Antiplatelet antibodies in cases of Glanzmann's thrombasthenia with and without a history of multiple platelet transfusion

Antiplatelet antibodies are known to be present in a wide spectrum of patients, which include chronic Idiopathic Thrombocytopenic Purpura (ITP), infections, etc., including Glanzmann\u2032s thrombasthenia (GT) patients who receive multiple platelet transfusions. The presence of natural antibodies to platelet receptors is not studied in cases of GT. We studied the antiplatelet antibodies in 23 patients with GT, 15 of which had received multiple transfusions and eight that had not received transfusions, along with 50 cases of chronic ITP. The prevalence and specificity of platelet-bound antibodies were detected by inhibition assays using O-group platelets on flow cytometry. The mean antiplatelet antibodies in 15 patients of GT who had not received transfusions and eight patients with multiple transfusions was 8427 + 2131.88 and 9038 + 2856 antibodies/platelet, respectively, while in case of the 50 ITP patients studied, it was 22166 + 5616 antibodies/platelet (Normal Range 1500-3200 antibodies/platelet). We conclude that GT patients who have not received transfusions may develop antiplatelet antibodies to the missing/abnormal receptor. Whether this is due to a molecular mimicry or due to some other mechanism needs to be explored

Phenotyping of Rh, Kell, Duffy and Kidd blood group antigens among non-tribal and tribal population of South Gujarat and its implication in preventing alloimmunisations in multitransfused patients.

Background:Sickle cell anaemia is common amongst Tribal population of south Gujrat. Alloimmunisation in multitransfused sickle cell anaemia patient is 10 times commoner in these patients than beta Thalassemia  major  patients from regular blood donor communities. Study design & methodology: Red cell antigen typing of Rh (D,C,E,c,e ), Kell (K, k), Duffy (Fya, Fyb) and Kidd (Jka, Jkb) were carried out in 222 regular voluntary blood donors who belonged to non-tribal population and in 113 samples of tribal population using conventional antisera.  Results: Rh D antigen frequency was 96.6% in non-tribal and 96.5% in tribal population. 2.4% of K antigen was found in non-tribal population whereas the antigen was absent in tribal population  .Amongst Rh antigens, e was the most common (100%) followed by D, C (91.0%, 85.8%), c (50.5%, 44.2%) and E (16.5%, 17.0%) with DCe/DCe (R1R1, 48.0%, 55.8%) being the most common phenotype in both the groups. In Kell antigens  k antigen was 100% ,Kidd and Duffy antigens  Jk (a+b-) (39.2%, 46.9%) and Fy (a+b-) (64.2%, 52.2%) were the most common phenotypes in non-tribal and tribal population respectively.  Conclusion: There is significant difference in Duffy , Kidd and Kell (k) antigen distribution between non tribal and tribal population . Total absence of Kell antigen in tribalsalong with. E antigen in a significant portion of blood donors and its absence in large number of tribals also increase the risk of alloimmunisation

Self-Reporting Theranostic: Nano Tool for Arterial Thrombosis

Arterial thrombosis (AT) originates through platelet-mediated thrombus formation in the blood vessel and can lead to heart attack, stroke, and peripheral vascular diseases. Restricting the thrombus growth and its simultaneous monitoring by visualisation is an unmet clinical need for a better AT prognosis. As a proof-of-concept, we have engineered a nanoparticle-based theranostic (combined therapy and monitoring) platform that has the potential to monitor and restrain the growth of a thrombus concurrently. The theranostic nanotool is fabricated using biocompatible super-paramagnetic iron oxide nanoparticles (SPIONs) as a core module tethered with the anti-platelet agent Abciximab (ReoPro) on its surface. Our in vitro feasibility results indicate that ReoPro-conjugated SPIONS (Tx@ReoPro) can effectively prevent thrombus growth by inhibiting fibrinogen receptors (GPIIbIIIa) on the platelet surface, and simultaneously, it can also be visible through non-invasive magnetic resonance imaging (MRI) for potential reporting of the real-time thrombus status

Novel deleterious sequence change in the NLRP12 gene in a child with autoinflammatory syndrome, joint hypermobility and cutis laxa from India.

An otherwise healthy male child of 9 years presented with paroxysmal fever and diffuse abdominal pain along with loss of appetite and nausea lasting for 3-4days every 4-6 weeks for last 2 years. He also has stretchable skin and hypermobile joint which he inherited from his mother who never suffered any paroxysmal attack of the kind.  Work up for acute intermittent porphyria, lead poisoning and familial mediterranean fever was negative. A novel harmful sequence change in NLRP12 gene was detected and a diagnosis of NLRP12 associated autoinflammatory syndrome was made. This sequence change with disease has not yet been reported in the literature and is the first such case of NLRP12 related autoinflammatory syndrome from India