COVID-19 infection in patients with haematological disease - A tertiary centre experience from north India

This retrospective study was aimed to understand the clinical, laboratory, radiological parameters and the outcome of COVID-19 patients with underlying haematological disease. All patients with known haematological disease admitted with COVID-19-positive status from April to August 2020 in the COVID-19 facility of a tertiary care centre in north India, were included. Their medical records were analyzed for outcome and mortality risk factors. Fifty four patients, 37 males, were included in the study. Of these, 36 patients had haematological malignancy and 18 had benign disorder. Fever (95.5%), cough (59.2%) and dyspnoea (31.4%) were the most common symptoms. Nine patients had severe disease at diagnosis, mostly malignant disorders. Overall mortality rate was 37.0 per cent, with high mortality seen in patients with aplastic anaemia (50.0%), acute myeloid (46.7%) and lymphoblastic leukaemia (40.0%). On univariate analysis, Eastern Cooperative Oncology Group performance status >2 [odd ratio (OR) 11.6], COVID-19 severity (OR 8.2), dyspnoea (OR 5.7) and blood product transfusion (OR 6.4) were the predictors of mortality. However, the presence of moderate or severe COVID-19 (OR 16.6, confidence interval 3.8-72.8) was found significant on multivariate analysis. The results showed that patients with haematological malignancies and aplastic anaemia might be at increased risk of getting severe COVID-19 infection and mortality as compared to the general population

Diagnosis and Management of Acquired Aplastic Anemia: Consensus Statement of Indian Academy of Pediatrics

Justification: In India, there is a lack of uniformity of treatment strategies for aplastic anemia (AA), and many children are managed only with supportive care due to non-availability of hematopoietic stem cell transplantation (HSCT). Process: Eminent national faculty members were invited to participate in the process of forming a consensus statement in Hyderabad in July, 2016. Draft guidelines were circulated to all members, and comments received in a online meeting in October, 2020 were incorporated into the final draft. These were approved by all experts. Objective: To facilitate appropriate management of children with acquired aplastic anemia. Recommendations: Key recommendations are: i) A bone marrow biopsy is must to make a diagnosis of AA; ii) Rule out inherited bone marrow failure syndromes (IBMFS), connective tissue disorders, viral infections, paroxysmal nocturnal hemoglobinuria (PNH), drug or heavy metal induced marrow suppression in all cases of AA; iii) Conservative approach to transfusions should be followed, with a target to keep hemoglobin >6 g/dL in children with no co-morbidities; iv) HLA-matched sibling donor HSCT is the preferred choice of treatment for newly diagnosed very severe/ severe AA; v) In absence of HLA-matched family donor, a matched unrelated donor (MUD) transplant or immunosuppressive therapy (IST) should be considered as alternate choice based on physician expertise; vi) Fludarabine, cyclophosphamide and anti-thymocyte globulin (ATG) based conditioning with cyclosporine and methotrexate as graft versus host disease (GvHD) prophylaxis is the preferred regimen; vii) Horse ATG and cyclosporine are the recommended drugs for IST. One should wait for 3-6 months for the response assessment and consideration of next line therapy.

Outcome of community based planned intervention targeting perceptions, attitudes and health seeking behaviour regarding cancers in New Delhi, India

Background: Cancer prevention and control needs to be based on effective implementation of all approaches. Community as well as individual level interventions to increase knowledge and early reporting have shown effectiveness. A community-based intervention trial was conducted to design, implement and test short-term outcomes of evidence based preventive strategies.Methods: A mixed methods, three phased study was conducted in three districts of Delhi, which were selected by a random process. Two randomly selected districts out of these three were intervention districts while third was the control. A total sample of 500 in each district was studied in quantitative pre and post -intervention phases. Multistage, stratified, cluster sampling was utilized. Pre-tested and validated tools were used.Results: Post intervention, statistically significant higher scores were observed in all domains in intervention districts as compared to control (p<0.01). Though comparison of slum versus non-slum showed significant increment in knowledge and practice scores, with higher scores for non-slum respondents, the attitudes were very similar (p>0.05). The increment score changes between males and females was similar in all classes of society versus control district. Comparison within the intervention and control groups revealed increased scores in intervention districts and decreased scores in control district, in all domains (p<0.05).Conclusions: Our study has helped in understanding the determinants of perceptions, attitude and practices regarding cancer in the community, This, helped in formulating the need-based intervention strategies. Testing the short-term outcome of intervention showed it to be effective.

Consensus Statement of the Indian Academy of Pediatrics in Diagnosis and Management of Hemophilia

Justification: Despite having standard principles of management of hemophilia, treatment differs in various countries depending onavailable resources. Guideline for management of hemophilia in Indian setting is essential.Process: Indian Academy of Pediatrics conducted a consultative meeting on Hemophilia on 18th September, 2016 in New Delhi, whichwas attended by experts in the field working across India. Scientific literature was reviewed, and guidelines were drafted. All expertcommittee members reviewed the final manuscript.Objective: To bring out consensus guidelines in diagnosis and management of Hemophilia in India.Recommendations: Specific factor assays confirm diagnosis and classify hemophilia according to residual factor activity (mild 5-40%,moderate 1-5%, severe <1%). Genetic testing helps in identifying carriers, and providing genetic counseling and prenatal diagnosis.Patients with hemophilia should be managed by multi-specialty team approach. Continuous primary prophylaxis (at least low-doseregimen of 10-20 IU/kg twice or thrice per week) is recommended in severe hemophilia with dose tailored as per response. Factorreplacement remains the mainstay of treating acute bleeds (dose and duration depends on body weight, site and severity of bleed).Factor concentrates (plasma derived or recombinant), if available, are preferred over blood components. Other supportive measures(rest, ice, compression, and elevation) should be instantly initiated. Long-term complications include musculoskeletal problems,development of inhibitors and transfusion-transmitted infections, which need monitoring. Adequate vaccination of children withhemophilia (with precautions) is emphasized

Effect of ANXA2 gene single nucleotide polymorphism (SNP) on the development of osteonecrosis in Indian sickle cell patient: A PCR-RFLP approach.

Osteonecrosis is a serious complication in sickle cell patients. The common sites of the necrosis are femoral head, head of the humerus and acetabulam. Annexin A2 (ANXA2) protein mainly functions in bone formation and bone resorption. Alteration of ANXA2 gene may affect the manifestations of osteonecrosis in the patients. PCR-RFLP is a common applicable technique for the detection of known mutation/polymorphisms. Here we are presenting application of the PCR-RFLP technique for determination of the ANXA2 gene single nucleotide polymorphism frequency and their clinical association among Indian sickle cell patients. Five known SNPs of ANXA2 gene (rs7170178, rs73435133, rs73418020, rs72746635 and rs73418025) were determined using the HpyCH4V, DdeI, HpyCH4III and Sau 961 restriction enzyme respectively. Restriction enzyme DdeI was common for rs73435133 and rs72746635 SNP. Only the rs7170178 SNP was detected among patient and control and the other four SNPs were absent in the studied groups. The frequency of ANXA2 gene rs7170178 SNP (A/G, G/G) was comparatively higher in sickle cell patients than controls and it was clinically associated with sickle cell osteonecrosis. The P value of heterozygotes (A/G) and homozygotes (G/G) genotypes were <0.001 and 0.001 respectively, which were highly significant. This study established the application of PCR-RFLP in detection of ANXA2 SNPs in sickle cell patients.

Imaging findings in hepatic Langerhans' cell histiocytosis.

We describe ultrasonographic and computed tomographic features of hepatic lesions in two cases of disseminated Langerhans' cell histiocytosis affecting children. In the first case, hyperechoic band like periportal lesions were observed at ultrasonography, which on computed tomography was found to be hypodense admixed with fatty attenuation (HU@23 to - 57) at places. In addition, the caudate lobe was very prominent. In the second case, the hepatic parenchyma showed predominantly hyperechoic diffusely heterogeneous echogenicity. There were features of cirrhosis of liver with portal hypertension in the form of atrophy of right lobe with hypertrophy of left lobe of liver with lobulated outline, prominent main portal vein and splenoportal axis, splenomegaly and gastroesophageal varices. Both the patients were put on chemotherapy as per schedule (Protocol: DAL HX - 83) and are on follow up.