Key Points• Thrombophilia in children with perinatal stroke is rare, with rates similar to those in the normal population.• Routine testing in childhood is not indicated.Perinatal stroke causes cerebral palsy and lifelong disability. Specific diseases are definable, but mechanisms are poorly understood. Evidence suggests possible associations between arterial perinatal stroke and prothrombotic disorders, but population-based, controlled, disease-specific studies are limited. Understanding thrombophilia in perinatal stroke informs pathogenesis models and clinical management.We conducted a population-based, prospective, case-control study to determine the association of specific perinatal stroke diseases with known thrombophilias. Children with idiopathic magnetic resonance imaging-classified neonatal arterial ischemic stroke (NAIS), arterial presumed perinatal ischemic stroke (APPIS), or fetal periventricular venous infarction (PVI) were recruited. Standardized thrombophilia evaluations were performed after 12 months of age on stroke cases and controls, including quantified proteins C and S, antithrombin, factors VIII/IX/XI, fibrinogen, lipoprotein(a), homocysteine, lupus anticoagulant, anticardiolipin antibodies and genotyping of factor V Leiden (FVL), factor II G20210A (FII), and methylenetetrahydrofolate reductase C677T. A total of 212 children were studied: 46 with NAIS, 34 with APPIS, 55 with PVI, and 77 controls (male, 53%; median age, 4.8 years). Of 14 parameters, no differences were observed in 12, including all common thrombophilias. Mean prothrombin time was shorter in arterial strokes (P < .001). Rates of antiphospholipid antibodies were low, comparable to those in controls, and resolved on repeat testing. FVL and FII rates were comparable to population norms. Total number of possible abnormalities did not differ between cases and controls. Our prospective, population-based, controlled, disease-specific study suggests minimal association between perinatal stroke and thrombophilia. This does not exclude the possibility of disordered coagulation at the time of stroke but suggests testing in childhood is not indicated. (Blood. 2017;129(20):2793-2800 Medscape Continuing
BackgroundDaratumumab (DARA) is a humanized Immunoglobulin G(IgG)1‐kappa monoclonal antibody against CD38 antigen that is shown to improve outcomes in relapsed/refractory plasma cell myeloma (PCM) patients. Since CD38 is expressed by different hematopoietic elements, DARA has the potential to interfere with flow cytometric assessment of bone marrow specimens.MethodsFlow cytometric analysis of bone marrow samples from 10 PCM on DARA and 5 control samples was performed using two different antibody panels.ResultsBone marrow samples from PCM patients on DARA exhibited a population of CD19+ CD10+ B‐lymphoid cells with kappa light chain restriction. Further morphological and immunophenotypic studies suggested that this population represents marrow hematogones. Marrow hematogones from control samples showed normal immunophenotypic profiles.ConclusionDARA on the surface of hematogones interferes with flow cytometric clonality study leading to artifactual kappa light chain restriction, which can result in false interpretation of a concurrent clonal B‐cell proliferation. In the era of rapidly growing list of therapeutic monoclonal antibodies, flow cytometry pathologists should be aware of potential interferences to avoid misdiagnosis. © 2019 International Clinical Cytometry Society
High-grade B cell lymphomas with MYC and BCL2 and/or BCL6 rearrangements ("double-hit" lymphoma (DHL)/"triple-hit" lymphoma (THL)) are mature B cell neoplasms with highly aggressive clinical behavior and poor response to therapy. Lymphoblastic transformation of mature B cell neoplasms is an uncommon event that is best recognized for follicular lymphoma (FL). To our knowledge, only one case of "B lymphoblastic" transformation of de novo DHL has been reported in the literature. Here, we describe another case of such transformation. The patient was a 57-year-old man who presented with hypercalcemia, high lactate dehydrogenase (LDH), and multiple lytic bone lesions and was diagnosed with high-grade B cell lymphoma with MYC and BCL2 rearrangements. The neoplastic cells were positive for CD45, CD19, CD20, CD79a, PAX5, CD10, BCL6, and BCL2 and negative for CD34, CD99, and terminal deoxynucleotidyl transferase (TdT). The patient received chemotherapy followed by autologous stem cell transplant and achieved complete remission. Nine months after the initial presentation, he developed general weakness and found to have cytopenias and circulating blasts. Bone marrow examination revealed extensive involvement by a high-grade B cell neoplasm co-expressing PAX5, CD10, and BCL2, in addition to precursor markers TdT and CD99. There was loss of CD20, CD79a, and BCL6 expression, and CD34 remained negative. A diagnosis of B lymphoblastic leukemia/lymphoma (B-LBL) was established. Similar MYC and BCL2 rearrangements were identified. IGH gene rearrangement studies confirmed clonal relatedness. The patient passed away 3 days after bone marrow examination. This case represents an extremely rare case of DHL transformation to B-LBL.
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