Objectives: To describe the case series of patients with myeloid sarcoma with their clinicopathological characteristics, cytogenetics, molecular markers, prognosis, and outcome. Material and Methods: Retrospective retrieval of data of myeloid sarcoma cases in acute myeloid leukemia was done from the electronic health records of our hospital and this case series includes the data of three years starting from January 2018 and the follow-up information was assimilated until December 2020. Results: We present twelve patients in this case series with myeloid sarcoma and all these patients had bone marrow involvement at presentation. Most of the cases were less than 20 years of age and orbit (66.7%) was the commonest site of presentation in this series. Aberrant CD 19 expression on immunophenotyping was a common associate (66.9%) and t(8;21) was the commonest cytogenetic abnormality reported in our case series. Despite of high dose intensive therapy with daunorubicin and cytarabine followed by high-dose cytarabine (HiDAC) consolidation, patients had a median relapse-free survival and median overall survival of 160 days and 299.5 days respectively. Local radiotherapy for consolidation in two of our patients had no additional benefit. Conclusion: Myeloid sarcoma is extramedullary collection of myeloid blasts with or without bone marrow involvement. They were commonly seen in young patients and t(8;21) being a common cytogenetic abnormality associated with it. The treatment outcome of patients with myeloid sarcoma seems dismal and systemic therapy remains the modality of choice.

Pure red cell aplasia (PRCA) is characterized by severe anemia with reticulocytopenia and bone marrow erythroblastopenia. The early erythroblasts are markedly decreased; however, in rare instances, they may be normal or raised in number. There are varied etiologies, namely congenital or acquired and primary or secondary. The congenital PRCA is known as “Diamond-Blackfan anemia.” Thymomas, autoimmune disease, lymphomas, infections, and drugs also may be familiar associates. However, the etiologies of PRCA are numerous, and many diseases/infections can be associated with PRCA. The diagnosis rests on clinical suspicion and appropriate laboratory workup. We evaluated nine cases of red cell aplasia, having severe anemia with reticulocytopenia. Nearly half of the cases showed adequate erythroid (> 5% of the differential count) but with a maturation arrest. The adequacy of the erythroid could confuse the hematologist and may even delay the diagnosis. Hence, it is empirical that PRCA could be considered a differential in every case of severe anemia with reticulocytopenia, even in the presence of adequate erythroid precursors in the bone marrow.

Introduction: Chronic myeloid leukemia (CML) is the commonest adult leukemia in India. Prognostication of newly diagnosed patients of chronic phase CML (CML-CP) is done by calculating pre-treatment risk scores as per Sokal and Hasford scoring systems, and patients are categorized into low-, intermediate- & high-risk groups. According to the latest NCCN guidelines, CML-CP patients with intermediate- or high-risk Sokal or Hasford score may preferentially benefit from second generation tyrosine kinase inhibitor (TKI) treatment. The two second generation TKI drugs available in India are dasatinib & nilotinib. Unfortunately, majority of CML-CP patients in India cannot afford upfront second generation TKI therapy, and generic imatinib is the mainstay of treatment even for intermediate-risk & high-risk patients. Achievement of early molecular response (EMR), defined as BCR-ABL1 (international scale, IS) ≤ 10% after 3 months of first-line TKI therapy, has emerged as one of the most important predictors of favourable long-term outcomes in CML-CP. The present study describes the rate of EMR achievement with first-line generic imatinib therapy in Sokal & Hasford intermediate- & high-risk patients. Objectives: To study the early molecular response rates with generic imatinib therapy at 400 mg/day dose in CML-CP patients with intermediate-risk & high-risk Sokal or Hasford scores. Methods: Our study enrolled 73 newly diagnosed CML-CP patients with intermediate- or high-risk Sokal/Hasford scores between March 2016 and March 2018. All the patients hailed from poor socio-economic background with severe financial constraint, and none of them had any medical insurance. All the patients were treated with generic imatinib mesylate 400 mg/day which was available free of cost at the hospital. None of the patients could afford dasatinib or nilotinib, despite adequate counseling & information regarding the efficacy of 2nd generation TKIs. Treatment response was monitored and defined as per European LeukemiaNet 2013 recommendations. Hematological response was assessed at 3 months for achievement of complete hematologic response (CHR). Molecular response was assessed at 3 months of first-line treatment by quantitative real-time PCR for BCR-ABL1 (IS). Complete data of 65 patients who were compliant to imatinib treatment for at least 3 months were available for analysis. Eight patients were lost to follow up. Results: The median age of patients was 35 years (age range 17 - 72 years; 40 male). CHR was achieved in 92% patients (60 out of 65 patients). Early molecular response at 3 months (BCR-ABL1, IS) ≤ 10%) was documented in 68% (44 out of 65) patients. The range of BCR-ABL1 transcript level at 3 months was 0.01% - 10% in patients who achieved EMR. EMR was not achieved in about 60% of Sokal high-risk patients and 30% of Hasford high-risk patients. Conclusion: The real scenario of CML treatment in developing countries with resource-constrained settings is very much different from that in the developed countries. The response rates to generic Imatinib therapy in Sokal/Hasford intermediate-risk & high-risk CML-CP patients are not impressive. There is scope for significant improvement in treatment response with upfront 2nd generation TKI therapy in intermediate- & high-risk CML-CP patients, if the drugs can be made available at affordable costs in developing countries. Disclosures No relevant conflicts of interest to declare.

Objectives: Translocation (8;21) is a RUNX1-RUNX1T1 fusion transcript, a favorable risk cytogenetic abnormality with a variable clinicopathological profile. However, there is a paucity of data on the outcomes of acute myeloid leukemia (AML) with t(8;21) from East India. This report is an analysis of data of AML with t(8;21) at our center. Material and Methods: De novo AML patients with the presence of t(8;21) cytogenetic abnormality from 2015 to 2019 were analyzed for clinical, pathological, and molecular characteristics and were compared with treatment outcomes. Relapse-free survival (RFS) and overall survival (OS) were determined using Kaplan–Meier curves. Results: Twenty-nine patients (10%) with de novo AML had t(8;21) with 18 male patients and a median age of 20 years. Aberrant expression of CD19, CD56, and CD7 expressions was noted in 44.8%, 17.24%, and 10.29% of patients, respectively. Additional cytogenetic abnormality was observed in 31.03%. CD19 had an 80% correlation with the occurrence of C-kit status. High-dose induction therapy had complete remission rates of 100%. The median duration of follow-up was 287.5 days. The presence of myeloid sarcoma (MS) and C-kit positivity had inferior OS and RFS (P < 0.05). The dose of cytosine arabinoside, given in consolidation of 3 g/m2 and 1.5 g/m2, had a median OS of 758 and 479 days (P = 0.661) and median RFS of 348 and 150 days (P = 0.002), respectively. In the group that received intensive therapy, by the end of 3 years, only 15.7% of patients remain in remission. Conclusion: AML with t(8;21) is seen in young patients with a positive correlation between CD 19 with C-kit positivity. The presence of MS and C-kit positivity endowed inferior OS and RFS. Cytosine arabinoside consolidation in a dose of 3 g/m2 offered an advantage in median RFS.