Dolutegravir-induced acquired sideroblastic anemia in a HIV positive patient: A challenging hematologic complication.

Dolutegravir, the most recent antiretroviral drug with high efficacy, good tolerability, infrequent drug-drug interactions, and a favorable safety profile has not been reported in current literature as a cause of acquired sideroblastic anemia. Here, we present a 35-year-old male patient who was diagnosed with acquired sideroblastic anemia to Dolutegravir therapy.

Outcome of ALL With ALL-BFM-95 Protocol in Nepal.

PURPOSE: Data on survival outcomes in patients with acute lymphoblastic leukemia (ALL) originating from Nepal are limited. We aim to present the real-world data on treatment outcomes of patients with de novo ALL treated with pediatric ALL-Berlin-Frankfurt-Muenster (BFM)-95 protocol in Nepal. PATIENTS AND METHODS: We used the medical records of 103 consecutive patients with ALL treated in our center from 2013 to 2016 to evaluate the overall survival (OS) and relapse-free survival (RFS) and analyzed the effects of clinicopathologic factors on survival outcomes in patients with ALL. RESULTS: The 3-year OS and RFS in the entire cohort was 89.4% (95% CI, 82.1 to 96.7) and 87.3% (95% CI, 79.8 to 94.7), with a mean OS and RFS of 79.4 months (95% CI, 74.2 to 84.5) and 76.6 months (95% CI, 70.8 to 82.4), respectively. Patients with prednisone good response (PGR) showed better mean OS and RFS, whereas complete marrow response on day 33 was associated with better mean OS alone. Patients with Philadelphia (Ph)-positive ALL showed worse mean RFS compared to those with Ph-negative status. On multivariate analysis, PGR (hazard ratio [HR], 0.11; 95% CI, 0.03 to 0.49; P = .004) and sagittal vein thrombosis (SVT; HR, 5.95; 95% CI, 1.30 to 27.18; P = .02) were the only independent predictors of OS and RFS, respectively. Adverse events on BFM-95 protocol included SVT (4.9%), peripheral neuropathy (7.8%), myopathy (20.4%), hyperglycemia (24.3%), intestinal obstruction (7.8%), avascular necrosis of femur (6.8%), and mucositis (46%). CONCLUSION: BFM-95 protocol appears to be a safe and effective strategy in adolescent and young adults and adult Nepalese population with ALL with a low toxicity profile.

Review of current status of cardiovascular and thoracic surgery in Nepal: An analysis from collection of institutional database.

OBJECTIVE: Data collected from various institutions around the country was analyzed to assess the current status of cardiovascular and thoracic surgery in the country. METHODS: We collected data from institutions performing cardiovascular and thoracic surgery from all over the country through direct correspondence for the year 2019. Individual institution data on the number of surgeries performed for cardiac, vascular, and thoracic surgery and its outcome in terms of mortality were compiled. The data were further evaluated depending on the type of procedures performed. RESULTS: Overall, a total of 2264 cardiac surgeries were performed in the country in the year 2019. The majority of the surgeries were for valvular heart surgery accounting for 34.3%, followed by congenital surgeries (32.8%) and surgeries for coronary artery disease (25.9%). A total of 649 thoracic surgeries were documented, which is probably marginally less than the actual numbers because we were unable to include an additional few institutions performing low-volume or isolated thoracic procedures in this report. A total of 852 vascular procedures were performed in the country, which is probably underreported. The mortality rates for complex congenital procedures were higher than those reported in the literature and that of adult procedures such as valvular heart disease and coronary artery disease similar to literature. CONCLUSION: We evaluated the recent status of cardiovascular and thoracic surgery in the country with respect to the type of procedures and the postoperative outcomes.

Hemophagocytic Lymphohistiocytosis: an Under-recognized and Life-threatening Condition.

Diagnosis of hemophagocytic lymphohistiocytosis is a challenge in Nepal because of limited resources and the high prevalence of tropical febrile illness mimicking hemophagocytic lymphohistiocytosis. We retrospectively reviewed medical records of 21 patients who were diagnosed with hemophagocytic lymphohistiocytosis from 2010 to 2015 at a single center in Nepal. Two patients had a mutation in their perforin gene and underwent successful haploidentical stem cell transplantation. Marrow hemophagocytosis was found only in 57% of the patients. Five patients had hematological malignancy and were treated with disease-specific chemotherapy. Seven patients developed hemophagocytic lymphohistiocytosis secondary to an infection, including visceral leishmaniasis, scrub typhus, and Epstein Barr virus. EBV-associated hemophagocytic lymphohistiocytosis was refractory to hemophagocytic lymphohistiocytosis 94 protocol, including the addition of rituximab. Malignancy and infection-associated hemophagocytic lymphohistiocytosis was more common. The most common clinical presentations included fever, splenomegaly, hyponatremia, liver function derangement, hyperfibrinogenemia, hyperferritinemia, and cytopenia. With a mortality of 29% in our study cohort, hemophagocytic lymphohistiocytosis should be considered a lethal disease, and clinicians should maintain a high index of suspicion to diagnose this disease. Keywords: Hemophagocytic lymphohistiocytosis; infection; malignancy.

Hematopoietic Stem Cell Transplantation in Nepal: International Partnership, Implementation Steps, and Clinical Outcomes.

Blood and marrow transplantation (BMT) is rarely available in many low- to middle-income countries (LMICs). In 2012, Civil Service Hospital, a government hospital in Kathmandu, partnered with the University of Illinois at Chicago to consult on the establishment of BMT in their hospital, train staff, and promote educational activities. The implementation of BMT occurred in 3 phases over 4 years and included regular onsite visits, training of personnel in Chicago, continuous remote communication, and co-organization of educational events in Kathmandu. The Nepalese government funded the construction of a state-of-the art BMT unit and stem cell laboratory inside Civil Hospital. Autologous (auto) hematopoietic stem cell transplantation (HSCT) was started in 2016, and allogeneic (allo) HSCT from matched related donors (MRDs) or haploidentical (haplo) donors was initiated in 2017. The cost of transplantation was $5200 for auto-HSCT, $10,000 for MRD HSCT, and $13,300 for haplo HSCT. The major socioeconomic determinants reported by Nepalese BMT providers were the cost of transplantation, loss of revenue of the patient and/or caregiver, and cost of transportation. All patients (n = 66) received peripheral blood stem cell grafts, and all allo-HSCT recipients were given post-transplantation cyclophosphamide as graft-versus-host disease (GVHD) prophylaxis. Among recipients of auto-HSCT (n = 30), with a median follow-up of 1029 days (range, 130 to 1653 days), 87% were alive, and transplantation-related mortality (TRM) was 10%. Among allo-HSCT recipients (n = 36), all patients engrafted, and at a median follow-up of 204 days (range, 12 to 1131 days), 75% of them were alive (MRD, 71%; haplo, 83%), with a TRM of 19%. Only 3 of 36 patients developed acute GVHD grade II-IV. The median overall survival in auto-HSCT recipients was 1610 days and was not reached in allo-HSCT recipients. The long-lasting partnership with University of Illinois at Chicago helped build capacity and allowed the Civil Service Hospital team to establish a BMT program in Nepal that has high quality standards at an affordable cost for the majority of patients.

Lung metastasis from renal cell carcinoma 16 years after nephrectomy: A case report and review of the literature.

Renal cell carcinoma can have lung metastasis even after a long interval of radical nephrectomy (16 years after nephrectomy in our case). If any pulmonary nodule is diagnosed with a history of RCC, pulmonary metastasis of RCC should be suspected and should be appropriately treated.

TKI-induced pure red cell aplasia: first case report of pure red cell aplasia with both imatinib and nilotinib.

Tyrosine-kinase inhibitors (TKIs) represent the only hopes for long-term survival for patients with chronic myeloid leukaemia (CML) and gastrointestinal stromal tumours. Thus, uninterrupted use of TKIs is of importance in such patients. Pure red cell aplasia (PRCA) is a rare disorder, not previously known to be associated with TKIs. We present, to the best of our knowledge, the first case of a patient with CML who developed PRCA secondary to both imatinib and nilotinib. Although PRCA was controlled on withdrawal of TKI, TKI continuation in the patient with CML is important. So we treated him with prednisone, but his haemoglobin started to drop on resumption of imatinib. He was changed to nilotinib but again developed PRCA, which did not improve with steroids. We treated him with cyclosporine and were able to reintroduce nilotinib at a reduced dose without further complications. This case report makes physicians aware of this rare complication of TKIs and also provides encouragement that PRCA could be controlled and TKI continued.

Delayed presentation of right and left ventricle perforation due to suicidal nail gun injury.

We describe a case of delayed presentation of attempted suicide with a nail gun that penetrated both the right and left ventricle. Nearly invisible entry point of the nail did not reflect the gravity of the injury. A prompt and accurate history along with chest X-ray and bedside transthoracic echocardiography facilitated localization of the nails and helped assess the damage. Despite cardiac arrest after induction of general anesthesia, the patient had a successful outcome. Issues related to the injury site, modalities of investigation and management strategies in a patient with cardiac tamponade are discussed.

Rapid detection of acute kidney injury by plasma and urinary neutrophil gelatinase-associated lipocalin after cardiopulmonary bypass.

BACKGROUND: Cardiopulmonary bypass (CPB) is associated with a significant risk of postoperative renal dysfunction. We studied the utility of a novel biomarker in predicting acute kidney injury (AKI) in adult patients undergoing cardiac surgery. METHODS AND RESULTS: Blood and urine were obtained from 50 patients undergoing CPB-requiring surgery. Patients were divided into group A (n=41) with normal creatinine pre-bypass and post-bypass and group B (n=9) who developed an increase in serum creatinine of >0.5 mg/dL within the first 48 hours post CPB. Plasma and urinary neutrophil gelatinase-associated lipocalin (NGAL) was determined at baseline and 2 hours after CPB.Plasma levels of NGAL were higher in patients who developed AKI [214+/-16.7 ng/mL (95% CI 176.9-252.9)] compared with those who did not [149.5+/-13.5 ng/mL (95% CI 122.1-175.7); P=0.035]. Two hours after CPB, there was a significant increase (P=0.0003) in NGAL levels, greater in those patients who developed AKI [476.1+/-41.1 ng/mL (95% CI 380.6-571.6); P=0.0003] compared with those who did not [278.4+/-22 ng/mL (95% CI 233.9-323.0)]. In the AKI group, urinary NGAL increased from 7.13+/-2.30 ng/mL (95% CI 2.5-11.8) to 2924+/-786 ng/mL (95% CI 1110-4739). In the non-AKI group, there was an increase from 1.6+/-0.6 (95% CI 0.3-3.0) to 749+/-179 ng/mL (95% CI 386-1113). The post-CPB levels of urinary NGAL were significantly different in the AKI group (P<0.0001) such that a suitable threshold for use as a diagnostic test could be determined. Receiver operating characteristics were determined for plasma and urinary NGAL with area under the curve (AUC) of 0.80 and 0.96, respectively. For a threshold of 433 ng/mmol creatinine, the test had 90% sensitivity and 78% specificity for the detection of post-CPB renal dysfunction. CONCLUSIONS: Measurement of this novel biomarker in the urine or plasma of patients in the first hours after CPB is predictive of subsequent renal injury. Although the AUC for plasma NGAL seemed inferior to urine, even an AUC of 0.8 as reported compares very favorably to that for other "outstanding" biomarkers (eg, AUCs in the 0.7 range for troponin).

Tuberculous pericardial effusion after coronary artery bypass graft.

We describe a case of a recurrent pericardial effusion after coronary artery bypass grafting. This was initially considered to be due to post-pericardiotomy syndrome, but was later treated empirically as tuberculosis. After definitive surgery for this condition, pericardial histology and immunohistochemistry confirmed the diagnosis of tubercular pericarditis. At 4-months follow-up, while continuing anti-tuberculous therapy and corticosteroids, the patient showed consistent improvement without further recurrence of his pericardial effusion. Local reactivation of tuberculosis after pericardiotomy has not been previously reported and merits careful consideration in population groups in which tuberculosis is highly endemic.