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Introduction: Mature T- and NK-cell Neoplasms (MTNKN) constitute around 12% of all non-Hodgkin lymphomas. They have a variable clinical course, ranging from indolent to highly aggressive tumours. Flow cytometry immunophenotyping is crucial for the diagnosis, staging, and classification of MTNKN. Aim: To determine the frequency, morphologic, and immunophenotypic profile of various subtypes of MTNKN diagnosed by flow cytometry. Materials and Methods: This was a retrospective descriptive study conducted at the Department of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India. All cases of MTNKN diagnosed by flow cytometry in peripheral blood, bone marrow aspirates, or body fluids from January 1, 2010 to June 30, 2020 (10.5 years duration) at the cancer centre were studied. The morphology of tumour cells and immunophenotype by flow cytometry were analysed. Clinical parameters (lymphadenopathy, hepatosplenomegaly, skin lesions, effusions, B symptoms) and follow-up details, including Progression-free Survival (PFS) and Overall Survival (OS), were also noted. Descriptive statistics for continuous variables and frequencies and percentages for categorical variables were obtained. The Kaplan-Meier method for survival plots was used. Results: Mature T- and NK-cell neoplasms constituted 83 cases. The median age of patients was 56 years. The majority of patients were males (n=49). Adult T-cell Leukaemia/Lymphoma (ATLL) (n=50) constituted the most common subtype, followed by Mycosis fungoides/Sezary syndrome (MF/SS) (n=14), T-cell Large Granular Lymphocytic Leukaemia (T-LGLL) (n=7), T-cell Prolymphocytic Leukaemia (T-PLL) (n=4), Aggressive NK-cell Leukaemia (ANKL) (n=3), Hepatosplenic T-cell Lymphoma (HSTL) (n=3), and Anaplastic Large Cell Lymphoma (ALCL) (n=2). OS and PFS at three years were 22.3% and 16.6%, respectively. Conclusion: Mature T- and NK-cell neoplasms presenting as leukaemia is rare. ATLL was the most common subtype of MTNKN. Flower cells, Sezary cells, and prolymphocytes are useful morphological clues for diagnosis. Immunophenotyping by flow cytometry, along with clinicopathologic correlation, is crucial for the diagnosis and subclassification of MTNKN. All subtypes except T-LGLL show inferior PFS and OS. [ABSTRACT FROM AUTHOR]

Introduction Burkitt's lymphoma (BL) is a highly aggressive B cell non-Hodgkin lymphoma (NHL) having three distinct subtypes: endemic, sporadic, and immunodeficiency-associated BL. Sporadic BL accounts for only 1 to 2% of adult NHL. Objectives The objective of this article was to study the clinical profile and treatment outcome of patients with BL. Materials and Methods This was a retrospective study of 60 patients with BL conducted in the department of medical oncology at a tertiary cancer center in India during a 10-year period. Patients with BL/leukemia above 14 years of age diagnosed during the study period were included and their clinical presentation, treatment details, and outcome were studied. Results Among 60 cases with BL, there were 41 males and 19 females. The median age at presentation was 42 years (range: 14–81 years). The main symptoms were lymphadenopathy, abdominal pain, and abdominal distension. Two patients each had paraparesis, breast lump, and jaw swelling and one patient had involvement of the cervix. Thirteen patients had features of tumor lysis at presentation. The Ann Arbor stage was I in 17, II in 16, III in 5, and IV in 22. Fifty-five patients received combination chemotherapy that included hyper-cyclophosphamide, vincristine, adriamycin, dexamethasone ± rituximab (hyper-CVAD ± R; 35), cyclophosphamide, adriamycin, vincristine, prednisolone/ cyclophosphamide, vincristine, prednisolone ± rituximab CHOP ± R (13), Berlin-Frankfurt-Munich protocol (4), and others (3). Thirty-four patients attained remission, 13 patients had progressive disease, and 8 patients died during chemotherapy. At a median follow-up of 113 months, 58% patients were alive. Conclusions BL accounts for 1.57% of NHL above the age of 14 years with male preponderance. Intensive, short-duration chemotherapy is the standard treatment. Treatment with hyper-CVAD ± R gives 8-year progression-free survival and overall survival (OS) of 60%. Treatment with CHOP ± R is an alternative option in elderly frail patients with an 8-year OS of 46%. [ABSTRACT FROM AUTHOR]

Background: Waldenstroms macroglobulinemia (WM) or Lymphoplasmacytic lymphoma is a rare B cell lymphoproliferative malignancy characterized by serum monoclonal immunoglobulin M (IgM) protein and lymphoplasmacytic infiltration in the bone marrow. Patients usually present in their seventh decade with symptoms related to the infiltration of the marrow or the effects of monoclonal IgM in the blood. Objective: Was to study the clinical characteristics and treatment outcome of patients diagnosed with WM. Materials and methods: This is a retrospective analysis of 26 cases of WM treated at a tertiary cancer centre. Results: WM constituted 0.68% of our nonHodgkin's lymphoma. The median age at presentation was 67 years with a male to female ratio of 2:1. Four patients had lymphadenopathy, six had splenomegaly and four patients had evidence of hyper viscosity. All patients had IgM paraproteinemia, the M band was IgM-kappa in 19 patients and IgM-lambda in seven patients. All patients had a histopathological confirmation. According to IPSS-WM, 12 patients each were in the intermediate and high risk group and 2 in the low risk group. Among the 26 patients, 20 patients received upfront treatment and four patients were kept under observation. The indications for treatment were cytopenia in ten patients, constitutional symptoms in five, hyper viscosity in four and symptomatic lymphadenopathy in one patient. Sixteen patients received rituximab-based chemotherapy. Four patients with features with hyper viscosity underwent plasmapheresis. The three year progression free survival and overall survival were 69.4% and 78 % respectively. Conclusions: WM is a low grade B cell lymphoproliferative disorder having an indolent course and do not require treatment for prolonged period. Diagnosis is challenging due to lack of distinct diagnostic features. Rituximab containing regimens are the standard of care. Newer targeted treatment options may improve the outcome of this incurable disease. [ABSTRACT FROM AUTHOR]

The emergence of additional chromosomal abnormalities (ACAs) in Philadelphia chromosome/BCR-ABL1 positive chronic myeloid leukemia (CML), is considered to be a feature of disease evolution. However, their frequency of incidence, impact on prognosis and treatment response effect in CML is not conclusive. In the present study, we performed a chromosome analysis of 489 patients in different clinical stages of CML, using conventional GTG-banding, Fluorescent in situ Hybridization and Spectral Karyotyping. Among the de novo CP cases, ACAs were observed in 30 patients (10.20%) with lowest incidence, followed by IM resistant CP (16.66%) whereas in AP and BC, the occurrence of ACAs were higher, and was about 40.63 and 50.98%, respectively. The frequency of occurrence of ACAs were compared between the study groups and it was found that the incidence of ACAs was higher in BC compared to de novo and IM resistant CP cases. Likewise, it was higher in AP patients when compared between de novo and IM resistant CP cases, mirroring the fact of cytogenetic evolution with disease progression in CML. In addition, we observed 10 novel and 10 rare chromosomal aberrations among the study subjects. This study pinpoints the fact that the genome of advanced phase patients was highly unstable, and this environment of genomic instability is responsible for the high occurrence of ACAs. Treatment response analysis revealed that compared to initial phases, ACAs were associated with an adverse prognostic effect during the progressive stages of CML. This study further portrayed the cytogenetic mechanism of disease evolution in CML.

Background: t(8;21)(q22;q22) is the most frequent recurrent translocation in acute myeloid leukemia (AML) resulting in an in-frame fusion of RUNX1/RUNX1T1 that regulates various genes involved in the signaling pathways. This leukemogenic alteration is usually associated with a favorable clinical outcome. Variants of t(8;21) can be formed involving a third or fourth chromosome in ~3-4% of t(8;21)-AML. Due to the rarity of variant t(8;21), its clinicopathological features and prognostic significance are still unclear. Here we present three AML cases with cryptic rearrangements of chromosomes 8 and 21 without standard RUNX1/RUNX1T1. Materials and Methods: Conventional karyotyping and fluorescence in situ hybridization and/or spectral karyotyping of the pretreatment bone marrow aspirate of de novo AML patients were performed to delineate chromosomal abnormalities. Results: We identified three cases with novel variants of t(8;21); der(13)t(8;21;13), isodicentric derivative 8 with chromosome 21[,+idicder(8)(q11.1)t(8;21)(q22;q11.1)] and der(21)t(8;12;21)(q22;q?;q22). Conclusion: AML with t(8;21)(q22;q22);RUNX1-RUNX1T1 forms a distinct WHO subcategory and hence the identification of variants or unusual translocations associated with t(8;21) deserves more attention. Contribution to the variant/ unusual t(8;21) database will further refine the risk stratification and may help to significantly advance the current treatment regimen. [ABSTRACT FROM AUTHOR]

Byline: Sureshkumar. Raveendran, Santhi. Sarojam, Geetha. Narayanan, Hariharan. Sreedharan Acute lymphoblastic leukemia is a malignant disease of the bone marrow in which early lymphoid precursors proliferate and replace the normal [...]