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Showing 4 results for Sharma

Dr Shailaja Shukla, Dr Aruna Chhikara, Dr Tanuja Bundela, Dr Sunita Sharma, Dr Jagdish Chandra,
Volume 10, Issue 3 (7-2020)
Abstract

Background: Acute leukemias comprise a heterogeneous group of diseases characterized by rapid and uncontrolled clonal expansion of progenitor cells of the hematopoietic system. Immunophenotyping helps subclassify acute leukemias into subgroups with prognostic implications.
Materials and Methods: This descriptive observational cross-sectional study was performed on 80 newly diagnosed cases of acute leukemia in children up to 18 years of age from August 2012 to Febuary 2014.
The immunophenotypic analysis was performed by Beckman Coulter Flow cytometer using monoclonal antibodies against various cell surface / cytoplasmic antigens.
Results: Out of 80 cases, 68 cases had acute lymphocytic leukemia (ALL) and 12 cases had acute myelocytic leukemia (AML). In this study, 68 cases of ALL could be categorized into 53 cases of B-ALL, 13 of T-ALL, and 2 cases of Bi-phenotypic acute leukemia (BAL). Twelve cases of AML comprised of 7 cases of AML with minimal differentiation; 2 of AML with cases of AML with maturation, and 3 cases of acute myelomonocytic leukemia. Hepatosplenomegaly was seen in the majority of cases in both ALL and AML. Most cases had a total leucocyte count between 10,000 and 50,000/µl and platelets <100,000/µl.  Hemoglobin levels were < 7.5 g/dl in the majority of them. CD19 and CD79a were 100 % sensitive for B ALL, while cCD3 was 100% sensitive for T-ALL. MPO was positive in all cases of AML.
Conclusion: Immunophenotyping must be done in all AL cases as it helps in risk-stratification and follow up of patients for evaluating minimal residual disease.

Dr Nupur Parakh, Dr Afreen Khan, Dr Sunita Sharma, Dr Jagdish Chandra,
Volume 11, Issue 3 (6-2021)
Abstract

Background: Eβ Thalassemia is characterized by clinical heterogeneity ranging from Non-Transfusion Dependent Thalassemia (NTDT) to Transfusion Dependent Thalassemia (TDT) state, causing management challenges for the clinicians, especially in the pediatric population. Therefore, this study was conducted to give an overview of the clinical profile and management in a tertiary care center.
Materials and Methods: This is a retrospective observational study on the clinical profile of 48 patients with Eβ Thalassemia, after ethical approval. Clinical and biochemical parameters of enrolled patients were entered in pre-designed proforma. The clinical phenotypes of these patients were classified based on the Sripichai scoring system.
Results: The mean age of subjects at presentation was 3.3±2.8 years (M: F=3.8:1). On presentation, 25 (52.02%) patients had severe disease. Their mean age and initial mean hemoglobin at diagnosis were 2.5±1.3 years and 4.9±0.8 g/dl, respectively. They had a relatively larger spleen (p=0.16) and liver size (p=0.67). They were treated as TDT. Twenty-three patients were managed as the NTDT group at baseline. During follow-up period 19 out of 23 patients in the NTDT group (82.6%) were continued to be managed as the NTDT whereas the other four out of 23, required regular transfusion for a short duration. Serum Ferritin was <1000 ng/l in 78 % of patients in NTDT group as compared to 48% in TDT group (p<0.05). Endocrine complications were present 8% in the TDT group. Correlation of severity of clinical manifestation and laboratory findings were done using Chi-square test and T-test.
Conclusion: In the present study, the proper standardized classification of disease severity, helped in the management of these patients. It was found that detailed clinical knowledge regarding the pathophysiology, genetic modifiers, and complications along with close and careful monitoring of these patients based on clinical scores helps the pediatricians to classify these patients for their appropriate management.

Dr Priyanka Singh, Dr Kusha Sharma, Dr Anu Maheshwari, Dr Sunita Sharma,
Volume 12, Issue 4 (10-2022)
Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of excessive immune activation, which is characterized by fever, hepatosplenomegaly, cytopenias, hyperferritinemia, hypertriglyceridemia, and/or hypofibrinogenemia, and evidence of hemophagocytosis. Secondary HLH is often seen in adults and categorized based on autoimmune, infections-related, and malignancy-associated etiologies such as A-HLH, I-HLH, and M-HLH, respectively. This study presented a rare case of HLH developing concurrently at the time of diagnosis of T-cell Acute Lymphoblastic Leukemia (T- ALL) with a unique presentation of membranous tonsillitis in a 10-year-old boy. In all of the cases of T-ALL reported in the pediatric age group, HLH develops post-therapy or at the relapse. The first presentation of leukemia as membranous tonsillitis and concurrent clinic laboratory findings of HLH is rare and can mislead the diagnosis. Therefore, prompt diagnosis is the mainstay of therapy and can considerably improve the prognosis.

Dr Vandana Puri, Dr Kusha Sharma, Dr Sunita Sharma, Dr Shailaja Shukla, Dr Nupur Parakh,
Volume 14, Issue 3 (7-2024)
Abstract

Myeloproliferative neoplasms are clonal hematopoietic stem cell disorders showing proliferation of one or more myeloid lineages. These disorders are characterized by Janus Kinase 2 (JAK2 V617F), Myeloproliferative leukemia (MPL), and Calreticulin (CALR) gene mutations and are seen more commonly in the elderly. These pathognomonic mutations are often absent in children and hence pose a diagnostic challenge. The entire onus of correct diagnosis relies heavily on detailed clinical and laboratory investigations. In the present work, we discuss 4 cases of pediatric myelofibrosis, three of which were secondary to hematolymphoid malignancies, while the remaining one had primary myelofibrosis. Pediatric primary myelofibrosis is a rare entity in children and quite different from adult primary myelofibrosis. The cases in our study show transformation into acute myeloid leukemia, which is associated with an adverse prognosis. Given the rarity of myelofibrosis in children, early and correct diagnosis helps in timely initiation of treatment, which may favorably alter the prognosis.


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