Journal of Hematology and Allied Sciences
Latest articles in this journal
Journal of Hematology and Allied Sciences pp 1-3; https://doi.org/10.25259/jhas_22_2021
Kasabach Merritt syndrome (KMS) is a rare disease in which a benign vascular tumor that is hemangioma grows rapidly, entraps red blood cells, platelets, and coagulation factors leading to activation of coagulation cascade resulting in life-threatening disseminated intravascular coagulation and microangiopathic hemolytic anemia. KMS affects newborns and infants. Rarely can affect older children and adults with only a few cases reported in the existing literature. Clinically patients present with large cutaneous hemangioma usually involving the extremities however visceral organs may be involved in some cases along with anemia, thrombocytopenia, coagulopathy, and bleeding. We report a case of KMS in a 28-year-old female who presented with bilateral subdural hematoma, thrombocytopenia, and consumption coagulopathy. She was given seven days course of methylprednisolone to which she responded well.
Journal of Hematology and Allied Sciences pp 1-2; https://doi.org/10.25259/jhas_24_2021
Journal of Hematology and Allied Sciences pp 1-2; https://doi.org/10.25259/jhas_21_2021
Journal of Hematology and Allied Sciences pp 1-10; https://doi.org/10.25259/jhas_18_2021
The coronavirus disease-19 (COVID-19) caused by the SARS-CoV-2 virus, is now an ongoing pandemic. First detected in December 2019 at Wuhan, China, this disease has now spread to all parts of the world. COVID-19 may affect anyone, without regard for age, sex, or underlying disease condition. Patients with benign or malignant diseases when affected, usually have a more severe outcome than people without comorbidities. Increasing one’s immunity by vaccination against COVID-19 will help to improve the disease outcomes of COVID-19 in patients who already have some underlying disease. The live-attenuated or killed and recombinant viral protein vaccines currently available can elicit both humoral and cellular immunities. However, in immunocompromised patients (either due to the disease pathology or treatment-related immunosuppression), immune response may not be as effective as expected. Depending on the underlying disease pathogenesis, the patient may not be able to mount an adequate immune response post-vaccination. However, in view of the severe risks posed by COVID-19 disease, vaccination is of utmost importance. This review aims at understanding the importance of SARS-CoV-2 vaccination in patients with hematological disorders, and also aims to understand the side effects which arise post-SARS-CoV-2 vaccination. We have tried to ascertain the best way to vaccinate patients with hematological disorders.
Journal of Hematology and Allied Sciences pp 1-2; https://doi.org/10.25259/jhas_16_2021
Journal of Hematology and Allied Sciences pp 1-5; https://doi.org/10.25259/jhas_15_2021
Telemedicine is healing at a distance, and it was under-utilized ever since its reintroduction till this COVID-19 pandemic. COVID-19 has moved the field of telemedicine and almost every health specialty has embraced it to provide remote, timely, safe health-care services to their patients from the comfort of their home and prevent undesired exposure to COVID-19 infection. With the reach of smart phones and cheaper internet data, this has reached tier II/III cities and is also making inroads in the rural areas. Telemedicine in hematology is becoming increasingly popular because many of the hematology patients are immunocompromised and are more prone to various infections including COVID-19. It is very beneficial in follow-up visits for benign disorders such as anemia, immune thrombocytopenia as well as certain chronic leukemias such as Myeloproliferative neoplasms and for the patients on oral therapy. Astronaut getting treated on NASA mission from the earth is ultimate telemedicine. COVID-19 pandemic has reinvented telemedicine which is helping not only patients in getting medical advice but also physicians and medical students in attending medical conferences and keep them up to date with advances in their field. After this pandemic end, telemedicine is here to stay along with the age-old personal visit like a hand in gloves.
Journal of Hematology and Allied Sciences pp 1-6; https://doi.org/10.25259/jhas_13_2021
Objectives: High dose factor prophylaxis in hemophilia has been proven to prevent joint bleeds in the western world effectively. We look for a cost-effective and feasible way for Indian patients to reduce the dose and frequency of factor infusion. Data on prophylaxis with a low dose, long-acting factor infusion twice a week dosing schedule is limited. The purpose was to study the efficacy and safety of long-acting factor VIII (Eloctate) for secondary/ tertiary prophylaxis in pediatric and young adult patients with moderate and severe hemophilia A. Materials and Methods: Thirty-eight patients with moderate and severe hemophilia A with an age range from 1 to 25 years were included in the study. During the initial 4 months, they received therapeutic doses of ELOCTATE (Factor VIII with Fc Fusion Protein) on an episodic basis after a clinical bleed. In the next 4 months, they received prophylactic intravenous ELOCTATE at the dose of 20 units/kg body weight twice a week. Annual bleeding rates (ABR), school absenteeism, emergency visits, joint scores, and adverse events were compared during both periods. Results: The total number of joint bleeds during the episodic treatment and prophylaxis period was 608 and 67, respectively. ABR was 47.9 during the episodic treatment period and 5.3 during prophylaxis showing an 88.9% reduction in joint bleeds. School/college absenteeism and emergency visits were significantly reduced during prophylaxis. No significant adverse events were noted during prophylaxis. Conclusion: Low dose, twice a week, and long-acting recombinant factor VIII-Fc (Eloctate) prophylaxis can be a reasonable options for patients with hemophilia A in developing countries.
Journal of Hematology and Allied Sciences pp 1-7; https://doi.org/10.25259/jhas_14_2021
COVID-19 infection causes substantial changes in blood coagulation. Understanding this process helps management of the patient with least injury through therapeutic misadventurism. At the heart of the disease process, there is widespread endothelial and pulmonary alveolar epithelial cell damage related to the entry and proliferation of the virus and subsequent cellular response to virus invasion. The virus directly triggers fibrinolytic system which positively increase cellular viral load, cytokine generation, exudation in the alveoli, and both intravascular and extravascular blood coagulation and fibrinolysis. The major coagulation catastrophe comes from immunocoagulation and contribution by specific and non-specific cells (lymphocytes, monocytes, and neutrophils) augmenting the process. Hypoxia also contributes and plays an independent role. Platelet activation, complement activation, and vasculitis or vasculitis-mimics take part in the process. Some of these mechanisms are well established and some are yet to be worked out. COVID-19 infection unequivocally points out the great role of cellular activation and cytokines play in coagulation process; indicates classical anticoagulants, antiplatelets, statins, complement inhibitors, and steroids in managing this infection. The author concentrates on the pathobiology of blood coagulation with perspectives on how to manage each of these steps.
Journal of Hematology and Allied Sciences pp 1-6; https://doi.org/10.25259/jhas_11_2021
Objectives: Myelodysplastic syndrome (MDS) is a group of myeloid neoplasms. The clinical manifestations and treatments vary depending on the subtype and risk stratification of the disease. There is a paucity of data on Indian patients with MDS. This study was undertaken to understand MDS with regard to their clinical presentation, pathological, cytogenetic profiles and also to assess their therapeutic outcomes and prognosis from our center in Eastern India. Material and Methods: This is a prospective observational study conducted in the department of hematology at a tertiary care teaching hospital from eastern part of India. The diagnosis of MDS was made from the peripheral blood examination, bone marrow aspirate examination, cytogenetics, and Fluorescence in situ hybridization results, according to the WHO guidelines. Patients were risk stratified using Revised International Prognostic Scoring System (R-IPSS) and subsequent therapeutic planning was done, with either supportive therapy in the form of recombinant human erythropoiesis stimulating agents, colony stimulating factors, packed red blood cell support as needed for low risk MDS patients. High risk patients were treated with hypomethylating agents such as Azacytidine, Decitabine, or Lenalidomide. Results: The mean duration of follow-up of patients with MDS from the point of diagnosis was 1.8 years (range 4 months–6 years). The median OS was 1.33 years. The median OS in the analysis of our patient cohort with low, intermediate, high, and very high R-IPSS was 1.67 years, 1.33 years, 1.67 years, and 1.67 years, respectively. No patients of very low risk group were identified in our study. Conclusion: Our findings reflect that MDS-MLD with low or intermediate R-IPSS risk groups is the most common types of MDS. Although supportive therapy was used to treat patients irrespective of other therapy given (depending on the risk group of the patient), it was used alone even in higher risk groups due to logistic reasons in some cases. Those patients who received supportive care alone also had a good survival duration. However, a longer follow-up duration is required to firmly establish this outcome. The median age of patients (55 years) was also lower than established studies with a median overall survival of 1.67 years.
Journal of Hematology and Allied Sciences, Volume 1, pp 12-21; https://doi.org/10.25259/jhas_2_2021
Objectives: Monoclonal antibodies (MAs) are increasingly becoming part of therapeutic armamentarium for hematologists and hemato-oncologists. There is paucity of review on majority of these antibodies in one place. The objective of this review is an attempt to fill the gap in paucity of review on majority of these monoclonal antibodies (MAs) in one place. Material and Methods: ‘Pubmed’ and ‘Scopus’ database was explored focusing on monoclonal antibodies (MAs) in clinical hematological practice. Emphasis was given to the more recently published review articles on different monoclonal antibodies (MAs). Results: In the present review, a total of 23 different monoclonal antibodies (MAs) were discussed; some are very frequently used and some rarely. Monoclonal antibodies (MAs) are used for treatment of diverse hematological conditions, i.e. malignant and benign disorders and at various phases of stem cell transplantation. These antibodies were used either alone or in combination with various chemotherapeutic agents, targeted small molecules or as immunoconjugates. Some of the side effect profiles of these antibodies were common and some were unique to the particular monoclonal antibody (MA). Unusual infections or organ dysfunctions were noted. Improved function of antibodies by protein engineering is also advancing rapidly. Dosage, frequency and route of administration depended on the convenience and condition for which the antibody is used. Conclusion: Monoclonal antibodies (MAs) are going to stay for hematological practice. Some amount of familiarity with their usage, advantages, disadvantages and side effects are essential in clinical practice.