Gonadotrophin-independent Precocious Puberty Secondary to an Estrogen Secreting Adrenal Tumor
Conclusion: We conclude that adrenocortical tumors should be considered in children presenting with gonadotrophin-independent precocious puberty and raised estrogens. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Capillaritis as a Skin Manifestation of Hepatosplenic Gamma-Delta T-Cell Lymphoma
We present the case of a 10-year-old boy who was admitted with 3 months of episodic febrile neutropenia and a new petechial rash. Routine bloods identified neutropenia, thrombocytopenia, and a raised alanine aminotransferase. The dermatology team was consulted in light of the symmetrical petechial eruption of the upper torso. A punch biopsy of the lesion was consistent with early capillaritis. The results showed superficial dermal red blood cell extravasation with mild perivascular lymphohistiocytic inflammatory infiltrate. There was no evidence of an atypical lymphoid infiltrate in the skin biopsy. An initial bone marrow ...
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Hodgkin Lymphoma–related Vanishing Bile Duct Syndrome Cholestasis Resolved After Chemotherapy
Vanishing bile duct syndrome is a rare paraneoplastic syndrome occasionally seen in pediatric Hodgkin lymphoma. It is usually regarded as a fatal disorder. Here, we present a case of vanishing bile duct syndrome cholestasis related to Hodgkin lymphoma that resolved after chemotherapy and radiation. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Isolated CNS Relapse in 2 High-Risk B-cell Acute Lymphoblastic Leukemia Patients Following SARS-CoV-2 Infection
We report on this clinical observation to raise awareness of this potential association. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

KMT2A-MLLT1 and the Novel SEC16A-KMT2A in a Cryptic 3-Way Translocation t(9;11;19) Present in an Infant With Acute Lymphoblastic Leukemia
About 25% of the patients with the translocation t(11;19)(q23;p13.3)/KMT2A-MLLT1 present three-way or more complex fusions, associated with a worse prognosis, suggesting that a particular mechanism creates functional KMT2A fusions for this condition. In this work, we show a cryptic three-way translocation t(9;11;19). Interestingly, long-distance inverse polymerase chain reaction sequencing revealed a KMT2A-MLLT1 and the yet unreported out-of-frame SEC16A-KMT2A fusion, associated with low SEC16A expression and KMT2A overexpression, in an infant with B-acute lymphoblastic leukemia presenting a poor prognosis. Our case illust...
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Extramedullary Infiltration in Pediatric Acute Myeloid Leukemia on Surveillance Magnetic Resonance Imaging and its Relationship With Established Risk Factors
Conclusions: Even if EMI symptoms are not evident, surveillance MRI scans at the initial diagnosis of pediatric AML patients are very helpful in detecting a significant number of EMIs. Younger age, some molecular features, and more severe bone marrow involvement of AML patients were related with EMI. However, there was no significant prognostic difference between patients with or without EMI regardless of risk group. Further prospective investigation is necessary to validate the prognostic effect of EMI in a larger group of patients with different risk groups. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Posterior Reversible Encephalopathy Syndrome in the Setting of Asparaginase-associated Pancreatitis in 2 Pediatric Patients With Acute Leukemia
Asparaginase, a critical component of current pediatric acute leukemia treatment protocols, is associated with a number of serious side effects, one of which is pancreatitis. Pancreatitis can result in significant morbidity and mortality from necrosis, pseudocyst formation, hemorrhage, systemic inflammation, intestinal perforation, and sepsis. Another rare complication of pancreatitis is posterior reversible encephalopathy syndrome, likely mediated by systemic inflammation secondary to pancreatic autodigestion and proinflammatory cytokine-mediated vascular endothelial damage. Here, we review this association in the literat...
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Pediatric Chronic Myelogenous Leukemia in T-lineage Blast Crisis: A Reminder in Relevance
We present a unique case of CML in T-lineage BC. The patient was treated with induction chemotherapy plus imatinib followed by matched unrelated donor hematopoietic stem cell transplantation. She is currently off all medications and in complete disease remission. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Blood Loss and Transfusion in a Pediatric Scoliosis Surgery Cohort in the Antifibrinolytic Era
We reported rates of blood loss and transfusion, compared patients receiving tranexamic acid to a ε-aminocaproic acid, and evaluated antifibrinolytic agent and laboratory parameters as predictors of blood loss and transfusion. Intraoperatively, 62% received tranexamic acid and 38% received ε-aminocaproic acid. Overall, blood loss (mean intraoperative estimated blood loss=14.9±9.7 mL/kg, 22% with clinically significant blood loss [>20 mL/kg], and mean calculated hemoglobin mass loss=175.9±70.1 g) and transfusion rates (15% with intraoperative allogeneic red blood cell transfusion and mean intraoperative allogeneic...
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

The Role of Follicle-stimulating Hormone in Vascular Dysfunction Observed in Hematopoietic Cell Transplant Recipients
Childhood cancer survivors who receive a hematopoietic cell transplantation (HCT) are at increased risk for follicle-stimulating hormone (FSH) abnormalities, which may have a substantial negative impact on vascular function. The purpose of this study was to examine the association of vascular function with FSH in HCT recipients, non-HCT recipients and healthy controls. The study included childhood cancer survivors who were HCT recipients (n=24) and non-HCT recipients (n=308), and a control group of healthy siblings (n=211) all between 9 and 18 years old. Vascular measures of carotid artery structure and function (complianc...
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

The Impact of Panel Reactive Antibodies and Different Desensitization Methods on Pediatric Allogeneic Hematopoietic Stem Cell Transplantation Outcomes
Conclusions: Different treatment strategies like plasmapheresis, steroid, rituximab, or combination therapies can be used for the desensitization of PRA-positive patients before hematopoietic stem cell transplantation. Patient-specific treatment modality for sensitized patients before transplant can increase the success rate. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Transition Preparation and Satisfaction of Care Among Adolescents and Young Adults With Sickle Cell Disease at the Ghana Institute of Clinical Genetics
Expanding services in Ghana for people with sickle cell disease is expected to increase childhood survival and need for transition to adult care. Little is known about patient transition experiences in sub-Saharan Africa. We sought to understand those experiences of adolescents and young adults at an adult sickle cell clinic in Accra, Ghana. Individuals 13 to 22 years of age receiving sickle cell care at the Ghana Institute of Clinical Genetics were interviewed to recall their advance preparation and early experiences in adult sickle cell clinic. Mean age of the 100 participants interviewed was 17.9±2.9 years, 65% female....
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Is There An Association Between Vitamin B12 Level and Vitamin D Status in Children?
In conclusion, vit B12 deficiency is common in school-age children, especially in the adolescent age group. It should be known that vit D deficiency may also be present in vit B12 deficiency cases. More comprehensive studies are needed to reveal the relationship between B12 and vit D deficiency. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Rate of Rise of Platelet Count After IVIG for Pediatric Immune Thrombocytopenia
This study aimed to calculate the rate of rise in pediatric ITP after a dose of IVIG and to analyze if patient characteristics affected the rate. For 116 children treated for ITP with IVIG at Hershey Medical Center, the rate of rise of the platelet count for all patients was calculated. The rate of rise ranged from −0.1 to +4.2 K/µL/hour (average 1.3, median 1.2). 78% of patients had a rate of rise of over 0.5 K/µL/hour. There was a statistically significant correlation between the rate rise of the platelet count and the initial platelet count (P=0.0197), but rate was not affected by age or sex. This study was able...
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

A Novel NUP98/RARG Gene Fusion in Pediatric Acute Myeloid Leukemia Resembling Acute Promyelocytic Leukemia
In this study, we report the clinical manifestations and experimental results of a 10-year-old male and review other cases of RARG gene rearrangement reported in the literature. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - March 29, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research