Anti NMDA receptor antibody encephalitis in Pakistan: Clinicopathological features and treatment outcomes.

Anti-NMDA receptor antibody encephalitis (anti-NMDAR Encephalitis) is the most common subtype of autoimmune encephalitis in which IgG antibodies directed against NR1 subunit of NMDA receptors are present. It is a potentially lethal encephalitis which responds favourably to timely immunosuppressive therapy. If untreated, its progression leads from delusions, paranoia, movement disorder, memory deficit and seizures into a state of unresponsiveness with autonomic instability and even death. We present clinicopathological features, treatment and outcomes of eight autoantibodyproven cases of anti-NMDAR Encephalitis. There were 7 females and 1 male with a mean age of 15 years (age range: 1 to 28 years). Clinical features included seizures, altered consciousness, memory deficit, delusions, paranoia and hallucinations. Hyperactivity and irritability were prominent features among the children. Patients treated with immunosuppressive therapy including steroids, IVIg, plasmapheresis and Rituximab, recovered completely within a month of therapy. Whereas patients who received only steroids as immunosuppressive therapy suffered from residual brain damage.

Chronic Lymphoproliferative Disorder of Natural Killer Cells: A Rare Event.

Chronic lymphoproliferative disorders are a diverse group of diseases derived from thymus lymphocytes (T cells), bursa of Fabricius cells (B cells), or natural killer (NK) cells. The diagnosis of chronic lymphoproliferative disorders of NK cells (CLPD-NK) is confirmed using antibody panels that are able to detect various stages of maturation of malignant cells. Autoimmune diseases and viral infections are often associated with an increase in circulating NK cells. It is hypothesized that certain viruses trigger the activation of NK cells which leads to the formation of NK cell clones. Majority of the cases are asymptomatic. However, some patients have systemic symptoms and cytopenias. Here, we report a case of CLPD-NK. Our patient's history and marked lymphocytosis on peripheral film raised the suspicion of a hematolymphoid malignancy for which flow cytometric analysis was done using an extensive panel which confirmed the diagnosis of CLPD-NK.

Transfusion Transmissible Infections: Maximizing Donor Surveillance.

Introduction The World Health Organization (WHO) recommends that all blood transfusion services must screen donated blood for human immunodeficiency virus (HIV) one and two, hepatitis B, hepatitis C and syphilis. A mandatory screening for malaria is also warranted in malaria endemic areas. Our study aimed at analyzing the prevalence and different diagnostic methods of screening transfusion transmitted infections (TTIs) in replacement and voluntary, non-remunerated donors in the blood bank of a tertiary care hospital in Islamabad, Pakistan. Methods The cross-sectional, descriptive study was conducted on 30,470 blood donors from July 2015 to October 2017, in the blood bank of a 500-bed teaching hospital in Islamabad. Initially all blood donors were screened for HIV one, HIV two, hepatitis B and hepatitis C by serological testing. The seronegative samples were further tested by nucleic acid amplification test (NAT). Malaria was screened using immuno-chromatographic antigen-detection tests, while treponema pallidum was screened by electrochemiluminescence immunoassay to detect treponema pallidum (TP) antibodies. All infected blood and blood products were discarded and donors were contacted. The donors were deferred from blood donation according to WHO guidelines. They were also counselled and referred to the infectious diseases clinic. The collected data was analyzed on IBM's statistical package for the social sciences (SPSS) version 21. Results The results revealed that amongst the 30,470 donors, 997 (3.27%) donors were found infected with one or more TTI while 29,473 (96.73%) donors were found safe. Individuals who tested positive on serology for hepatitis B were 322 (1.06%), hepatitis C were 392 (1.29%) and HIV were 49 (0.16%). The seronegative donors were tested by NAT. NAT on seronegative samples showed that 10 (0.03%) donors tested positive for hepatitis B virus deoxyribonucleic acid, while only three (0.01%) were positive for hepatitis C ribonucleic acid. No donor was found positive for HIV by NAT testing. Syphilis testing revealed a frequency of 228 (0.75%) positive results while only five (0.02%) donors were found infected with malaria. Conclusion The results testify that standardized blood component screening can save transmission of infections through blood transfusion. They also establish the superiority of NAT screening over serological tests in decreasing the residual risk of transfusion transmitted infections.