A rare case of sinonasal glomangiopericytoma post operative accidental diagnosis and managment-A case report.

INTRODUCTION: Glomangiopericytoma is a rare neoplasm of low malignant potential. It is a rare type of haemangiopericytoma located in nasal cavity. This neoplasm has good prognosis and complete surgical excision is treatment of choice. This case report is representing one such neoplasm. This reporting is done in line with the SCARE criteria (Agha et al., 2018 [1]). CASE PRESENTATION: We presenting a case of 54 year old male patient from upper socioeconomic status who presented at our institution with history of surgery (endoscopic sinus surgery with Septoplasty) 15 days back. A Final histopathology report suggested glomangiopericytoma. Since primary surgery was not done as per oncologic principals, patient was advised for adjuvant radiation. At our institute patient was evaluated again. Revision surgery was done. Patient was discharged next day. DISCUSSION: Glomangiopericytoma is a rare neoplasm with incidence of less than 0.5% of all neoplasms of sinonasal cavity. Prognosis is very good after complete surgical excision. It often confuses clinicians with nasal polyps. Here also patient was operated initially considering as benign polyposis outside. Patient was re-operated again to ensure the complete clearance. CONCLUSION: This is the typical case of converting dual modality treatment to single modality with the help of knowledge, communication, transparent team work. This also a rare type of neoplasm and by reporting this rare case we are contributing to data pool of nasal tumors where lack of reporting is major obstacle in the formation of uniform treatment guidelines.

Improving Outcome of Thalassemia with Hematopoetic Stem Cell Transplantation: An Experience of Gujarat Cancer Research Institute.

Total 26 children of thalassemia underwent hematopoetic stem cell transplantation from September 2006 to December 2014. Out of these 17 were matched sibling transplantation (MST) and 9 were unrelated umbilical cord blood transplantation (UCT). Median age was 4 years. At a median follow up of 46.5 months, 12 of 17 (70 %) MST and 3 out of 9 (33.33 %) UCT were cured of thalassemia. Three (11.53 %) patients died due to transplant related mortality. Average cost of MST was 6 lakhs and that of UCT was 20 lakhs.

Improving Outcome of Hodgkins Disease with Autologous Hematopoietic Stem Cell Transplantation.

We report analysis of all consecutive Hodgkins disease patients undergoing autologous hematopoietic stem cell transplant from September 1999 to December 2014. Out of total 38 patients 26 were males and 12 were females. 32 were adults and 6 were pediatric (<18 years). None were elderly. Median age was 28 years (9-61). All received BEAM protocol as conditioning regimen. Median engraftment time for granulocytes was 12 and 14 days for platelets. Thirty three (86.84 %) patients achieved complete remission out of which 8 (24.24 %) had further relapse. Transplant related mortality occurred in 4 (10 %) patients. Finally 26 (78.78 %) patients were disease free at median follow up of 60 months and median disease free survival (DFS) was 35 months. DFS was 66.66 and 65 %, respectively on 3 and 5 years. While overall survival was 70.83 and 70 % on 3 and 5  years, respectively.

Safety and Efficacy of Eltrombopag in Post-hematopoietic Stem Cell Transplantation (HSCT) Thrombocytopenia.

Twelve adult patients (median age 29.5 years) were started on Eltrombopag 25-50 mg/day for post-hematopoietic stem cell transplantation (HSCT) thrombocytopenia. All patients were having primary thrombocytopenia after HSCT. No patient had other secondary cause for thrombocytopenia. Two patients were allogenic subsets (1 acute myeloid leukemia i.e., AML and 1 aplastic anemia), and 10 were autologous transplants (3 multiple myeloma, 6 lymphoma and 1 AML). Nine patients were males, three were females. The median time of starting Eltrombopag was 21 days post-stem cell infusion (range day +17 to +60) at a median platelet count of 9,000/cmm (range 3,000-11,000/cmm). The median duration for treatment was 29 days. Median total dose of 812.5 mg was received by patients and they had a median platelet increment of 36,000/cmm. We observed that there were no adverse effects in these patients and there was a gradual increase in platelet count so that none of the patients had any complication due to thrombocytopenia. The cost of treatment was less than the cost of extended hospitalization and irradiated single donor platelet transfusion.

Improving Outcome of Aplastic Anaemia with HLA-Matched Sibling Donor Hematopoietic Stem Cell Transplantation: An Experience of Gujarat Cancer and Research Institute (GCRI).

Fifteen patients, with a median age of 19 years having severe aplastic anaemia (SAA) underwent human leucocyte antigen (HLA) identical sibling donor hematopoietic stem cell transplantation (HSCT) using conditioning regimens containing cyclophosphamide with antithymocyte globulin (ATG) or a combination of fludarabine and cyclophosphamide with or without ATG during December 2007 to May 2013. Cyclosporine and mini methotrexate were used as graft versus host disease (GVHD) prophylaxis. Graft source included peripheral blood stem cells in 11, bone marrow in 3 and both in 1. One patient had primary graft failure while 14 patients were engrafted with a median neutrophil and platelet engraftment time of 13.5 days. One patient had secondary graft rejection. Acute GVHD occurred in 3 patients and chronic GVHD in 4. One year death rate in engrafted patients was 14.28 %. At a mean follow-up of 21.2 months, 12 (80 %) are alive and well. One of the donors was a patient of haemophilia but the disease did not occur in the recipient. The graft was successful and the recipient is alive till date.

Unrelated Umbilical Cord Blood Transplant for Children with ß-Thalassemia Major.

Beta thalassemia major, one of the most prevalent hemoglobinopathy throughout the word, can be cured by allogenic stem cell transplantation (SCT) (Bone Marrow Transplant 36:971-975, 2005). Many patients, however, lack a suitably matched related sibling donor. Unrelated umbilical cord blood (UCB) can be used as an alternative stem cell source for these patients. This report describes SCT for nine children with beta-thalassemia major using partially HLA-matched unrelated UCB. Conditioning included oral busulfan 16 mg/kg (day -10 to -7), cyclophosphamide (Cy) 200 mg/kg (day -5 to -2), fludarabine 90 mg/kg (day -13 to -11), and antithymocyte globulin (rabbit) 7.5 mg/kg (day -3 to -1). The infused cell dose was 10.71 × 10(7)/kg total nucleated cells (TNC) (range 6.5-17 × 10(7)/kg TNC). The patients ranged in age from 1.5 to 7 years, in weight from 10.5 to 17 kg. A second transplant with two unrelated cord blood units was attempted in two patients who had primary graft failure. The retransplant recipients were preconditioned with i.v Cy 120 mg/kg (day -3 to -2). Five of the nine patients engrafted promptly with 50-100 % donor chimerism (56 %). They engrafted at a median of 17 days (range 12-19). One patient is transfusion free for 36 months; a second patient is transfusion free for 18 months and a third is transfusion free for 9 months. There was no transplant related mortality. Four of the nine children had autologous recovery without engraftment. Primary graft rejection is the major complication. Post transplant complications were mild hepatic veno-occlusive disease, acute GVHD grade II, and CMV interstitial pneumonia. The chronic GVHD was limited and could be controlled by Methylprednisolone combined with Mycophenolate. The lack of a marrow donor registry in India makes UCBT from related and unrelated donors a good alternative. Transplant should be delayed until the child is at least 18 months of age. The dose of UCB stem cells is the most important factor for engraftment. UCB has the advantages of rapid availability and low risk of severe GVHD despite donor-recipient HLA disparity (Transplant Proc 37:2667-2669, 2005). We demonstrate the feasibility of this procedure in the setting of a developing country.

Acquired pure red cell aplasia in a patient of rheumatoid arthritis.

Pure red cell aplasia (PRCA) is characterized as a normocytic anemia associated with reticulocytopenia and the absence of erythroblasts in the bone marrow. PRCA can be induced by various causes such as thymoma, connective tissue disease, viral infection, lymphoma, and adverse drug reactions. There have been only a few reports of PRCA associated with rheumatoid arthritis (RA). We report a 45 year old female presented with symptomatic anemia of several months duration on a background of long standing seropositive deforming RA. Bone marrow examination revealed marked suppression of erythroid precursors with normal myeloid and megakaryocytic series, thereby confirming PRCA. Anemia improved following therapy with prednisolone 1 mg/kg/day. This article also reviews the current status of therapy in acquired PRCA.

Neutropenic enterocolitis in a child with acute myeloid leukemia successfully treated with early surgical intervention.

Neutropenic entrocolitis (NE) is a life threatening complication of acute leukemia. The case presented here is of a 12 year old boy with acute myeloid leukemia, who developed neutropenic enterocolitis after induction with BFM-93 protocol. Patient underwent exploratory laparotomy during grade 4 neutropenia after failure on conservative line of management of NE. Patient withstood the procedure with supportive care and recovered. This case is reported because NE is a rare but potentially fatal complication and there are no clear guidelines for surgical intervention.

A retrospective study of central venous catheters GCRI experience.

BACKGROUND: The use of central venous catheters (CVCs) has greatly improved the quality-of-care in cancer patients, yet these catheters may cause serious infectious and thrombotic complications. The aim of this retrospective study was to study the various types of CVCs and their complications. MATERIALS AND METHODS: We studied retrospectively 213 cases of CVCs in our institute with their indications, type and complications from August 2010 to July 2011. RESULTS: A total of 213 CVCs were inserted in patients with hematological (62%) and solid organ malignancies (38%). Ninety-eight patients (46%) had peripheral inserted central catheter (PICC), 90 (42%) patients had Hickman catheters and 25 (12%) had a port. The median duration of retention of Hickman catheters was 104 days (3-365 days), for the peripherally inserted central catheters was 59 days (3-100 days) and for the port it was 280 days (45-365 days). Non-infective complications were more than infective (12% vs. 7%). The most common complication was non-infective occlusion and thrombophlebitis. In one patient with PICC thrombosis occurred in the cephalic, radial and ulnar vein and in one patient with port thrombosis occurred in the superior vena cava. Organisms were isolated in 60% (12 out of 20) of cultures. Common organisms isolated were Pseudomonas aeruginosa in 5 (42%), Staphylococcus aureus in 2 (16%), Escherichia coli in 2 (16%) and Aspergillus in 3 (25%) patients. 7 out of 12 infected patients had negative blood cultures within 7 days of antibiotic treatment, 5 patients remained positive for more than 7 days with antibiotics. In 155 patients (73%), the desired treatment protocol was completed and at present there are still 28 patients (13%) with catheters. 5 patients (2.3%) died of febrile neutropenia and septicemia with multi-organ failure. In 5 patients (2.3%), the catheters (1 Port, 1 Hickman and 3 PICC) were prematurely removed because of thrombosis. CONCLUSION: CVCs are better options to facilitate the long-term vascular access provided infection is prevented with meticulous care and treated promptly with proper antibiotics. Most CVCs is acceptable to patients.