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BACKGROUND: Mediastinal lymphoma is uncommon, and only 10% of lymphomas occur primarily in the mediastinum. Very few Indian studies have discussed the frequency, presentation, and diagnostic approach of primary mediastinal lymphomas. AIMS: This study aims to detail the frequency and histological features of mediastinal lymphomas diagnosed in our Institute, discuss the close differentials, and compare the morphological and immunohistochemistry (IHC) features of primary mediastinal lymphomas. SETTINGS AND DESIGN: A retrospective observational study. MATERIAL AND METHODS: Fifty-nine cases of primary mediastinal lymphomas diagnosed over 5 years using hematoxylin and eosin (H and E)-stained and IHC slides. STATISTICAL ANALYSIS USED: Descriptive statistics. RESULTS AND CONCLUSIONS: Forty-five (76%) cases had predominant involvement of the anterior mediastinum; Hodgkin lymphoma, and non-Hodgkin lymphoma constituted 12 and 47 cases, respectively. T lymphoblastic lymphoma comprises the most common primary mediastinal lymphoma. There is a marked difference between the demographic profiles of cases with primary mediastinal lymphomas in India and worldwide. A meticulous histological examination to direct a limited panel of IHC markers can help arrive at the final diagnosis in settings where molecular and cytogenetic studies are not performed.
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Early T-cell precursor lymphoblastic leukemia (ETP-ALL) has a unique immunophenotype with very early T-cell differentiation. The current study summarises the distinct clinicopathological aspects of ETP-ALL and compares them with non-ETP-ALL. Twenty-nine ETP-ALL and 191 non-ETP-ALL cases were retrieved between 2018 and 2021. A P value was determined for each of the patient charaterisics (Table 1) to see for any significant relationship (P < 0.05) with ETP-ALL versus non-ETP-ALL. Kaplan-Meier log rank test was applied to look for any significant differences in OS for both the ALLs. ETP-ALL had an incidence of 12.6% out of total T-Acute lymphoblastic leukemia (T-ALL/LBL) in the past 3-years. Compared to non-ETP-ALL, ETP-ALL cases were associated with lower median age and male-to-female ratio. There was no statistically significant difference in the complete remission rate between both the subtypes. ETP-ALL was seen to be associated with high induction failure and relapse rate compared to non-ETP-ALL. To summarise, since the 2-year OS was poor compared to western research (for both ALLs), an intensive chemo-regimen should be implemented in the current situation. Some unusual markers were observed on flow-cytometry (ETP-ALL), which can be useful for MRD quantification, prognosis, and further trials for newer targeted therapies.
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BACKGROUND: Hairy Cell Leukemia (HCL) is an uncommon, indolent lymphoproliferative disorder of mature B lymphoid cells, accounting for 2% of all lymphoid tumors. The present study evaluated the clinical-hematological profile of HCL patients diagnosed at a single tertiary care center over a 11-year period. METHODS: The retrospective observational study was done between October 2010 and September 2021. The relevant clinical and laboratory information were retrieved from hospital medical records and electronic databases. The statistical analysis was performed using version 23.0 of SPSS. RESULTS: 66 (5.9%) of 1125 cases of chronic lymphoproliferative disorder were HCL. Splenomegaly was found in 47 (71.2%), hepatomegaly in 26 (39.5%), and lymphadenopathy in 17 (25.7%) of the cases. The mean hemoglobin, total leukocytes count, and platelets count were 8.04 g/dl, 6.76 X 109/L, and 77 X 109/L, respectively. Pancytopenia was detected in 40 cases (60.61 %). Bone marrow biopsies were majorly hypercellular and showed predominantly diffuse infiltration by atypical lymphoid cells. In two patients, initially thought of having refractory/hypoplastic anemia, the bone marrow biopsy and flow cytometry revealed HCL involvement. 42 cases of HCL underwent flow cytometry. CD20, CD 11c, CD 25 and CD 103 were positive in all the cases. The aberrant expression of CD5, CD10, and CD23 was found in frequencies of 5.71 %, 31.42 %, and 19.35%, respectively. In 40 cases for which follow-up information was available, there was full remission in 26 patients (65%), and later three showed relapse (7.5%) of which one died, and persistent leukemic activity in five (10%). Eight patients (20%) died even before the initiation of treatment. One patient died within one month of therapy. No patient was examined for BRAF V600E mutation analysis. CONCLUSION: CD 10+ HCL was the most prevalent atypical immunophenotypic subgroup. Bone marrow biopsy and flow cytometry are crucial diagnostic tools to rule out hairy cell leukemia. However, BRAF V600E mutation analysis should be performed in cases with unusual presentation or resistance to treatment.
