Transfusion related babesiosis in a non-endemic region (Western New York).

Babesiosis, an emerging tick-borne zoonosis caused by parasites of the genus Babesia, is typically transmitted via the black-legged tick. Occasionally, can also be transmitted through red blood cell transfusion. In this report, we present a case of babesiosis resulting from a red blood cell transfusion in an area where the pathogen is not endemic.

Peritoneal and Pleural Drains in Pediatric Hematopoietic Cell Transplant Recipients with Veno-Occlusive Disease are Safe and Do Not Adversely Impact Clinical Outcomes.

There is a lack of data on the safety and efficacy of peritoneal drain (PD) and chest tube (CT) in the management of effusions in stem cell transplant recipients with veno-occlusive disease (VOD). In this retrospective pediatric study, clinical outcomes and health resource utilization (HRU) were compared in 32 patients with VOD who had a PD (PD+) post-HCT versus 27 patients who did not (PD-). Nine patients also had a CT (7 PD+ and 2 PD-).

Pulmonary infection with : A case report.

is a non-tuberculous mycobacterium that is an uncommon cause of infection in humans. Risk factors for infection include immunosuppression and pre-existing lung pathology. Herein, we present a case of a 42-year-old male with chronic obstructive pulmonary disease with pulmonary infection caused by that was successfully treated with a regimen of rifampin, isoniazid, pyrazinamide and ethambutol for 2 months, followed by rifampin, isoniazid and azithromycin for an additional 8 months.

Mesenteric venous thrombosis precipitated by foodborne gastrointestinal illness.

Foodborne illnesses are common and are usually considered as part of the differential diagnosis when a patient presents with gastrointestinal symptoms including nausea, vomiting, abdominal pain, diarrhea and fever. The majority of foodborne illness is transient and self-limited, while life threatening complications are rare. Here, we describe a case of a patient presenting with inflammatory diarrhea after consumption of undercooked seafood.

Successful use of reduced-intensity conditioning and matched-unrelated hematopoietic stem cell transplant in a child with Diamond-Blackfan anemia and cirrhosis.

For patients with DBA who are transfusion dependent, HSCT is the only cure. Chronic transfusions can lead to cirrhosis secondary to iron overload, making them poor candidates for myeloablative HSCT. RIC regimens are associated with lower morbidity and mortality compared to myeloablative regimens, but use of RIC in DBA has been limited.

Secondary Acute Myeloid Leukemia in a One-Year-Old Girl Diagnosed with JAK2-V617F Mutation Positive Myeloproliferative Neoplasm.

Myeloproliferative neoplasms (MPNs) are a group of clonal disorders characterized by hyperproliferation of hematologic cell lines and have been associated with tyrosine kinase JAK2-V617F mutations. Secondary acute myeloid leukemia (sAML) is a known complication of JAK2-V617F+ MPNs and bears a poor prognosis. Although the evolution of a JAK2-V617F+ MPN to a mixed-lineage leukemia has been reported in the pediatric population, no evolutions into sAML have been described.

Reduced intensity transplantation for congenital amegakaryocytic thrombocytopenia: report of a case and review of the literature.

CAMT is a bone marrow failure syndrome that usually presents with isolated thrombocytopenia soon after birth. HSCT is curative, and MAC is associated with increased transplant-related morbidity and mortality, especially in the unrelated setting. We used a RIC regimen with alemtuzumab, fludarabine, and melphalan in a seven-month-old patient with CAMT who underwent a MUD HSCT.

Hypervirulent (hypermucoviscous) Klebsiella pneumoniae: a new and dangerous breed.

A new hypervirulent (hypermucoviscous) variant of Klebsiella pneumoniae has emerged. First described in the Asian Pacific Rim, it now increasingly recognized in Western countries. Defining clinical features are the ability to cause serious, life-threatening community-acquired infection in younger healthy hosts, including liver abscess, pneumonia, meningitis and endophthalmitis and the ability to metastatically spread, an unusual feature for enteric Gram-negative bacilli in the non-immunocompromised.

HLA-matched sibling transplantation for severe aplastic anemia: impact of HLA DR15 antigen status on engraftment, graft-versus-host disease, and overall survival.

The HLA class II DRB1 antigen DR15 (common alleles *1501, *1502) is an important marker in the pathobiology of severe aplastic anemia (SAA). We studied 1204 recipients of HLA-matched sibling bone marrow transplantation for SAA to determine whether HLA DR15 status (as determined by allele-level typing) affected hematopoietic recovery, graft-versus-host disease (GVHD), or overall survival (OS). In multivariate analysis, secondary graft failure rate at 2 years was lower in patients who were HLA DR15+ (hazard ratio = 0.

Levetiracetam for busulfan-induced seizure prophylaxis in children undergoing hematopoietic stem cell transplantation.

Anti-seizure prophylaxis is routinely utilized during busulfan administration for HSCT. We evaluated the feasibility and efficacy of levetiracetam in children undergoing HSCT. A total of 28 children and young adults received levetiracetam during HSCT and the outcomes and costs were compared to a historical, but similar cohort of 25 patients who had received fosphenytoin.

Heterozygous M1V variant of ELA-2 gene mutation associated with G-CSF refractory severe congenital neutropenia.

Severe congenital neutropenia is an autosomal recessive disorder characterized by maturation arrest at the promyelocyte/myelocyte phase in the bone marrow, absolute neutrophil count <0.5 × 10(9) /L and recurrent bacterial infections. Homozygous mutations of either HAX-1 or ELA-2 have been described.

Disseminated Rhizomucor pusillus causing early multiorgan failure during hematopoietic stem cell transplantation for severe aplastic anemia.

Matched sibling donor hematopoietic stem cell transplantation is the standard of care for severe aplastic anemia, with an overall survival of 80% to 90%. Only 60% to 70% of patients respond to treatment with immunosuppressive therapy. The main life threatening complications are infections, graft failure, and graft versus host disease.

Tumor immune surveillance defect of X-linked severe combined immunodeficiency is not Epstein-Barr virus specific.

Immunodeficient patients are at an increased risk of developing Epstein-Barr virus (EBV) associated lymphomas. We report a patient with X-linked severe combined immunodeficiency (X-SCID), who presented with an EBV-negative, B-cell non-Hodgkin lymphoma. The tumor did not resolve with chemotherapy or rituximab, but only after recovery of functional donor T-cells cell following hematopoietic stem cell transplantation (HSCT).

Ganciclovir inhibits lymphocyte proliferation by impairing DNA synthesis.

Cytomegalovirus (CMV) disease-related mortality in allogeneic hematopoietic stem cell transplant (HSCT) recipients has dramatically declined because of ganciclovir prophylaxis and preemptive therapeutic strategies. However, ganciclovir has not improved overall survival in randomized studies despite effectively preventing overt CMV disease. Moreover, recurrent posttransplant CMV antigenemia, associated with prolonged ganciclovir exposure, is a predictor of increased relapse of malignancy.