Sickle cell disease (SCD) is common in tribal belt of Gujarat, but not addressed effectively as it should be with effective use of Hydroxyurea, supportive care and counseling. In our single centre study of 70 patients of SCD who were only on Folic acid and Blood transfusion support, were analyzed and followed up for 1 year in terms of their clinical symptoms, Blood transfusion requirement, laboratory parameters before and after Hydroxyurea therapy. We found statistically significant improvement in clinical symptoms and positive changes in laboratory parameters studied. This validates the well established role of Hydroxyurea in SCD as seen in the various international trials. Hence it is imperative that the well documented benefits of Hydroxyurea in various International studies should be translated into clinical practice. SCD should be treated like a chronic disorder needing preventive therapy in form of Hydroxyurea and counseling with regular follow up.

Introduction: Thrombopoietin receptor agonist (TPO RA) is an approved second line of treatment in Persistent and Chronic Immune Thrombocytopenic Purpura (ITP).However in India due to cost constraints, the majority prefer Immunosuppression therapy. A biosimilar of Romiplostim (Romy®) is developed and launched in India, by Intas Pharmaceuticals, India, in July 2019, its monthly cost is around $160, a huge relief to ITP patients (pts), now accessible to the masses in Lower Middle Income Country (LMIC), India. Real-world experience data on Romiplostim (Romy®) in ITP is sparse. Aim: A single centre retrospective data analysis to evaluate the Safety and Efficacy of Biosimilar Romiplostim (Romy®) in patients of Immune Thrombocytopenic Purpura. Method: A total of 54 steroid-refractory or steroid-dependent ITP pts were registered at Haemato-oncology Care Centre Vadodara from July 2019 to January 2020, who received Injection Romiplostim (Romy®) treatment. Pre-treatment Complete Blood Count(CBC) Platelet count (PLT) ,Liver Function Test ,Renal Function Tests ,PT,PTT,ANA,HIV,HBsAg,HCV,Bone Marrow,USG Abdomen, Chest X-Ray was done.All patients had pretreatment Platelet count less than 30000/cumm.The dose of Romiplostim was 1mcg /kg to 6 mcg/kg, subcutaneous once a week.12 /54 patients who had active bleeding also received concomitant Rituximab 100mg IV infusion once a week for 4 weeks. The response was assessed by CBC PLT every week for the first 4 weeks, later on, every monthly. The response was labelled as Optimum if PLT count maintained between 50000/cumm to 4,00,000/cumm, Suboptimal if PLT count increased from baseline but remained below 50,000/cumm, No response if no increment from baseline PLT count. Romiplostim was continued with the same dose if response was Optimum, escalated if response was Suboptimal, de-escalated if PLT increased above 1, 50,000/cumm and stopped if PLT increased above 4,00,000/cumm or no response found after 4 weeks of treatment. Results: Atotal of 54 pts were evaluated, 23(42%) Male, 31(58%) Female, Median age 40 years (range 8 yrs to 85yrs).Persistent ITP was the diagnosis in 38/54(70%)pts, Chronic ITP in 16/54(30%) pts, Secondary ITP in 17(30%) pts, with 14 patients had ANA positive, 2 patients had HCV positive, 1 patient had tubercular lymphadenopathy. The overall response rate (ORR) was 50/54(93%).Optimum response in 49 /54 (91%),Suboptimal response in 1/54 (2%),No response in 4/54(7%)pts. ORR in patients who received Rituximab with Romiplostim was 12/12 (100%). Time to achieve response was 1 week in 46 (85%) pts, 2 weeks in 3(5%) pts, and 3 weeks in 1 (2%) pts. Follow up assessment showed a total of 21/54 (40%) patients had sustained optimum response for more than 6 months with continuation of once a week Romiplostim,9/54 (16%) had sustained response for more than 6 months even on discontinuation of Romiplostim after 4 weeks.8/54(15%) pts with ANA positive reports required a combination of immunosuppressant with Romiplostim to maintain prolonged sustained response.4/54(7%) pts did not respond to Romiplostim hence it was stopped after 4 weeks. Mild adverse effects were observed headache 5(9%), myalgia 3 (5%), bone pains 6(11) Abdominal pain 4(7%) pts. Romiplostim was stopped in 12 (22%) pts because of thrombocytosis (PLT above 4, 50,000/cumm) after a first single dose of Romiplostim. None of the patients had serious adverse events Conclusion: Biosimilar Romiplostim, (Romy®) has shown rapid, excellent and sustained response in the majority of ITP pts in our study. Its affordable cost fulfils an unmet need of ITP patients, requiring the best second line of treatment in LMIC Disclosures No relevant conflicts of interest to declare.

Introduction: Sickle cell disease (SCD) poses a considerable health burden in lower middle income country (LMIC) like India. One ICMR survey reported about 20% of children with SCD died by the age of two years and 30% of children with SCD amongst the tribal community in India died before they reached adulthood. Data on mortality rate in the adult population with SCD is sparse. Our centre data shows that despite the availability of Hydroxyurea and supportive care, almost one-third of hospitalized Sickle Cell Crisis patients develop life threatening complications. Manual Partial Exchange Transfusion is a cost-effective intervention to decrease mortality in Sickle Cell Crisis. But perhaps, it is an underutilized therapy. In India, the cost incurred for each session of exchange transfusion, if done by the RBC apheresis machine, is $420. Whereas Manual Exchange Transfusion costs only $70(including the cost of the central venous catheter) for the first session followed by $15 for each subsequent session. Aim: A single centre retrospective data analysis to evaluate the outcome of Sickle Cell Crisis patients undergoing Manual Partial Exchange Transfusion during hospitalization. Material and Method: A total of 553 SCD patients on regular follow up at the Haemato Oncology Care Centre (HOCC) from July 2012 to July 2020 were evaluated.169 patients were hospitalized for treatment of Sickle Cell Crisis at the Bhailal Amin General Hospital and Sterling Hospital Vadodara. The data was retrieved after the IRB/Apex committee approvals for retrospective analysis. The indication for exchange transfusion was Acute Chest Syndrome 19(35%), Hepatic cell crisis 11(20%), Vaso Occlusive Crisis not responding to the conservative line of therapy in 48 hours of treatment 11(20%), Cerebrovascular Accident (CVA) 5 (9%), Avascular Necrosis of Femur with excruciating pain 3 (6%), Complicated Dengue with Multi-organ failure 3 (6 %), Priapism 1 (2%) and Splenic Sequestration 1 (2%). Central venous access was secured with a central venous catheter or dialysis catheter. Simple packed cell volume was transfused to 11(20%) patients and 5(9%) patients received platelet transfusion. The cut-off values for HB and Platelet count were 8 gm% and 50000/cumm, respectively at the time of the Manual Partial Exchange Transfusion procedure. Blood volume withdrawn was 5 to 10 ml /kg, followed by an equal volume of packed cell volume transfusion at every session. The procedure was repeated every 12 hr or 24 hr depending on the clinical condition of the patient. HbS value was reassessed post 4 sessions with repeat testing done after 2 to 4 sessions if the observed HbS value was more than 30% or more than 10% (for CVA) after 4 sessions. The endpoint was clinical recovery with HbS less than 30% or no clinical recovery despite the achievement of HbS less than 10%. In CVA the endpoint was HBS less than 10%. Results: Manual Partial Exchange Transfusion was carried out in 54/169 (32 %) hospitalized patients. The median age was 25yrs (range 5 yrs to 69 yrs), with male predominance [Males 42(77%) and females 12(23%)].Pre procedure HbF value was <10% in 13(24%), 10 to 20% in 23 (43%) and >20% in 18(33%). A total of 50 out of 54 (93%) patients recovered completely. 28 (52%) patients were hemodynamically stable with normal SPO2 on room air at the time of Manual Partial Exchange Transfusion with an average of 7 days of hospitalization. All of these patients recovered completely with less than 5 sessions of Manual Partial Exchange Transfusion. 26(49%) patients were critically ill and had an average of 12 days of hospitalization. They were on Ventilator and Inotrope support at the time of Manual Partial Exchange Transfusion. 14/26(54%) critically ill patients recovered completely with an average of 6 sessions of Manual Partial Exchange Transfusion. 12/26(46%) critically ill patients succumbed even though post exchange HBS value decreased to less than 10%. The overall mortality rate of SCD patients in this analysis was 12/553 (2.1%), significantly lower than what was historically reported as 30% in the ICMR survey. Underlying Dengue viral infection associated with Multi-organ dysfunction and Fulminant hepatic failure were risk factors for mortality observed in our study. Conclusion: Manual Partial Exchange Transfusion is highly effective in reducing mortality in Sickle Cell Crisis. It is feasible and cost-effective in small centres lacking apheresis machine facility. Disclosures No relevant conflicts of interest to declare.