Pediatric allogeneic stem cell transplantation from adult SARS-COV-2 positive donor

Background: Severe acute respiratory syndrome coronavirus 2 (SARS-COV-2) is the cause of COVID-19. Almost 50% of infected people with the virus are asymptomatic. After the introduction of the COVID-19 vaccine, there is a significant reduction in symptomatic infection among vaccinated individuals. The possibility of viral transmission through blood products is unconfirmed yet. Case report: We report a successful hematopoietic stem cell transplant (HSCT) in a patient with sickle cell anemia from an asymptomatic COVID-19-positive donor who underwent stem cell collection under general anesthesia. No complications were encountered during and after the procedure. The marrow was infused safely with good immune reconstitution in the recipient. Conclusion: The report suggests that an asymptomatic COVID-19 positive person might be an acceptable HSCT donor possibly due to existing milder variants of COVID-19

Hepatic vasculitis mimicking liver abscesses in a patient with systemic lupus erythematosus

Clinical and radiological liver diseases are uncommon in patients with systemic lupus erythematosus (SLE). We report a 29-year-old female with SLE who presented with right upper quadrant abdominal pain, thrombocytopenia, elevated liver enzymes and multiple hypodense lesions in the liver on a computed tomography (CT) study that mimicked multiple liver abscesses. A liver biopsy showed mild chronic inflammation. Culture results were negative. With steroid therapy the patient improved clinically, the platelet count returned to the normal range and the multiple liver lesions disappeared radiologicaly. This patient represents a rare case of SLE that had hepatic vasculitis mimicking multiple liver abscesses

Hemopoietic stem cell transplantation in thalassemia: a report from the European Society for Blood and Bone Marrow Transplantation Hemoglobinopathy Registry, 2000-2010

Allogeneic hemopoietic stem cell transplantation (HSCT) is the only method currently available to cure transfusion-dependent thalassemia major that has been widely used worldwide. To verify transplantation distribution, demography, activity, policies and outcomes inside the European Group for Blood and Marrow Transplantation (EBMT), we performed a retrospective non-interventional study, extracting data from the EBMT hemoglobinopathy prospective registry database. We included 1493 consecutive patients with thalassemia major transplanted between 1 January 2000 and 31 December 2010. In total, 1359 (91%) transplants were performed on patients <18 years old, 1061 were from a human leukocyte Ag-identical sibling donor. After a median observation time of 2 years, the 2-year overall survival (OS) and event-free survival (EFS; that is, thalassemia-free survival) were 88±1% and 81±1%, respectively. Transplantation from a human leukocyte Ag-identical sibling offered the best results, with OS and EFS of 91±1% and 83±1%, respectively. No significant differences in survival were reported between countries. The threshold age for optimal transplant outcomes was around 14 years, with an OS of 90-96% and an EFS of 83-93% when transplants were performed before this age. Allogeneic HSCT for thalassemia is a curative approach that is employed internationally and produces excellent results

Trio, a Rho Family GEF, Interacts with the Presynaptic Active Zone Proteins Piccolo and Bassoon.

Synaptic vesicles (SVs) fuse with the plasma membrane at a precise location called the presynaptic active zone (AZ). This fusion is coordinated by proteins embedded within a cytoskeletal matrix assembled at the AZ (CAZ). In the present study, we have identified a novel binding partner for the CAZ proteins Piccolo and Bassoon. This interacting protein, Trio, is a member of the Dbl family of guanine nucleotide exchange factors (GEFs) known to regulate the dynamic assembly of actin and growth factor dependent axon guidance and synaptic growth. Trio was found to interact with the C-terminal PBH 9/10 domains of Piccolo and Bassoon via its own N-terminal Spectrin repeats, a domain that is also critical for its localization to the CAZ. Moreover, our data suggest that regions within the C-terminus of Trio negatively regulate its interactions with Piccolo/Bassoon. These findings provide a mechanism for the presynaptic targeting of Trio and support a model in which Piccolo and Bassoon play a role in regulating neurotransmission through interactions with proteins, including Trio, that modulate the dynamic assembly of F-actin during cycles of synaptic vesicle exo- and endocytosis