Under normal conditions, the red blood cell (RBC) mass in humans is tightly controlled and remains relatively constant in a given individual. The numbers of senescent RBCs lost daily are replaced by newly formed ones by a carefully controlled network…
Introduction and Historical Perspective Chronic myeloid leukemia (CML), a rare condition, has had a profound impact on the development of hematology/oncology and modern medicine as a whole ( Fig. 69.1A and B ). The story started in the 1830s with…
Acute lymphoblastic leukemia (ALL) is a heterogeneous group of diseases characterized by clonal proliferation of lymphoid progenitors (lymphoblasts). Improved diagnostic tools permit accurate and prompt diagnosis and aid in evaluation of minimal residual disease (MRD). There have been significant advances…
Serial risk-directed clinical trials have optimized the combination of chemotherapeutic agents and, along with advances in supportive care, have led to current cure rates of childhood acute lymphoblastic leukemia (ALL) exceeding 85% compared with those of less than 10% in…
Introduction Normal lymphoid precursors undergo somatic recombination at their immunoglobulin (Ig) or T-cell receptor (TCR) gene loci, and the successful completion of V(D)J recombination, with the resultant formation of a functional Ig or TCR, is required for the survival of…
Myelodysplastic syndromes (MDS) and myeloproliferative neoplasms (MPN) are a heterogeneous group of clonal stem cell disorders that result in ineffective hematopoiesis and an increased risk of developing acute myeloid leukemia (AML). Whereas in MDS ineffective hematopoiesis results in progressive cytopenias,…
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a hematopoietic-derived malignancy thought to occur by transformation of plasmacytoid dendritic cells (pDCs) or pDC progenitors. Normal pDCs are immune cells that recognize microbes via Toll-like receptors and are the principal producers of…
Acute myeloid leukemia (AML) is a complex and heterogeneous group of malignancies in which genetic and epigenetic alterations lead to differentiation arrest and/or uncontrolled expansion of myeloid cell precursors. Pediatric AML is characterized by a wide array of genetic aberrations…
Recent advances in molecular diagnostics continue to shed light on genetic underpinnings of myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). However, with notable exceptions, both diseases remain therapeutic challenges. Fortunately, our ability to provide more precise prognostic information has…
The myelodysplastic syndromes (MDS) include a group of clonal hematopoietic disorders characterized by ineffective hematopoiesis and blood cytopenias, abnormal blood and bone marrow cell morphology ( Fig. 61.1 ), and a risk of clonal evolution including progression to acute myeloid…