Rekha Hans

Nucleic acid testing-benefits and constraints

Nucleic acid testing (NAT) is a molecular technique for screening blood donations to reduce the risk of transfusion transmitted infections (TTIs) in the recipients, thus providing an additional layer of blood safety. It was introduced in the developed countries in the late 1990s and early 2000s and presently around 33 countries in the world have implemented NAT for human immunodeficiency virus (HIV) and around 27 countries for hepatitis B virus (HBV).[1] NAT technique is highly sensitive and specific for viral nucleic acids. It is based on amplification of targeted regions of viral ribonucleic acid or deoxyribonucleic acid (DNA) and detects them earlier than the other screening methods thus, narrowing the window period of HIV, HBV and hepatitis C virus (HCV) infections. NAT also adds the benefit of resolving false reactive donations on serological methods which is very important for donor notification and counseling. In a recent Malaysian study[2] 1388 donor samples were tested by serology as well as NAT, authors found 1.37% samples reactive on standard serology methods but non-reactive by NAT. These samples were confirmed to be “false reactive” on confirmatory serological tests.

Efficacy and safety of therapeutic plasma exchange by using apheresis devices in pediatric atypical hemolytic uremic syndrome patients

Therapeutic plasma exchange (TPE) in atypical hemolytic uremic syndrome (aHUS) is considered as first line treatment as per current American Society for Apheresis (ASFA) guidelines. But there is very limited data available in the literature regarding efficacy and safety of TPE procedures in pediatric aHUS patients.

Combination of rituximab with therapeutic plasma exchange in a refractory case of thrombotic thrombocytopenic purpura (TTP)

Therapeutic plasma exchange is established treatment for patients with thrombotic thrombocytopenic purpura (TTP) and for refractory cases immunosuppressive therapy can also be considered. We present a refractory case of TTP which was managed with therapeutic plasma exchange and rituximab.

Dramatic response to plasma exchange in systemic lupus erythematosus with acute complications: Report of two cases

Acute exacerbations and complications are common in patients of systemic lupus erythematosus (SLE) despite of adequate long-term therapy with immunosuppressive drugs. So other options like therapeutic plasma exchange (TPE) can be considered as part of management. Here, we share our experience of two patients of SLE with acute complications who were successfully managed with TPE.

Weber-Christian Panniculitis: Is it a Disorder of Immune System?

To the Editor: Weber-Christian (WC) panniculitis is a rare form of panniculitis with unclear etiopathogenesis, though auto-immune mechanism is suggested [1–3]. A 20-mo-girl presented with recurrent, painful, nodular skin lesions, progressive pallor, and intermittent fever since 6 mo of age. Examination revealed growth failure, severe pallor, cachexia, loss of subcutaneous fat (Fig. 1a), triangular facies, periorbital puffiness, and hepatosplenomegaly. Multiple painful, reddish nodular swellings were seen over calf, thighs, palms, finger tips and toes (Fig. 1b). Investigations revealed microcytic hypochromic anemia (Hb 4.5 g%); ESR 73 mm; normal platelet and leucocyte count, liver and renal functions, lipid profile, NBT reduction, IgE, and IgM levels; elevated IgG (16.56 g/L; normal 3.70–15), IgA (1.71 g/L; normal 0.30–1.30), C3 (1.93 g/L; normal 0.50–1.5), and C4 (0.57 g/L; normal 0.20–0.50). Screening for Tuberculosis; HIV; Toxoplasma, Rubella and CMV; Celiac disease and connective tissue disorders was negative. Bone marrow examination was normal. Skin biopsy revealed lobular panniculitis without vasculitis (Fig. 1c). In hospital, after 2nd blood transfusion (1 wk after first uneventful transfusion) she developed severe acute hemolytic transfusion reaction manifesting as vomiting, rigors, hemoglobinuria; unconjugated jaundice (total serum bilirubin 12 mg/dl), plasma hemoglobin 170 mg/dl, SGOT 925 IU/L, SGPT 86 IU/L, and LDH 8741 IU/L. Kidd-A antibody was positive and direct Coombs test (DCT) negative. Soon she developed hematemesis, epistaxis with shock due to disseminated intravascular coagulation (DIC) and succumbed to it. The evidence of activated immune response in the index case in the form of increased levels of IgG, IgA, C3, and C4; and severe immune mediated acute hemolytic transfusion reaction supports the possible mechanism of immune disturbance in etiopathogenesis of WC panniculitis [1, 2]. Defect in humoral and cellular immunity, alternate pathway of complement system, and increased delayed hypersensitivity reaction have been reported in patients with WC panniculitis [2, 3]. Immunological basis of the disease is further substantiated by reported response to immuno-modulator drugs (steroids, infliximab, and cyclosporine) [2, 4, 5]. This report highlights that WC panniculitis can have significant immune dysregulation and can lead to fatal immune mediated hemolytic transfusion reaction, though such reaction has not been previously documented with this condition. * Rakesh Kumar drrakesh.pgi@gmail.com

Therapeutic Thrombocytapheresis for Symptomatic Thrombocytosis in Hemato-Oncology Patients: Letter to the Editor

GPP case (severity score (2); 11) caused by DITRA. Although the number of pustules decreased following etretinate at 30 mg/day, erythema, pustules and edema persisted, with a decrease in serum albumin to 2.2 g/dL and high CRP level (11.03 mg/dL), and the GPP severity continued to increase (severity score; 13). Therefore, 20 mg/day of prednisolone (PSL) was added; however, edema exacerbated and pleural effusion appeared. We therefore performed GMA once a week for five consecutive weeks (2). After the first session of GMA, all symptoms including erythema with pustules, edema, and fever were completely resolved, and pleural effusion vanished with a reduction in serum IL‐6 level to 49 pg/mL. The GPP severity score was reduced to 7 after the first GMA session (Fig. 1c) and to 0 after the fifth session (Fig. 1b). Serum levels of IL‐6 (7 pg/mL), CRP, and albumin restored within normal range. The serum levels of IL‐ 36, IL‐22, IL‐23, IL‐8, IL‐18 and TNF‐α were all within normal ranges through GMA therapy. The doses of PSL and etretinate were reduced and the administration was finally discontinued. As IL‐36RN is functionally absent in DITRA, inflammatory signaling is continuously activated by IL‐36, resulting in induction of cytokines such as IL‐ 6. GMA therapy was remarkably effective in reducing clinical symptoms and restoring laboratory data in the present case. GMA therapy in DITRA has been reported in only a few cases (1,3,4). Although serum IL‐8 and IL‐6 levels were reported to respond to GMA therapy in a case of DITRA, the reduction of these cytokines was nominal (3). In the present case, IL‐6 levels remarkably dropped in response to GMA therapy, indicating that IL‐6 level may be an indicator of efficacy of GMA therapy.

Cost-minimization analysis in the Indian subcontinent for treating Guillain Barre Syndrome patients with therapeutic plasma exchange as compared to intravenous immunoglobulin

Therapeutic Plasma Exchange (TPE) and Intravenous Immunoglobulin both are first‐line treatments for Guillain Barre Syndrome; however, there is a significant difference in cost. We undertook this study to assess the cost minimization for treating Guillain Barre Syndrome patients.

Therapeutic exchange with reconstituted whole blood using plasma exchange kit on a cell separator in G6PD deficient patient requiring red cell exchange for methemoglobinemia

To the Editor, We here present a case of multiple drug poisoning with Glucose-6 phosphate dehydrogenase deficiency (G6PD) deficiency with methemoglobinemia associated with acute liver failure and renal injury. Methemoglobinemia is a life threatening condition and is characterized by severe impairment of oxygen carrying capacity of hemoglobin as ferrous part of the heme molecule has been oxidized to a ferric state. Methylene Blue is generally given in this condition and acts by reacting within red blood cells to form leukomethylene blue, a reducing agent of oxidized hemoglobin converting the ferric ion back to its oxygen carrying ferrous state. Dose commonly used is 1–2 mg/kg of 1% Methylene Blue solution. However, it is contraindicated in G6PD deficient patients. Present case was a young male 33 years old with very poor glasgow coma scale of E1V1M1 (E: eye opening, 1: no response; V: best verbal response, 1: no response; M: best motor response 1: no response) with bilateral reacting pupil. He was on mechanical ventilation with oxygen saturation as 59%, BP-110/60 mm of Hg. There was history of drug abuse (Heroin, Cannabis?) and patient was on drug de-addiction therapy. Urine examination revealed presence of both benzodiazepine and morphine. A Call for red cell exchange was received in department from the respiratory Intensive Care Unit and a red cell exchange was planned on emergency basis with some modifications on a cell separator (Cobe Spectra, Lakewood Colarado) as G6PD deficiency is a contraindication to administer methylene blue. Patient baseline hemoglobin was 98 g/l, TLC-34.6 3 10/l, platelet count147 3 10/l, G6PD-0.03 nkat/g hemoglobin, methemoglobin proportion was 18% of total hemoglobin equivalent to 2734.20 mmol/l of total methemoglobin. His liver functions were deranged; SGOT-179.29 mkat/l, SGPT-104.84 mkat/l, Serum bilirubin as 342.08 mmol/l. Coagulogram was also deranged with PT/APTT as 53/43 seconds respectively and PTI-24%, patient was anuric with deranged renal functionsserum urea was 26.42 mmol/l and creatinine was 407.52 mmol/l and underwent two sessions of hemodialysis. Patient was on ionotropes and shifted to the department for red blood cell exchange with a mobile ventilator. Informed high risk consent was taken from the patient’s attendants. In view of patient’s critical condition and non availability of red cell exchange kit, plasma exchange kit was used with a plan to exchange patient’s plasma (hemolysed plasma) and red cells by group specific and cross matched (packed red blood cell) PRBC, in which thawed group specific fresh frozen plasma (FFP) was added using sterile connecting device (T-SCD, Terumo Penpol). The tubing of thawed FFP bag was docked to the tubing of the crossmatched PRBC bag by using sterile connecting device. During the procedure patient’s plasma was collected in plasma waste bag as done routinely in plasma exchange procedures and return line was connected to other waste bag as a modification to discard patient’s red cells, so as to reduce methemoglobin concentration. A separate return line was used for infusing reconstituted whole blood at the flow rate of 30 ml/minute, equivalent to inlet rate as shown by the cell separator. The inlet flow rate and return flow rates were monitored throughout the procedure. One blood volume (3000 litres of whole blood, patient weight-64 kgs) was exchanged using six units of PRBC and

Use of “smartphone” technology for managing workload in transfusion-transmissible infection (nucleic acid testing) laboratory for platelet concentrate inventory management resulting in timely issue of platelets: Experience from a tertiary care center

Sir, Our institute is a large tertiary care center in N o r t h I n d i a w i t h m u l t i s p e c i a l t i e s s u c h a s hemato‐oncology (pediatric and adult), bone marrow transplant, advanced trauma care, cardiothoracic vascular surgery, obstetrics and gynecology, and solid organ transplant units which require platelet concentrates (PCs) for their patients. Our department collects 60,000 whole blood units annually and prepares about 32,000 random donor platelets (RDPs) in addition to 2000 single donor apheresis platelets (SDAPs) to cater demand of large number of patients admitted in different specialties. All the collected whole blood units are subjected to transfusion‐transmissible infection screening using semiautomated individual donation (ID) nucleic acid testing (NAT) in addition to the third‐generation ELISA. However, the SDAPs are prepared as and when required for the patients and respective donors are screened accordingly by performing rapid tests in duplicate using kits from two different lots.