A Saudi multicenter experience on therapeutic plasma exchange for patients with thrombotic thrombocytopenic purpura: A call for national registry.

BACKGROUND: The improvement in the clinical care for patients with thrombotic thrombocytopenic purpura (TTP) is evolving, and many efforts are being put to standardize it. Here, we aimed to assess the provided care at a national level and identify deficiencies. METHODS: A national Saudi retrospective descriptive study was carried out at six tertiary referral centers and included all patients who underwent therapeutic plasma exchange (TPE) for the diagnosis of TTP between May 2005, and July 2022. Collected information included demographic data, clinical features on presentation, and the results of laboratory investigations at admission and discharge. In addition, the number of TPE sessions, days till the first session of TPE, usage of immunological agents, and clinical outcomes were all collected. RESULTS: One hundred patients were enrolled, predominantly female (56%). The mean age was 36.8 years. At diagnosis, 53% of patients showed neurological involvement. The mean platelet count at presentation was 21 × 109 /L. All patients had anemia (mean hematocrit 24.2%). Schistocytes were present in the peripheral blood film of all patients. The mean number of TPE rounds was 13 ± 9.3, and the mean days to start TPE since admission for the first episode was 2.5 days. ADAMTS13 level was measured in 48% of patients and was significantly low in 77% of them. Assessing for clinical TTP scores, 83%, 1000%, 64% of eligible patients had an intermediate/high PLASMIC, FRENCH, and Bentley scores, respectively. Caplacizumab was used on only one patient, and rituximab was administered to 37% of patients. A complete response for the first episode was achieved in 78% of patients. The overall mortality rate was 25%. Neither time to TPE, the use of rituximab or steroid affected survival. CONCLUSIONS: Our study shows an excellent response to TPE with a survival rate approximate to the reported international literature. We observed a deficiency in using validated scoring systems in addition to confirming the disease by ADAMTS13 testing. This emphasizes the need for a national registry to facilitate proper diagnosis and management of this rare disorder.

Validation of long-term handling and storage conditions for hematopoietic stem cell products for autologous transplants.

Hematopoietic stem cells (HPSCs) are multipotent stem cells that can differentiate into lymphoid and myeloid progenitors, giving rise to white blood cells (WBCs), red blood cells (RBCs), and platelets. HPSCs are a widely used treatment for many hematological non-malignant and malignant disorders. HPSCs can be used in the fresh or cryopreserved state for future use. Fresh HPSCs are typically stored at 2-6°C for up to 72 hours and are primarily used for allogeneic transplants or autologous transplants in myeloma and lymphoma patients. However, in some cases of autologous donations, HPSC transplantation is delayed more than three days after collection. In such situations, the cells are thawed after short-term preservation, resulting in a 35% cell viability loss. This study aimed to investigate the quality of HPSCs products after long-term storage exceeding 72 hours. The quality of HPSCs products was assessed by measuring viable CD34+ cell count, the total number of nucleated cells (TNC), and HPSCs recovery after different storage intervals of up to 120 hours in hypothermal storage. The mean total cell viability decreased by 2.18% within 72 hours and 7.4% within 120 hours, while mean CD34+ cell recovery was 92.61 % at 72 hours and 83.83 % at 120 hours in hypothermal storage. The mean TNC recovery was 89.93% at 72 hours and 76.18 % at 120 hours. All products were free from bacterial contamination for up to 120 hours under hypothermal storage conditions.

Triple Thoracic Injury Caused by Foreign Body Ingestion: A New Approach for Managing an Unusual Case.

BACKGROUND In most cases, esophageal perforation is caused by ingested foreign bodies which can migrate through the esophageal wall, damaging nearby vital organs like the aorta or pericardium, thereby having potentially fatal outcomes. Early diagnosis and intervention are key to decreasing morbidity and mortality. Appropriate treatment involves extracting the foreign body, repairing the esophagus and other injured organs (aorta, trachea, or pericardium), and draining and cleaning the mediastinum. CASE REPORT A 31-year-old man presented with a 2-h history of severe chest pain radiating to the back and associated with profuse sweating after eating. The patient had ingested a sharp metal object that injured the thoracic esophageal wall close to the aorta and the left atrium, causing hemopericardium. The presence of pericardial effusion on echocardiogram examination raised a high suspicion of cardiac and/or aortic injury. Left thoracotomy was done because the injury was in the distal third of the esophagus. Therefore, exploration of the pericardium and drainage of the mediastinum was essential, along with the use of resuscitative endovascular balloon occlusion of the aorta (REBOA) to control the proximal aorta while exploring the thoracic aorta. CONCLUSIONS In cases of esophageal injury when aortic involvement is suspected, we suggest using REBOA in selected cases, when an expert team is available, as a mean of gaining better proximal control over the aorta to safely explore and repair any possible injuries. This is an unusual case management scenario that needs further literature and clinical support.