Volume 2, Issue 1, March 2017
Opinions
Gene, Life, and Disease – A Node Theory | Page 1-13 |
X. Frank Zhao | |
Abstract The 21st century embraces a combination of the Genomic and Information Ages, characterized by enormous genetic data deposition and use of numerous powerful analytical tools. With more and more accumulated data, the concept of genes needs to be redefined and research methods further rationalized based on the properties of life: economy, optimization and survival. Disease should be considered the condition where homeostasis is disrupted. Biological equilibrium is maintained by a network of numerous functional nodes: molecules located in various pathways that are classified into decision nodes and alternative nodes, which shift their roles in response to a changing environment. Organized both spatially and temporally, the nodal network carries out its functions for the optimal outcome of the cell. Although central nodes often play vital roles for cell functions, connector nodes in the “structural hole” are more effective targets for treatment. Resources should be prioritized to delineate the roles of functional nodes and identify critical nodes for optimal therapy. |
Case Report
Diagnostic challenge: Acute leukemia with biphenotypic blasts and BCR-ABL1 translocation | Page 14-18 |
Ling Wang and Xiangdong Xu | |
Abstract Chronic myelogenous leukemia (CML) and mixed phenotype acute leukemia (MPAL) are two distinct entities according to the current WHO classification. However, the distinction can be difficult when adequate clinical history is not available, because both entities could have BCR-ABL1 translocation and B/myeloid biphenotypic blasts. Here we report a 64-year-old man with no previous history of myeloproliferative disorders who presented with marked leukocytosis and 30% circulating blasts. The bone marrow had 39% of blasts and 32% maturing myeloid cells. The blast population showed both B and myeloid differentiation. Cytogenetics revealed the BCR-ABL1 translocation as the sole abnormality. Based on one small-sized case series, many features of our case favored the diagnosis of CML in blast phase with biphenotypic blasts, including: 1) left-shifted granulocytic maturation in peripheral blood; 2) a significant portion of maturing myeloid cells in bone marrow; 3) the diagnostic marrow with higher percentage of BCR-ABL1 harboring cells than that of the blasts; 4) BCR-ABL1 translocation as the sole cytogenetic abnormality; 5) post-induction marrow showed hypercellularity. |
Case Report
CD20-positive primary cutaneous extranodal NK/T-cell lymphoma | Page 19-23 |
Xiangdong Xu, Laura Romero, Young Kim, and X. Frank Zhao | |
Abstract Cutaneous extranodal NK/T-cell lymphoma, nasal type, and primary cutaneous g/d T-cell lymphoma (PCGD-TCL) are rare cutaneous lymphoid neoplasms with overlapping features. Although EBV positivity favors the former, EBV+ PCGD-TCL has recently been reported. They also share cytotoxic markers and a poor prognosis. Here we report an unusual case of cutaneous NK/T-cell lymphoma with overlapping features of PCGD-TCL in a 55-year-old man who also developed hemophagocytic lymphohistiocytosis. Although it presents the typical pathological and immunophenotypic features of a cutaneous NK/T-cell lymphoma and PCGD-TCL, it aberrantly expresses CD20, giving an impression of mixed populations of T cells and B cells that are commonly seen in reactive conditions. This rare case represents a diagnostic pitfall and intends to raise more awareness among both pathologists and dermatologists. The CD20 positivity may also provide a target for rituximab therapy. |