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Article ; Online: Skin Lesions as a Presenting Feature of Dimorphic T-Lymphoblastic Leukemia in an Adolescent Male.

Chapman, Stacy / Almiski, Muhamad / Marko, Daniel / Sekiguchi, Debora / Dawson, A J / Keijzer, Richard / Fromm, Jonathan / Israels, Sara J

Journal of pediatric hematology/oncology

2023 Volume 45, Issue 4, Page(s) 209–212

Abstract: Cutaneous involvement is rare in acute lymphoblastic leukemia/lymphoma, particularly within the T-cell lineage. Review of the literature for cutaneous involvement in T-cell lymphoblastic lymphoma/leukemia identifies mostly case reports, with the majority ...

Abstract	Cutaneous involvement is rare in acute lymphoblastic leukemia/lymphoma, particularly within the T-cell lineage. Review of the literature for cutaneous involvement in T-cell lymphoblastic lymphoma/leukemia identifies mostly case reports, with the majority of cases involving adults. We describe an adolescent male presenting with cervical lymphadenopathy and skin lesions leading to a diagnosis of early T-cell precursor lymphoblastic leukemia. Unique to this case is the age of the patient, presence of a dimorphic blast population, and the skin lesions preceding other signs of disease by at least 1 month.
MeSH term(s)	Adult ; Humans ; Male ; Adolescent ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/pathology ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/pathology ; Skin Diseases ; Skin Neoplasms/pathology ; Lymphoma, T-Cell
Language	English
Publishing date	2023-03-16
Publishing country	United States
Document type	Review ; Journal Article
ZDB-ID	1231152-2
ISSN	1536-3678 ; 1077-4114 ; 0192-8562
ISSN (online)	1536-3678
ISSN	1077-4114 ; 0192-8562
DOI	10.1097/MPH.0000000000002659
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Article ; Online: Acquired haemophilia A: A 15-year population-based review of incidence rate, patient demographics and treatment outcomes.

Tian, Chantal / Perija, Brittany / Kotb, Rami / Houston, Brett L / Israels, Sara J / Houston, Donald S / Rimmer, Emily / Zarychanski, Ryan

Haemophilia : the official journal of the World Federation of Hemophilia

2023 Volume 29, Issue 5, Page(s) 1269–1275

Abstract: Introduction: Acquired haemophilia A (AHA) is a rare bleeding disorder characterized by autoantibodies against coagulation factor VIII (FVIII). Estimates of AHA incidence are largely based on registry data, which may be prone to referral bias. ... ...

Abstract	Introduction: Acquired haemophilia A (AHA) is a rare bleeding disorder characterized by autoantibodies against coagulation factor VIII (FVIII). Estimates of AHA incidence are largely based on registry data, which may be prone to referral bias. Population-based studies can enhance our understanding of the epidemiology, presentation and outcomes of AHA. Methods: We conducted a retrospective, population-based cohort study of all AHA diagnosed and treated in Manitoba, Canada over a 15-year period. Using records from the sole provincial reference laboratory, we identified all patients with FVIII inhibitors who did not have congenital haemophilia. Using a piloted case report form, patient data was ascertained from hospital and bleeding disorder clinic records. Results: From 2006 to 2021, we identified 34 patients with AHA, corresponding to a population-based incidence rate of AHA of 1.78 cases per million per year. The median age at presentation was 76 years and most cases were idiopathic (79%). Almost all patients (97%) presented with bleeding, of which 58% were considered major bleeds and required haemostatic agents in 67%. Longstanding unexplained bleeding symptoms were commonly reported, suggesting delayed diagnosis. Immunosuppressive therapy (IST) was administered in 88% of patients. Remission was achieved in 79% of patients; median time to remission was 2.1 months. There were two deaths due to bleeding. No deaths due to IST were reported. Conclusion: The population-based incidence of AHA in Manitoba is 1.78 cases/million/year. Bleeding is common and can be life-threatening. AHA outcomes are encouraging with the use of haemostatic agents and IST. Serious treatment-associated morbidity and mortality is uncommon.
MeSH term(s)	Humans ; Hemophilia A/drug therapy ; Hemophilia A/epidemiology ; Incidence ; Cohort Studies ; Retrospective Studies ; Treatment Outcome
Language	English
Publishing date	2023-08-28
Publishing country	England
Document type	Journal Article
ZDB-ID	1229713-6
ISSN	1365-2516 ; 1351-8216 ; 1355-0691
ISSN (online)	1365-2516
ISSN	1351-8216 ; 1355-0691
DOI	10.1111/hae.14845
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Zs.A 4249: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (2.OG) ab Jg. 2022: Lesesaal (EG)
Zs.MO 450: Show issues

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Article ; Online: Mental Disorders Among Adolescents and Young Adults With Cancer: A Canadian Population-Based and Sibling Cohort Study.

Oberoi, Sapna / Garland, Allan / Yan, Adam P / Lambert, Pascal / Xue, Lin / Decker, Kathleen / Israels, Sara J / Banerji, Shantanu / Bolton, James M / Deleemans, Julie M / Garand-Sheridan, Bronwen / Louis, Deepak / Lix, Lisa M / Mahar, Alyson L

Journal of clinical oncology : official journal of the American Society of Clinical Oncology

2024 , Page(s) JCO2301615

Abstract: Purpose: To compare the cumulative incidence of mental disorders among adolescents and young adults (AYAs) diagnosed with cancer with the general population and their unaffected siblings.: Methods: A retrospective, population-based, matched cohort ... ...

Abstract	Purpose: To compare the cumulative incidence of mental disorders among adolescents and young adults (AYAs) diagnosed with cancer with the general population and their unaffected siblings. Methods: A retrospective, population-based, matched cohort design was used to investigate the impact of cancer diagnosis on mental disorders among individuals age 15-39 diagnosed between 1989 and 2019. Two cancer-free cohorts were identified: matched population-based and sibling cohorts. Outcomes included incidence of mood and anxiety disorders, substance use disorders, suicide outcomes, psychotic disorders, and any of the preceding four categories within 5 years of cancer diagnosis. Competing risk regression was used to estimate adjusted subhazard ratios (aSHR) and 95% CIs. Results: Among 3,818 AYAs with cancer matched to the population-based cancer-free cohort, individuals with cancer were more likely to be diagnosed with incident mental disorders than those without cancer; the risk was highest immediately after a cancer diagnosis and decreased over time with aSHR [95% CI] for mood and anxiety disorders at 0-6 months (11.27 [95% CI, 6.69 to 18.97]), 6-12 months (2.35 [95% CI, 1.54 to 3.58]), and 12-24 months (2.06 [95% CI, 1.55 to 2.75]); for substance use disorders at 0-6 months (2.73 [95% CI, 1.90 to 3.92]); for psychotic disorders at 0-6 months (4.69 [95% CI, 2.07 to 10.65]); and for any mental disorder at 0-6 months (4.46 [95% CI, 3.41 to 5.85]), 6-12 months (1.56 [95% CI, 1.14 to 2.14]), and 12-24 months (1.7 [95% CI, 1.36 to 2.13]) postcancer diagnosis. In sibling comparison, cancer diagnosis was associated with a higher incidence of mood and anxiety and any mental disorder during first 6 months of cancer diagnosis. Conclusion: AYAs with cancer experience a greater incidence of mental disorders after cancer diagnosis relative to population-based and sibling cohorts without cancer, primarily within first 2 years, underscoring the need to address mental health concerns during this period.
Language	English
Publishing date	2024-02-09
Publishing country	United States
Document type	Journal Article
ZDB-ID	604914-x
ISSN	1527-7755 ; 0732-183X
ISSN (online)	1527-7755
ISSN	0732-183X
DOI	10.1200/JCO.23.01615
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Zs.A 1847: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG)
Zs.MO 650: Show issues

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Article ; Online: Acute Myeloid Leukemia Presenting as a Posterior Fossa Tumor.

Vanan, Magimairajan Issai / Li, Jie / Bunge, Martin / Serletis, Demitre / Almiski, Muhamad / Israels, Sara J

Journal of pediatric hematology/oncology

2021 Volume 43, Issue 7, Page(s) 277–278

Abstract: Extramedullary leukemia in pediatric acute myeloid leukemia can manifest as a myeloid sarcoma (MS), also known as granulocytic sarcoma or chloroma, in a variety of sites, or as leukemic blasts in the cerebrospinal fluid. Isolated MS of the central ... ...

Abstract	Extramedullary leukemia in pediatric acute myeloid leukemia can manifest as a myeloid sarcoma (MS), also known as granulocytic sarcoma or chloroma, in a variety of sites, or as leukemic blasts in the cerebrospinal fluid. Isolated MS of the central nervous system is rare. We report a case of acute myeloid leukemia with central nervous system-MS presenting as a posterior fossa mass mimicking a primary intracranial tumor.
MeSH term(s)	Brain Neoplasms/complications ; Brain Neoplasms/diagnosis ; Child ; Diagnosis, Differential ; Humans ; Infratentorial Neoplasms/complications ; Infratentorial Neoplasms/diagnosis ; Leukemia, Myeloid, Acute/complications ; Leukemia, Myeloid, Acute/diagnosis ; Male ; Prognosis ; Sarcoma, Myeloid/complications ; Sarcoma, Myeloid/diagnosis
Language	English
Publishing date	2021-10-28
Publishing country	United States
Document type	Case Reports ; Journal Article
ZDB-ID	1231152-2
ISSN	1536-3678 ; 1077-4114 ; 0192-8562
ISSN (online)	1536-3678
ISSN	1077-4114 ; 0192-8562
DOI	10.1097/MPH.0000000000002269
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Zs.A 1537: Show issues

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Article ; Online: Precursor B-Cell Acute Lymphoblastic Leukemia With MYC and BCL2 Rearrangements Presenting as Extensive Extranodal Disease in an Adolescent.

Oberoi, Sapna / Dawson, Angie / Marko, Daniel / Almiski, Muhamad / Higgins, Rick / Israels, Sara J

Journal of pediatric hematology/oncology

2021 Volume 43, Issue 4, Page(s) e501–e504

Abstract: Combined rearrangements of MYC and BCL2 are rare in precursor B-cell acute lymphoblastic leukemia (B-ALL). A 14-year-old boy presented with swelling of the knee and face. Imaging revealed diffuse infiltration of lacrimal glands, parotid glands along with ...

Abstract	Combined rearrangements of MYC and BCL2 are rare in precursor B-cell acute lymphoblastic leukemia (B-ALL). A 14-year-old boy presented with swelling of the knee and face. Imaging revealed diffuse infiltration of lacrimal glands, parotid glands along with the extensive epidural disease. Morphology and immunophenotype of knee joint aspirate were consistent with precursor B-ALL. Fluorescent in situ hybridization identified rearrangements of MYC and BCL2 genes. The disease was refractory to intensive treatment. The patient died of progressive disease. Precursor B-ALL with combined MYC and BCL2 rearrangements is rare, characterized by an aggressive clinical course, and has an inadequate response to standard therapeutic approaches.
MeSH term(s)	Adolescent ; Disease Progression ; Gene Rearrangement ; Humans ; In Situ Hybridization, Fluorescence ; Male ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma/genetics ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma/pathology ; Proto-Oncogene Proteins c-bcl-2/genetics ; Proto-Oncogene Proteins c-myc/genetics
Chemical Substances	MYC protein, human ; Proto-Oncogene Proteins c-bcl-2 ; Proto-Oncogene Proteins c-myc
Language	English
Publishing date	2021-02-19
Publishing country	United States
Document type	Case Reports ; Journal Article
ZDB-ID	1231152-2
ISSN	1536-3678 ; 1077-4114 ; 0192-8562
ISSN (online)	1536-3678
ISSN	1077-4114 ; 0192-8562
DOI	10.1097/MPH.0000000000002051
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Article: Second malignant neoplasms within 5 years from first primary diagnosis in pediatric oncology patients in Canada: a population-based retrospective cohort study.

Ricci, Christina / Subburaj, Divya / Lim, Kate / Shukla, Neetu / Kaur, Jaskiran / Xie, Lin / Laverty, Meghan / Zakaria, Dianne / Pole, Jason / Pelland-Marcotte, Marie-Claude / Barber, Randy / Israels, Sara J / Tran, Thai-Hoa / Oberoi, Sapna / Renzi, Samuele / MacDonald, Tamara / Sung, Lillian / Kulkarni, Ketan

Frontiers in oncology

2024 Volume 14, Page(s) 1376652

Abstract: Introduction: From the advancement of treatment of pediatric cancer diagnosis, the five-year survival rate has increased significantly. However, the adverse consequence of improved survival rate is the second malignant neoplasm. Although previous ... ...

Abstract	Introduction: From the advancement of treatment of pediatric cancer diagnosis, the five-year survival rate has increased significantly. However, the adverse consequence of improved survival rate is the second malignant neoplasm. Although previous studies provided information on the incidence and risk of SMN in long term survivors of childhood cancer, there is still scarce information known for short term (< 5 years) prognosis. This study aims to assess the incidence, characteristics, management, and outcome of children who develop SMN malignancies within 5 years of diagnosis of their initial cancer. Method: This is a retrospective cohort study of early Second Malignant Neoplasms (SMN) in pediatric oncology patients. The Cancer in Young People - Canada (CYP-C) national pediatric cancer registry was used and reviewed pediatric patients diagnosed with their first cancer from 2000-2015. Results: A total of 20,272 pediatric patients with a diagnosis of a first malignancy were analyzed. Of them, 0.7% were diagnosed with a SMN within the first 5 years following their first cancer diagnosis. Development of a SMN impacted survival, shown by an inferior survival rate in the SMN cohort (79.1%) after three years compared to that of the non-SMN cohort (89.7%). Several possible risk factors have been identified in the study including the use of epipodophyllotoxins, exposure to radiation, and hematopoietic stem cell 169 transplant. Discussion: This is the first national study assessing the incidence, 170 characteristics, risk factors and outcome of early SMN in Canadian children 171 from age 0-15 from 2000-2015.
Language	English
Publishing date	2024-03-28
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2649216-7
ISSN	2234-943X
ISSN	2234-943X
DOI	10.3389/fonc.2024.1376652
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Article: Laboratory diagnosis of inherited platelet function disorders.

Rand, Margaret L / Reddy, Emily C / Israels, Sara J

Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis

2018 Volume 57, Issue 4, Page(s) 485–493

Abstract: Platelets respond to vessel wall injury by forming a primary hemostatic plug to arrest blood loss. Hemostatic plug formation is complex, and involves platelet adhesion to the subendothelium that results in platelet activation and ultimately, aggregation. ...

Abstract	Platelets respond to vessel wall injury by forming a primary hemostatic plug to arrest blood loss. Hemostatic plug formation is complex, and involves platelet adhesion to the subendothelium that results in platelet activation and ultimately, aggregation. If any of these processes are deficient, primary hemostasis is impaired. Inherited platelet function disorders (IPFDs) are a heterogeneous group of defects in these processes, with patients experiencing mainly mucocutaneous bleeding symptoms that can range from very mild to life threatening, depending on the specific disorder. Here, we review the approach to an initial patient assessment required to inform laboratory testing, and the frequently used clinical laboratory assays for diagnostic evaluation of IPFDs. Newer testing approaches that may improve laboratory diagnosis in the near future are described.
MeSH term(s)	Blood Platelets/metabolism ; Clinical Laboratory Techniques/methods ; Humans ; Platelet Function Tests/methods
Language	English
Publishing date	2018-07-24
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	2046795-3
ISSN	1878-1683 ; 1473-0502
ISSN (online)	1878-1683
ISSN	1473-0502
DOI	10.1016/j.transci.2018.07.009
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Zs.A 1739: Show issues

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Article ; Online: Diagnostic evaluation of platelet function disorders in neonates and children: an update.

Israels, Sara J

Seminars in thrombosis and hemostasis

2009 Volume 35, Issue 2, Page(s) 181–188

Abstract: Investigation of platelet function disorders in infants and small children requires the collaborative efforts of clinicians and clinical laboratories. A detailed personal, family, and medication history, and a search for additional clinical phenomena may ...

Abstract	Investigation of platelet function disorders in infants and small children requires the collaborative efforts of clinicians and clinical laboratories. A detailed personal, family, and medication history, and a search for additional clinical phenomena may help to direct diagnostic laboratory investigations. Testing for these disorders in young children presents several challenges: the requirement of relatively large volumes of blood, lack of standardization, and the absence of well-established age-specific reference ranges. Neonates show the most notable differences in platelet function compared to older children and adults; the decreased platelet activation responses persist for the first 2 to 4 weeks after delivery. Small studies of normal postneonatal pediatric populations have provided data on platelet function assays, including bleeding times, PFA-100 closure times, thromboelastography, aggregation, secretion, and flow cytometry. The majority of these studies, comparing normal children with adults, found only minor differences in platelet responses measured by these assays.
MeSH term(s)	Blood Coagulation Tests ; Blood Platelet Disorders/blood ; Blood Platelet Disorders/diagnosis ; Child, Preschool ; Humans ; Infant ; Infant, Newborn ; Platelet Function Tests
Language	English
Publishing date	2009-03
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	196901-8
ISSN	1098-9064 ; 0094-6176
ISSN (online)	1098-9064
ISSN	0094-6176
DOI	10.1055/s-0029-1220326
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Zs.A 1259: Show issues

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Article ; Online: What we have learned from inherited platelet disorders.

Israels, Sara J / Rand, Margaret L

Pediatric blood & cancer

2013 Volume 60 Suppl 1, Page(s) S2–7

Abstract: Identifying the molecular basis of inherited platelet disorders has contributed to our understanding of normal platelet physiology. Many of these conditions are rare, but close observation of clinical and laboratory phenotype, and subsequent ... ...

Abstract	Identifying the molecular basis of inherited platelet disorders has contributed to our understanding of normal platelet physiology. Many of these conditions are rare, but close observation of clinical and laboratory phenotype, and subsequent identification of the abnormal protein and mutated gene, have provided us with unique opportunities to examine specific aspects of platelet biogenesis and function. Phenotype-genotype association studies are providing a detailed understanding of the structure and function of platelet membrane receptors, the biogenesis and release of platelet granules, and the assembly of the cytoskeleton. Genetic polymorphisms contributing to decreased or increased platelet adhesion and activation may translate into increased clinical risks for bleeding or thrombosis. More recently, genome wide association studies have identified new genes contributing to the variation in normal platelet function.
MeSH term(s)	Blood Platelet Disorders/genetics ; Blood Platelet Disorders/physiopathology ; Blood Platelets/metabolism ; Blood Platelets/physiology ; Genetic Association Studies ; Genome-Wide Association Study ; Hemorrhage/genetics ; Hemostasis ; Humans ; Phenotype ; Platelet Adhesiveness/genetics ; Polymorphism, Genetic ; Thrombosis/genetics
Language	English
Publishing date	2013
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	2131448-2
ISSN	1545-5017 ; 1545-5009
ISSN (online)	1545-5017
ISSN	1545-5009
DOI	10.1002/pbc.24345
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Zs.A 1131: Show issues

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Article ; Online: Epidural Spinal Mass as the Presenting Feature of B-Acute Lymphoblastic Leukemia in a Young Child.

Chapman, Stacy / Li, Jie / Almiski, Muhamad / Moffat, Hayley / Israels, Sara J

Journal of pediatric hematology/oncology

2019 Volume 42, Issue 8, Page(s) e845–e847

Abstract: An isolated epidural mass is a rare presentation of childhood B-acute lymphoblastic leukemia with an estimated incidence of 0.4%. Of the cases reported in the literature, the majority involve adults presenting with spinal cord compression and/or systemic ...

Abstract	An isolated epidural mass is a rare presentation of childhood B-acute lymphoblastic leukemia with an estimated incidence of 0.4%. Of the cases reported in the literature, the majority involve adults presenting with spinal cord compression and/or systemic evidence of disease. We describe a young child presenting with pain leading to a refusal to weight-bear secondary to a sacral epidural mass. A biopsy of the sacral lesion confirmed the diagnosis of B-acute lymphoblastic leukemia. Unique to this case is the young age of the child and the lack of spinal cord compression.
MeSH term(s)	Acute Disease ; Female ; Humans ; Infant ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma/complications ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma/diagnosis ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma/therapy ; Prognosis ; Spinal Cord Compression/complications ; Spinal Cord Compression/diagnosis ; Spinal Cord Compression/therapy
Language	English
Publishing date	2019-10-07
Publishing country	United States
Document type	Case Reports ; Journal Article
ZDB-ID	1231152-2
ISSN	1536-3678 ; 1077-4114 ; 0192-8562
ISSN (online)	1536-3678
ISSN	1077-4114 ; 0192-8562
DOI	10.1097/MPH.0000000000001609
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