Indian Society of Hematology and Blood Transfusion (ISHBT) Consensus Document on Hematological Practice During COVID-19 Pandemic.

The SARS-CoV-2 (COVID-19) pandemic is a worldwide public health emergency with widespread impact on health care delivery. Unforeseen challenges have been noted during administration of usual haematology care in these unusual COVID-19 times. Medical services have been overstretched and frontline health workers have borne the brunt of COVID-19 pandemic. Movement restrictions during lockdown prevented large sections of population from accessing health care, blood banks from holding blood drives, and disrupted delivery of diagnostic hematology services. The disruption in hematology care due to COVID-19 pandemic in India has been disproportionately higher compared to other subspecialities as hematology practice in India remains restricted to major cities. In this review we chronicle the challenges encountered in caring for hematology patients during the COVID-19 pandemic in India and put forth recommendations for minimizing their impact on provision of hematology care with special emphasis on hematology practice in lower and middle income countries (LMICs).

Diagnostic accuracy of point-of-care devices for detection of anemia in community settings in India.

BACKGROUND: Accurate diagnosis of anemia by community workers using a point-of-care device is a challenge. The objective of the study was to establish the diagnostic accuracy of point-of-care devices for detecting anemia in community settings. METHODS: It was diagnostic accuracy study with cross-sectional design on adult patients attending the outpatient department of rural/ urban health centres of Medical colleges from India. The index tests were HemoCue, TrueHb, Massimo's device and spectroscopic device, compared against autoanalyzer (gold standard). Accuracy was expressed by sensitivity, specificity, likelihood ratios, predictive values, area under the curve (AUC) and levels of agreement. For the diagnostic accuracy component, 1407 participants were recruited with a minimum of 600 for each device. An additional 200 participants were considered to elucidate the performance of devices in different weather conditions. RESULTS: HemoCue and TrueHb performed better than Massimo and spectroscopic devices. Detection of anemia by technicians was similar between TrueHb and HemoCue (AUC 0.92 v/s 0.90, p > 0.05). Community workers performed better with Hemocue for detecting anemia compared to TrueHb (AUC 0.92 v/s 0.90, p < 0.05). For detection of severe anemia, accuracy of TrueHb was significantly better with technicians (AUC 0.91 v/s 0.70; p < 0.05) and community workers (AUC 0.91 v/s 0.73; p < 0.05). HemoCue showed a bias or mean difference (95%CI) of 0.47 g/dl (0.42, 0.52) for all values, and 0.92 g/dl (0.82, 1.03) for severe anemia. For TrueHb, it was - 0.28 g/dl (- 0.37, - 0.20) for all readings, and 0.06 g/dl (- 0.52, 0.63) for severe anemia. TrueHb appeared to be more consistent across different weather conditions, although it overestimated Hb in extreme cold weather conditions. CONCLUSION: For detection of anemia, True Hb and HemoCue were comparable. For severe anemia, True Hb seemed to be a better and feasible point-of-care device for detecting anemia in the community settings.

Inherited Macrothrombocytopenia: Correlating Morphology, Epidemiology, Molecular Pathology and Clinical Features.

Inherited macrothrombocytopenia is increasingly being recognized as a relatively common condition. This descriptive review aims at focusing on the different areas of advancement that have taken place with this condition with particular reference to India. A pubmed search of articles between January 1990 and October 2017 with the key words-macrothrombocytopenia, asymptomatic macrothrombocytopenia, macrothrombocytopenia India, syndromic macrothrombocytopenia, molecular pathology, megakaryopoiesis and platelet formation were searched. The shortlisted articles were then read. Review articles provided additional references and the articles thus obtained were also read. Special interest and research conducted by the authors provided further sources of information. A total of 487 articles were found of which 68 articles were related to our subject of review. Review articles were read and additional articles from the reference quoted. Forty-four percent of nonsyndromic Inherited macrothrombocytopenia showed mutations of MYH9, GP1BB, GP1Ba, GPIX, ABCG5 and 8, ACTN, FLI, TUBB and RUNX1 frequently in heterozygous state. All types of inheritance pattern namely autosomal dominant, recessive and sex linked patterns have been described. Syndromic causes of this phenomenon are well known and have been described. Many asymptomatic patients do have mild or moderate bleeding history. Clinical algorithms to differentiate chronic ITP associated macrothrombocytopenia from inherited variety have been explored. Inherited macrothrombocytopenia is an emerging area of interest in platelet biology with its implication in diagnosis, prognosis, genetic counseling, management and in transfusion medicine.

Management of Lymphomas: Consensus Document 2018 by an Indian Expert Group.

The clinical course of lymphoma depends on the indolent or aggressive nature of the disease. Hence, the optimal management of lymphoma needs a correct diagnosis and classification as B cell, T-cell or natural killer (NK)/T-cell as well as indolent or high-grade type lymphoma. The current consensus statement, developed by experts in the field across India, is intended to help healthcare professionals manage lymphomas in adults over 18 years of age. However, it should be noted that the information provided may not be appropriate to all patients and individual patient circumstances may dictate alternative approaches. The consensus statement discusses the diagnosis, staging and prognosis applicable to all subtypes of lymphoma, and detailed treatment regimens for specific entities of lymphoma including diffuse large B-cell lymphoma, Hodgkin's lymphoma, follicular lymphoma, T-cell lymphoma, chronic lymphocytic leukemia/small lymphocytic lymphoma, Burkitt's lymphoma, and anaplastic large cell lymphoma.

Veno-occlusive disease following high dose melphalan.

Hepatic veno-occlusive disease (VOD) is one of the complications following bone marrow transplantation. This complication is uncommon after HDT for autologous SCT (ASCT) in patients with multiple myeloma (MM). Here we report on a 54 years male with MM developed VOD on day 16 of transplant. The Patient was died due to multi-organ failure. The present case suggests that after HDT for ASCT in patients with MM can complicated with VOD.

Prevalance of iron deficiency in thalassemia minor: a study from tertiary hospital.

Conflicting data are available about iron metabolism in thalassemia minors. As iron deficiency prevails largely in India, a study of 150 people was conducted to assess the iron level of ß thalassemia minor. The study population comprises of 59 males and 91 female who either attended outdoor services and with diagnosed thalassemia minor by hemoglobin high performance liquid chromatography or were the parents (diagnosed thalassemia minor) of ß Thalassemia patients visiting daycare services for transfusion. 29.67% females and 3.38% males are found to be iron deficient. Thus we can conclude that iron deficiency is one of the common co-existing conditions in ß thalassemia minors.

Churg-strauss syndrome.

Churg-Strauss syndrome (CSS) is a rare granulomatous necrotizing small vessel vasculitis characterized by the presence of asthma, sinusitis, and hypereosinophilia. The cause of this allergic angiitis and granulomatosis is unknown. Other common manifestations are pulmonary infiltrates, skin, gastrointestinal, and cardiovascular involvement. No data have been reported regarding the role of immune complexes or cell mediated mechanisms in this disease, although autoimmunity is evident with the presence hypergammaglobulinemia, increased levels of IgE and Antineutrophil cytoplasmic antibody (positive in 40%). We report the case of a 27-year-old lady presenting with painful swelling of predominantly lower limbs with extensive vesicles and ecchymotic patches and fever shortly after stopping systemic steroids taken for a prolonged duration (2002--2010). The aim of this case report is to point to the possibility of CSS in patients presenting with extensive skin lesions masquerading as Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Syndrome (SJS/TENS).

Disseminated intravascular coagulation as an unusual presentation of Kala-azar: report of two cases.

Kala-azar (visceral leishmaniasis) is a common problem in various well-defined areas of India. It is characterized by fever of long duration, enlarged liver and spleen, anaemia and leucopoenia. Bleeding is an uncommon manifestation of kala-azar. We report 2 cases, in which disseminated intravascular coagulation was an unusual complication.