Inflammatory diseases in hematology: a review

Am J Physiol Cell Physiol. 2022 Aug 8. doi: 10.1152/ajpcell.00356.2021. Online ahead of print.ABSTRACTHematopoietic cells are instrumental in generating and propagating protective inflammatory responses to infection or injury. However, excessive inflammation contributes to many diseases of the blood, bone marrow, and lymphatic system. We review three clinical categories of hematological inflammatory diseases in which recent clinical and translational advances have been made. The first category are monogenic inflammatory diseases. Genotype-driven research has revealed that previously mysterious diseases with protean manifestations are characterized by mutations which may be germline (e.g. deficiency of ADA2 or GATA2 deficiency) or somatic (e.g. VEXAS syndrome). The second category are the cytokine storm syndromes, including hemophagocytic lymphohistiocytosis and Castleman disease. Cytokine storm syndromes are characterized by excessive production of inflammatory cytokines including interleukin-6 and interferon-gamma, causing end-organ damage and high mortality. Finally, we review disorders associated with monoclonal and polyclonal hypergammaglobulinemia. The serum protein electrophoresis (SPEP) is typically ordered to screen for common diseases such as myeloma and humoral immunodeficiency. However, monoclonal and polyclonal hypergammaglobulinemia on SPEP can also provide important information in rare inflammatory diseases. For example, the autoinflammatory disease Schnitzler s...
Source: American Journal of Physiology. Cell Physiology - Category: Cytology Authors: Source Type: research