG), we noticed significant incidence of high grade aGVHD (54.55%). We amended our protocol in early 2015. Rabbit ATG was added at a dos...
Introduction: Fanconi Anemia (FA) is an autosomal recessive disorder characterized by bone marrow... more Introduction: Fanconi Anemia (FA) is an autosomal recessive disorder characterized by bone marrow failure (BMF), constitutional anomalies and high risk of developing cancer. Distinguishing FA from severe aplastic anemia (SAA) can be challenging especially in asyndromic patients. We undertook a clinical and laboratory cohort study of adolescent and young adult (AYA) patients with a diagnosis of FA treated at our institution to characterize the clinical features in our population, and conducted a prospective translational study to explore integration of a genomic and proteomic approach for improved diagnosis and molecular characterization of FA. Methods: Data on FA patients was obtained from an institutionally approved BMF database and hematopoietic stem cell transplant (HSCT) database. Further data was obtained from a register of chromosomal breakage (CB) analysis results. Index cases were identified if they were older than 14 years of age at the time of diagnosis or under the care o...
Introduction: Induction with 3+7 has been standard practice in acute myeloid leukemia (AML) for o... more Introduction: Induction with 3+7 has been standard practice in acute myeloid leukemia (AML) for over 40 years. Addition of a 3rd agent or use of high dose Ara-C has been reported to show better CR rates, but this has not demonstrated a consistent improvement in overall survival. We have consistently used induction with idarubicin, cytarabine 100mg/m2 /day for 7 days and etoposide at 100mg/m2 in young patients, omitting etoposide for suspected secondary AML or AML with myelodysplasia related features, or in those in whom excess toxicity is suspected. Methods: All patients wholly treated for newly diagnosed AML were identified from the prospective institutional AML database. Patients were treated with either ICE (Idarubicin 12mg/m2 day 1-3, Ara-C 100mg/m2 day 1-7, Etoposide 100mg/m2 d1-3) or the same doses of idarubicin and Ara-C without etoposide (3+7). The latter was given in patients with dysplastic features or secondary AML or where there were concerns of toxicity. Results: We Ide...
A Significant Proportion of Young Adult Patients with Post -HCT Relapse Of AML Benefit From Aggre... more A Significant Proportion of Young Adult Patients with Post -HCT Relapse Of AML Benefit From Aggressive Salvage And 2nd Cellular Therapy. INTRODUCTION: There is currently no standard of care for patients with AML who relapse following hematopoietic cell transplantation (HCT), and outcomes in these patients are generally poor. Given this fact, there is great variability in practice, and many patients may be palliated in the absence of suitable clinical trials, especially following early relapses. We sought to analyse long-term survival of young adults with AML based on whether or not they received a second cellular therapy (CT) (second transplant or donor lymphocyte infusion [DLI]) following post-HCT relapse. METHODS: We retrospectively analysed data on patients who had received a HCT between 2000 and 2012 and had a post HCT relapse. The patients were stratified by whether or not they had 2nd CT with or without prior chemotherapy. Baseline characteristics and outcomes were compared. R...
4894 Background: Pretreatment karyotypes of leukemic cells provide the core for risk-stratificati... more 4894 Background: Pretreatment karyotypes of leukemic cells provide the core for risk-stratification schemes in acute myeloid leukemia (AML) and cytogenetic abnormalities are well established as the strongest prognostic factor for response to therapy and for survival in AML patients. Several cytogenetic risk classifications have been built to stratify AML patients. Only the recent European Leukemia Net (ELN) recommendations by an international panel proposed a new standardized cytogenetic reporting system for AML. More importantly, few studies attempted so far to validate the ELN requirements for risk assessment in AML patients treated with allogeneic hematopoietic stem cell transplantation (HSCT). We investigated whether our series of adult AML patients treated with HSCT could validate the ELN classification criteria. We also assessed the prognostic value of this new risk stratification scheme. Methods: ELN cytogenetic reporting criteria were applied to a series of 110 adult AML pat...
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Papers by Shad Ahmed