A case report and review of literature on the role of automated red cell exchange in managing sickle cell crisis in India.

Red cell exchanges (RCE) help in the treatment of complications of sickle cell anemia (SCA) by reducing the viscosity of blood and improving the oxygen-carrying capacity. We present a case of sickle cell crisis (SCC) managed with automated RCE and also reviewed the literature to assess the utilization and clinical efficiency of this therapy in India. A 19-year-old gentleman diagnosed with SCA presented with acute chest syndrome. Hemoglobin (Hb) was 8.8 g%, hematocrit (HCT) was 24%, and HbS was 90%. As there was worsening of symptoms with conventional management, the patient underwent two procedures of automated RCE. The clinical condition of the patient was improved, HbS was reduced to 16% and HCT was remained at 21% postprocedure. Articles on automated RCE in SCA conducted in India were reviewed and four articles were analyzed based on the search strategy. All the included articles concluded automated RCE as an effective procedure for complications of SCA. Common indication in India was SCA patients undergoing surgery as a prophylactic measure. Automated RCEs are promising as an acute treatment for indicated sickle cell complications. This therapy is underutilized in the Indian scenario, especially in patients with SCC.

ADAMTS13 factor deficiency in severe COVID-19 may not be immune mediated - report from a pilot study.

BACKGROUND: The assessment of ADAMTS13 factor activity and inhibitor levels was conducted in severe COVID-19 patients as an observational study. RESULTS: A total of 14 patients were included and the average ADAMTS13 activity level at the time of admission was 28.54±30.74% (range 1.83-86.67%) which was reduced compared to controls (88.09±14.77). Nine patients had reduced ADAMTS13 factor activity (<40%) and 77.7% among them had severe deficiency (<10% activity). ADAMTS13 inhibitor was positive (>15 IU/mL) only in two patients and an overall mean value was 8.15±5.8. Elevated D-Dimer and length of hospital stay had significant correlation with ADAMTS13 activity (-0.247 and 0.306 respectively). No features of thrombotic microangiopathy were observed and hence no plasma exchange was performed. CONCLUSION: Reduced ADAMTS13 factor activity without inhibitor development may give a clue to the disease progress in COVID-19.

Self-ignored onsite adverse donor reaction among whole blood donors: A single center experience from India.

OBJECTIVES: Adverse donor reactions (ADR) may often go unreported due to donor related or blood center related factors. Possible donor related factors are self-ignorance of the adverse reaction, inertia to notify blood centre and non-compliance to follow-up. A better understanding of the self-ignored adverse donor reaction (SIADR) helps in early detection, avoidance of complications, adoption of mitigation strategies, and retention of donors. In the current study, we aim to identify the incidence and reasons for onsite SIADR among whole blood donors. MATERIALS AND METHODS: Prospective single-center observational study where 501 participants who completed whole blood donation were recruited. They were interviewed twice by an experienced investigator to identify any onsite SIADR occurred. First interview was conducted just before leaving the premise and second two days after donation using a peer reviewed and validated questionnaire. Cross-tabulation and Chi-square test were used for bivariate analysis. RESULTS: Twelve participants out of 501 (2.39%) were found to have onsite SIADR which was twice the frequency of reported onsite ADR (1.20%) during the study period in our center. A majority (75%) of them experienced grade I vaso-vagal reactions (VVR). Around 58.3% of the SIADR donors ignored the reaction as they perceived it as mild, while 25% perceived the symptoms but failed to interpret them as a reaction. CONCLUSION: In our center, incidence of onsite SIADR was double the incidence of ADR and majority were VVR grade I. Commonest reason for SIADR was interpretation of reaction as mild. Blood center team shall be proactive and vigilant to identify and report SIADR.

Naturally occurring anti-Kpa in an infant with recurrent bacterial infection: A case report and review of the literature.

Naturally occurring anti-Kpa antibody is extremely rare and was first reported in 1957, named after the first producer 'Penney'. However, the subsequent anti-Kpa reports presented were all anti-Kpa due to isoimmunization. Individuals with severe bacterial infections particularly Gram-negative bacteria are known to be capable of producing cross-reactive antibodies against Kell blood group system. However, such uncommon antibodies like anti-Kpa can be easily missed in routine pre-transfusion testing unless the panel cells containing low incidence antigen are used for antibody screening. Here, we report a case of naturally occurring anti-Kpa antibody, identified incidentally during pre-transfusion testing of a 12-month-old female infant with the diagnosis of Niemann-Pick disease and recurrent bacterial (Escherichia coli) infection.

A case of severe donor reaction leading to grievous injury.

Adverse events of variable severity may occur occasionally following whole blood donation. We are reporting a case of severe donor reaction leading to grievous injury. A first-time male voluntary blood donor donated blood in our blood donation camp. The donation was uneventful, and he left the premise after 20 min of postdonation observation and advice. Several minutes later, he fell on the road and injured himself complicated with severe delayed vaso-vagal reaction. The donor had lacerated wound over the chin, fracture neck of the left mandible, fracture left lower incisor and left lower molar tooth. Appropriate medical care was catered to the donor with full medical support from Department of Transfusion Medicine and was fully recovered from the injury. Blood collection team must take up the responsibility of the management of all complications related to blood donation.