Therapy related AML in a case of chronic lymphocytic leukemia.

In comparison to the general population, patients with chronic lymphocytic leukemia (CLL) are at a higher risk of developing secondary malignancies. Several factors may contribute to pathogenesis, including direct effects of chemotherapy and radiation as well as the reduction of immune surveillance. Factors influencing the increased risk include the increasing age of CLL patients, chronic antigenic stimulation, and immune impairment related to CLL or chemotherapy. Compared to patients with acute myeloid leukemia (AML) that developed from de novo, therapy-related AML (t-AML) has had a poorer outcome. The range of cytogenetic abnormalities in therapy-related AML is comparable to that in de novo AML, although these patients have a significantly higher frequency of unfavourable cytogenetics, such as a complex karyotype or a deletion or loss of chromosomes 5 and/or 7. Herein, we describe a case of therapy-related AML with monocytic differentiation and t(8;16) with a residual CLL population. The aim of the present case is to highlight rare occurrence of therapy related AML with t(8;16) in CLL after fluderabine based chemotherapy (FCR: fludarabine, cyclophosphamide, and rituximab). This case also highlights flowcytometric immunophenotyping as an ideal tool to characterize secondary AML along with the identification of minimal residual disease of CLL clone, which could have ignored at t-AML diagnosis. The pathogenesis of myeloid and lymphoid malignancies as well as their co-existence can be studied by focusing on such patients. Factors predisposing to the development of t-AML should be studied further, which would help in monitoring these patients more carefully.

Value of new advanced hematological parameters in early prediction of severity of COVID-19.

INTRODUCTION: COVID-19 usually presents with upper respiratory tract infection in varying severity which can lead to sepsis. Early prediction of sepsis may reduce mortality by timely interventions. The intended purpose of this study was to determine whether the advanced parameters like the extended inflammation parameters (EIPs) can predict prognosis and early progression to sepsis as a sequel of COVID-19 infection and can be used as a screening profile. Also, to evaluate the Intensive Care Infection Score (ICIS) and the COVID-19 prognostic score and validate the scores for our population. METHODS: Prospective observational study of 50 reverse transcription- polymerase chain reaction (RT-PCR) proven admitted COVID-19 patients. The data assessed included complete blood counts (CBC) with EIP measurements, from Day 1 of admission to Day 10. The following groups were studied: noncritical (NC) and critical illness (CI) in COVID-19 positive cases, COVID negative sepsis and nonsepsis cases, and healthy volunteers for reference range. RESULTS: The parameters that showed statistically significant higher mean in CI group compared to the NC group are reactive lymphocyte number and percentage (RE-LYMPH#, RE-LYMPH%), antibody synthesizing lymphocyte number and percentage (AS-LYMPH#, AS-LYMPH%), Reactive monocyte count and percentage (RE-MONO#, RE-MONO%/M), ICIS, COVID-19 prognostic score (p-value <0.05). The AUC confirmed the diagnostic accuracy of all these parameters. From the multivariate logistic regression, the significant risk factor was RE-LYMPH# with cut-off >0.10 (p value: 0.011). CONCLUSION: The new EIP parameters, RE-MONO#, RE-MONO%/M, ICIS score and COVID-19 prognostic score are useful for early prediction of critical illness. AS-LYMPH is the most useful predictor of critical illness on multivariate analysis. RE-MONO# and RE-MONO%/M parameter are useful in distinguishing critical and noncritical non-COVID and COVID-19 patients.

Hepatosplenic T Cell Lymphoma in an Immunocompetent Female Diagnosed using Flow Cytometry: A Rare Clinical Entity.

Hepatosplenic T-cell lymphoma is a rare haematopoietic malignancy that comprises less than 1% of Non-Hodgkin lymphomas. We are reporting a case of a 26-year-old female, who presented with pallor, weight loss, jaundice, pancytopenia and hepatosplenomegaly. The bone marrow examination showed infiltration by lymphoid cells. These cells on flow cytometric evaluation showed the phenotype of hepatosplenic T cell lymphoma. The cells were positive for CD3, CD8, CD56 and TCR γδ and negative for CD5, CD4, CD8, CD16, CD57, TCRαß along with B cell markers. This case is reported for being a rare clinical entity and its presence in an immunocompetent female making it rarer.

Prompt diagnosis and management of Epstein-Barr virus-associated post-transplant lymphoproliferative disorder and hemophagocytosis: A dreaded complication in a post-liver transplant child.

EBV-associated PTLD is increasingly recognized as an important cause of morbidity and mortality in both solid organ and hematopoietic stem cell transplant recipients. Mortality rates due to PTLD and virus-induced HLH are reported to be quite high. We report a case of EBV-associated PTLD and HLH in a child after liver transplantation who was successfully managed due to timely intervention. This case highlights that measurement of EBV load by quantitative polymerase chain reaction assays is an important aid in the surveillance and diagnosis of PTLD and early detection of EBV-induced PTLD, and aggressive treatment with rituximab is a key to survival in patients who have undergone liver transplantation.

Testicular swelling: a rare manifestation of chronic lymphocytic leukemia presenting with Richter's syndrome.

Richter syndrome (RS) represents the clinico-pathologic transformation of indolent lymphomas to an aggressive lymphoma, most commonly diffuse large B-cell lymphoma. Majority of the patients have a previous diagnosis of Chronic Lymphocytic Leukemia and the median time to transformation is 2-4 years. De novo RS is extremely uncommon. RS frequently arises in the lymph nodes or bone marrow and rarely presents with extra nodal involvement, common sites being the gastrointestinal tract, eye, central nervous system, lung and kidney. Involvement of testis by RS is extremely rare and we came across only one such reported case in the literature. We are reporting this case as our patient presented with de novo RS at an extremely uncommon extra nodal site, testis.

Persistent monotypic plasma cells with absence of neoplastic B cell component in a treated case of waldenström macroglobulinemia: a sign of residual disease?

Waldenström macroglobulinemia (WM) is a rare indolent variant of non- Hodgkin's lymphoma characterised by lymphoplasmacytic infiltration of bone marrow (BM) associated with a serum IgM paraprotein. The WHO classification states that the neoplastic cells of WM usually are positive for monotypic surface immunoglobulin light chain, IgM, CD19, and CD20 and are negative for CD5, CD10, and CD23. Serum monoclonal protein detection by serum protein electrophoresis and bone marrow aspirate and biopsy are required for WM diagnosis, monitoring and response assessment. Pathologist must dissuade themselves from making a hasty decision on calling a complete response in WM when neoplastic B cell component is absent. Evaluation of clonality of any residual plasma cells must be done in all cases of WM to evaluate the presence and extent of residual or persistent disease. Role of additional therapy targeted at these residual plasma cells in WM can be evaluated as tools for achieving complete remission. Herein, we present a case of WM with residual monotypic plasmacytosis in BM, without B lymphocytes after therapy.

Imaging findings in spinal subarachnoid hemorrhage in patient with hemophilia A: an unusual cause for back pain.

Back pain and presentation with spinal canal hemorrhage in hemophilia is not common; however, these are significant clinical issues and may lead to significant neurological issues and morbidity. We present an interesting case of severe back pain in a young patient with moderate hemophilia A. Imaging confirmed subarachnoid hemorrhage in the spinal canal without intracranial hemorrhage. To the best of our knowledge this is the first described case report of subarachnoid hemorrhage in hemophilia A in the English literature. We also describe the anatomy and imaging features of hemorrhage in the different spinal canal compartments, including the subarachnoid space. Spinal canal hemorrhage in hemophilia is an emergency and serious condition and must be diagnosed and treated promptly. It is important to be aware of the diagnostic features of the spinal canal hemorrhage and carefully assess the spinal canal in hemophiliacs on cross-sectional studies.