EFFICACY OF AUTOLOGOUS STEM CELL TRANSPLANTATION IN ADULT BURKITT/BURKITT-LIKE LYMPHOMA: A SYSTEMATIC REVIEW.

BACKGROUND: Burkitt and Burkitt like lymphoma (BL/BLL) are highly proliferative germinal or post-germinal B cell tumors. Few studies have evaluated the impact of autologous stem cell transplantation (ASCT) on disease outcomes. AIM: We performed a systematic review to analyze the efficacy of ASCT as frontline consolidation and for treatment of relapsed/refractory cases in adult BL/BLL. MATERIALS AND METHODS: Eligible studies with clear outcome measures on the efficacy of ASCT in adult patients with BL/BLL were identified through systematic search. The overall survival (OS), progression-free survival (PFS), complete response (CR), partial response (PR), and progression/relapse were used to assess the efficacy. RESULTS: For patients who underwent ASCT in first CR, 5-year PFS and OS ranged between 70-78% and 70-83% respectively. For relapsed/refractory disease, 5-year PFS and OS were 27% and 31%, respectively. Patients undergoing ASCT for chemoresistant disease fared poorly with 3-year OS of 7% vs 37% for chemosensitive disease (p ≤ 0.00001). The overall response rate to ASCT for patients transplanted in first CR ranged between 71% and 93% and was 37% for patients who were transplanted in disease status other than first CR. Disease progression/relapse was observed in 16-29% of the patients transplanted in first CR, and 55% to 60% in relapsed disease. CONCLUSION: We found insufficient evidence to support ASCT over chemotherapy alone in the first remission for adult BL/BLL. Evidence supports guidelines recommending ASCT for chemosensitive disease but suggests there is no benefit to ASCT for chemoresistant disease.

Safety and Efficacy of Azathioprine as a Second Line Therapy for Primary Immune Thrombocytopenic Purpura.

INTRODUCTION: Immune thrombocytopenic purpura remains common blood disease in Nepal. Azathioprine is an oral immunosupressive medicine which has been used widely in various autoimmune disease and solid organ transplant patients. It is inexpensive, easily available and well tolerated medicine. This study was carried out to evaluate efficacy and safety of azathioprine as a second line medicine for primary ITP patients who were refractory to steroid therapy. METHODS: The observational, pre-post study was conducted at Government of Nepal Civil Service Hospital, Kathmandu from January to October 2014. Twenty four primary ITP patients who were steroid refractory were treated with Azathioprine. Patients were termed steroid refractory if platelet counts were less than 30,000/ul on day 21st of steroid therapy. From day 22 onwards oral azathioprine 2mg/kg was started and steroids were tapered 10mg/week and stopped. Platelet counts of more than 30000/ul after one month of stopping steroid, while still on azathioprine, were termed response to azathioprine. Platelet count of more than 100,000/ul was termed complete response. The associations among age, gender, duration and platelets counts were analyzed by chi square test and Fisher's exact test (when individual cell frequency was less than 5). The comparison of platelets counts among the start and day 90 of Azathioprine therapy was performed by the paired t-test. RESULTS: The study showed that there was not significant association among age and gender of the patients and their platelets count on the start of Azathioprine therapy (p value 0.354 and 0.725 respectively) and on day 90 of Azathioprine therapy (p value 0.082 and 0.762 respectively). The duration-wise comparisons of platelets count on both the start and day 90 of Azathioprine therapy were significant (p values 0.029 and 0.008 respectively). The paired comparison among platelets count on the start and day 90 of Azathioprine therapy was highly significant (p value 0.000). CONCLUSIONS: The study showed the therapeutic implication of azathioprine in ITP patients. It also showed that efficacy of azathioprine was comparable with other modes of treatment. In low income countries like Nepal azathioprine can be considered as second line treatment for steroid refractory ITP patients.

Perioperative management of two cases of hemophilia with spontaneous intracerebral hemorrhage undergoing emergency craniotomy in resource constrained setup of Nepal.

Patients with hemophilia are prone to develop spontaneous intracranial hemorrhage. It carries a significant risk of morbidity and mortality. In this case series, we report two cases of hemophilia who suffered spontaneous intracerebral hemorrhage with features of raised intracranial pressure and were successfully managed perioperatively. The patients were managed with early intensive care unit management, measures to reduce intracranial pressure, perioperative clotting factor administration, airway management and surgery to decrease the raised intracranial pressure. Both patients improved following surgery and were discharged home. Perioperative multidisciplinary management of hemophilia is discussed in this series.

Lambda light chain myeloma with oliguric cast nephropathy and remission with bortezomib, doxorubicin and dexamethasone.

Cast nephropathy is one of the major causes of renal failure in patients with multiple myeloma resulting from precipitation of free light chains inside the tubules. Timely diagnosis and treatment confers a better prognosis though around 10% of patients with cast nephropathy remain dialysis dependent in spite of treatment. We report the clinical course and outcome of a patient presenting with acute kidney injury and oliguria, preceded by acute gastroenteritis and intake of Chinese medications and dialysis dependent state for eight weeks. Kidney biopsy revealed cast nephropathy with lambda light chain restriction and severe tubular injury. Serum protein electrophoresis was normal with no "M spike" but serum free light chain ratio was altered with very high lambda and normal kappa light chain levels. Bone marrow biopsy showed >85% atypical plasma cells. Haemodialysis was continued and chemotherapy with bortezomib, doxorubicin and dexamethasone was started. Kidney function gradually improved with discontinuation of dialysis after 1 month and complete remission of acute kidney injury and myeloma in 4 months of chemotherapy.

Traditional healers' roles on eye care services in Nepal.

AIM: To evaluate the traditional healer (TH) training programme carried out by Nepal Netra Jyoti Sangh, a non-governmental organisation in Nepal, by measuring the changes in knowledge and practices of trained THs in providing primary eye care services. METHODS: 103 trained THs practising in six districts of Nepal were interviewed with a semistructured questionnaire. Their knowledge about various illnesses and eye care practices were compared before and after the training. RESULTS: A significant change in the number of THs with accurate perceived knowledge about trachoma (28.2% v 70.9%, p<0.0001) and cataract (54.4% v 94.2%, p<0.0001) was found after the training. In total, 98 (95%) THs stopped using traditional eye medicines after receiving the training (p<0.0001). The referral practices of THs improved significantly after the training (15% v 100%, p<0.0001). After the training, 95% of the THs used an eye care kit to treat patients with red eyes and simple ocular trauma. CONCLUSION: The findings show that a TH training programme on primary eye care services convinced traditional healers to stop the use of traditional eye medicines and improve referral practices in Nepal.

Trauma and loss as determinants of medically unexplained epidemic illness in a Bhutanese refugee camp.

BACKGROUND: We sought to identify personal factors that placed people at risk during an epidemic of medically unexplained illness in a Bhutanese refugee camp in southeastern Nepal. METHODS: We conducted a case-control study, involving 68 cases and 66 controls. Caseness was defined as experiencing at least one attack of medically unexplained fainting or dizziness during the time of the epidemic. We performed hierarchical logistic regression analysis to identify significant predictors of case status. RESULTS: In terms of Western psychiatric constructs, the illness involved somatoform symptoms of both acute anxiety and dissociation. Sixty per cent reported visual and 28% reported auditory hallucinatory experiences. Cases and controls were similar on all demographic variables, school performance, number of attacks witnessed and psychopathology before the onset of the epidemic. Recent loss, early loss, childhood trauma and pulse-rate were predictors of case status. CONCLUSION: We identified trauma, early loss and, especially, recent loss as predictors of attacks during medically unexplained epidemic illness in a Bhutanese refugee community.