There is sparse literature on the occurrence of acute leukemia in association with Gaucher disease in adults. Earlier, only two cases have been published describing acute leukemia in association with Gaucher disease in the pediatric age group. In this case report, we have described a case of acute myeloid leukemia along with Gaucher disease in an 8-year-old female child who presented with fever with hepatosplenomegaly. Measurement of β-glucosidase activity was the key modality in diagnosis. The possibility that the reduction of the enzyme in Gaucher disease is related to the development of hematological malignancies needs to be explored.

Background and Aims: Superior imaging techniques have increased the recognition of adrenal pathology. Distinguishing benign from malignant adrenocortical tumors is not always easy. Several criteria and immunohistochemical markers have been discovered which help to differentiate between adrenocortical adenoma (ACA) and adrenocortical carcinoma (ACC). Our aim here was to evaluate the diagnostic and prognostic role of steroidogenic factor-1 (SF-1) in adult adrenocortical tumors (ACT) diagnosed using the Weiss criteria. In this cohort, we have also analyzed Ki67 and p53 expression and the extent of agreement between SF-1 and Ki-67. Methodology: This was a retrospective, observational study comprising 24 cases of adult ACT over 10 years. Immunohistochemical staining for SF-1, Ki67, and p53 was done in all the cases, and the results correlated with the morphological diagnosis made using Weiss criteria. Results: SF-1 was 100% sensitive and 80% specific as a marker of malignancy. Increased SF-1 expression correlated with worse survival. There was a moderate degree of agreement between Ki-67 labeling-index and SF-1 as a marker of malignancy with the kappa coefficient being 0.75. The sensitivity of p53 was lower than Ki67 in diagnosing ACC. Conclusion: In adult ACTs, SF-1 has diagnostic significance and prognostic implication. SF-1 is a crucial, dosage-dependent survival factor in ACC. There is a moderate extent of agreement between Ki-67 and SF-1 as a marker of malignancy.

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Adult T-Cell Leukaemia/Lymphoma (ATLL) is a mature T-cell neoplasm. It is caused by human retrovirus Human T-cell Lymphotropic Virus Type 1(HTLV-1). The neoplastic cells after monoclonal integration begin to express T-cell associated antigens namely CD2, CD3 and CD5. These leukaemic cells are highly pleomorphic in light microscopic appearance and also they have a highly variable clinical presentation ranging from acute to lymphomatous to chronic to smouldering. There is a chance of missed or miss diagnosis due to their morphological and clinical heterogeneity and specialised test like immunophenotyping or flow cytometry is essential for exact categorisation. Authors hereby, report a case of 45-year-old female patient suffering from ATLL whose peripheral smear showed leukaemic cells with unusual hairy projections resembling hairy cell leukaemia posing diagnostic dilemma.