Lineage Switch in an Infant B-Lymphoblastic Leukemia With t(1;11)(p32;q23); , Following Blinatumomab Therapy.

We report a 6 month-old infant girl with t(1;11)(p32;q23), -rearranged B-acute lymphoblastic leukemia (B-ALL) that was refractory to traditional ALL-directed chemotherapy. Following administration of blinatumomab, she experienced lineage switch from B-ALL to acute myeloid leukemia (AML). Myeloid-directed chemotherapy resulted in clearance of AML by flow cytometry, though a residual CD19+ B-ALL population persisted (0.

Brain tissue gadolinium retention in pediatric patients after contrast-enhanced magnetic resonance exams: pathological confirmation.

Background: Retained gadolinium from gadolinium-based contrast agents (GBCAs) used in MR exams has been inferred based on signal changes on serial brain MRI and subsequently demonstrated pathologically in adults. Retention has been similarly inferred in children but pathological demonstration in pediatric patients is limited. The long-term effects of retained gadolinium are unknown but are potentially of greater concern in children given their increased vulnerability from continuing development and their expected longer period of exposure.

A fatal case of bortezomib-induced lung toxicity in a young adult heart transplant recipient.

Bortezomib is approved for the treatment of multiple myeloma but increasingly used in heart transplant (HTx) recipients with antibody-mediated rejection (AMR). Severe pulmonary toxicity is a rare complication in multiple myeloma patients treated with bortezomib, but has not been described in a solid organ transplant recipient. A 20-year-old man 7 years post-HTx presented with acute rejection with hemodynamic compromise.

Consideration of Maternal Anti-enterocyte IgA Transfer With Resulting Infantile Alloimmune Enteropathy.

Autoimmune enteropathy is a rare cause of infantile diarrhea. Cases typically involve infants with a protracted course of diarrhea found to have underlying autoimmune disease or immune dysfunction, leading to chronic intestinal inflammation. We describe a case of immune-mediated enteropathy in an infant with no identifiable autoimmune disease.

The unusual suspects: a curious case of acute kidney injury-Questions.

A previously healthy 8-year-old boy presented for evaluation of three weeks of daily fevers to 101 °F, myalgia, gingival swelling, mouth sores, blurry vision, and periorbital rash with new onset of dark urine. His family history was significant for rheumatologic disease without known renal involvement. Initial workup demonstrated mild anemia with hemoglobin of 10.

Evaluation of the Utility of Bone Marrow Morphology and Ancillary Studies in Pediatric Patients Under Surveillance for Myelodysplastic Syndrome.

Objectives: To evaluate the utility of flow cytometry, karyotype, and a fluorescence in situ hybridization (FISH) panel in screening children for myelodysplastic syndrome (MDS).

Methods: Bone marrow morphology, flow cytometry, karyotype, and FISH reports from 595 bone marrow specimens (246 patients) were analyzed.

Results: By morphology, 8.

Improving Time to Antibiotics for Pediatric Oncology Patients With Suspected Infections: An Emergency Department-Based Quality Improvement Intervention.

Objective: Studies in pediatric patients with fever and neutropenia demonstrate that shorter time to antibiotics is associated with a decrease in pediatric intensive care unit admissions and in-hospital mortality. In 2012, a 2-phase quality improvement intervention was implemented in a pediatric emergency department (ED) to improve care for this high-risk patient population.The objective was to determine if the introduction of (1) a rapid absolute neutrophil count (ANC) test and (2) a standardized prearrival process decreased time to antibiotics for febrile hematology/oncology(heme/onc) patients presenting to the ED.

Hematoxylin Bodies in Pediatric Bone Marrow Aspirates and their Utility in the Diagnosis of Systemic Lupus Erythematosus.

In our recent case report, the finding of lupus erythematosus (LE) cells in a bone marrow aspirate led to the diagnosis of systemic lupus erythematosus (SLE) and appropriate treatment, although the patient was not clinically suspected to have SLE. To determine whether LE cells are present in the bone marrow aspirates of SLE patients, but overlooked in routine bone marrow morphology review, bone marrow aspirates from 30 pediatric patients (15 with SLE and 15 with other diagnoses) evaluated by rheumatologists were reviewed. LE cells were found in the bone marrow aspirates of only 1 SLE patient and none in non-SLE patients.

Is Identification of Lupus Erythematosus Cells Still Useful? A Case Report.

A 13-year-old girl presented with significant weight loss, depression, anemia, and neutropenia. The preliminary diagnosis was anorexia nervosa combined with depression. Due to peripheral cytopenia, a bone marrow biopsy was performed to rule out leukemia.

Adalimumab-Induced Cutaneous Lupus Erythematosus in a 16-Year-Old Girl with Juvenile Idiopathic Arthritis.

Tumor necrosis factor α (TNF-α) antagonists are used in the treatment of numerous autoimmune conditions. Adalimumab is the first monoclonal antibody to TNF-α and is used to treat juvenile idiopathic arthritis. A growing body of literature associates anti-TNF-α therapies with several adverse dermatologic manifestations, including drug-induced lupus erythematosus (LE).

Immune cell function assay in pediatric heart transplant recipients.

The ImmuKnow ICFA reports ex vivo CD4 lymphocyte activation to quantify immunosuppression. Limited organ and age-specific data exist for pediatric heart transplant recipients. We sought to examine their normative values and ICFA's association with rejection/infection.

Isolated appendiceal typhlitis masquerading as perforated appendicitis in the setting of acute lymphoblastic leukemia.

Abdominal pain is common during chemotherapy for childhood leukemia. Clinically differentiating typhlitis from appendicitis can be difficult. We present an 8-year-old boy with abdominal pain in the setting of acute lymphoblastic leukemia and neutropenia.

Improving the value of costly genetic reference laboratory testing with active utilization management.

Context: Tests that are performed outside of the ordering institution, send-out tests, represent an area of risk to patients because of complexity associated with sending tests out. Risks related to send-out tests include increased number of handoffs, ordering the wrong or unnecessary test, specimen delays, data entry errors, preventable delays in reporting and acknowledging results, and excess financial liability. Many of the most expensive and most misunderstood tests are send-out genetic tests.

Clinical validation and implementation of a multiplexed immunosuppressant assay in dried blood spots by LC-MS/MS.

Background: Therapeutic drug monitoring of immunosuppressive drugs is important in transplant patients. We developed and validated liquid chromatography-mass spectrometry (LC-MS/MS) assay for simultaneous quantitation of tacrolimus (TaC), sirolimus (SrL), and cyclosporin A (CsA) in dried blood spots (DBSs) to offer patients home sample collection, avoiding travel for blood draws.

Methods: After extraction, samples were analyzed by LC-MS/MS in multiple reaction monitoring mode.

Implementation of filmarray respiratory viral panel in a core laboratory improves testing turnaround time and patient care.

The FilmArray respiratory virus panel detects 15 viral agents in respiratory specimens using polymerase chain reaction. We performed FilmArray respiratory viral testing in a core laboratory at a regional children's hospital that provides service 24 hours a day 7 days a week. The average and median turnaround time were 1.

Chronic myelogenous leukemia presenting in blast phase with nodal, bilineal myeloid sarcoma and T-lymphoblastic lymphoma in a child.

A 14-year-old boy presented with chronic myelogenous leukemia (CML) in blast phase with segregated extramedullary (nodal) myeloid sarcoma and T-lymphoblastic lymphoma. Immunohistochemical stains performed on the lymphadenectomy sample demonstrated T lymphoblasts in the lymph nodes and myeloblasts in the adjacent soft tissue. Fluorescence in situ hybridization performed on paraffin sections confirmed that both T-lymphoblast and myeloblast populations were positive for the t(9;22) BCR/ABL1 translocation.

Pure erythroid leukemia following precursor B-cell lymphoblastic leukemia.

Therapy-related acute myeloid leukemia is an unfortunate sequel to current multimodal intensive chemotherapy. The patient described was diagnosed with pure erythroleukemia, AML-M6b, during therapy for precursor B-cell acute lymphoblastic leukemia. To the best of our knowledge, this is the first report of this unusual association.

Malignant progression in two children with multiple osteochondromas.

Multiple Osteochondromas (MO) is a disease of benign bony growths with a low incidence of malignant transformation. Secondary chondrosarcoma in children is rare even in children with MO. Making a diagnosis of malignancy in low-grade cartilage tumors is challenging and requires consideration of clinical, radiographic, and histopathological factors.

Laboratory and clinical predictors of thrombosis and hemorrhage in 29 pediatric extracorporeal membrane oxygenation nonsurvivors.

Extracorporeal membrane oxygenation (ECMO) is a life-saving therapy for infants and children with cardiac and respiratory failure. However, thrombosis and hemorrhage are common complications. To determine clinical and laboratory predictors of thrombosis and hemorrhage resulting from ECMO, records and slides were reviewed from 29 consecutive autopsies from 2004 through 2008 of pediatric patients who received ECMO at our institution.