Hematology: Basic Principles and Practice

Hairy Cell Leukemia

Hairy cell leukemia (HCL) is one of the diseases exemplifying the importance of the application of appropriate diagnostic techniques and the importance of treatment strategies to obtain the best treatment outcomes for the individual patient. The disease was first described…

Chronic Lymphocytic Leukemia

Over the past several years, major advances have been realized in terms of improved understanding of the pathophysiology and therapeutic options for chronic lymphocytic leukemia (CLL). The plethora of information about CLL has increased dramatically and made management of what…

Mast Cells and Mastocytosis

Paul Ehrlich used his 1878 doctoral thesis to characterize a new cell type—the mast cell (MC)—based on its reactivity to aniline dyes and the metachromatic appearance of its cytoplasmic granules. He referred to MCs as Mastzellen and he speculated that…

Eosinophilia, Eosinophilic Neoplasms, and the Hypereosinophilic Syndromes

Eosinophils are highly specialized granulocytic effector cells that produce and store numerous biologically active mediators, including cytotoxic proteins, lipid mediators, chemotactic peptides, and cytokines ( Table 74.1 ). Under various pathologic conditions, blood eosinophils transmigrate through the endothelial layer and…

Myelodysplastic Syndrome/Myeloproliferative Neoplasm Overlap Syndromes

Myelodysplastic syndrome (MDS)/myeloproliferative neoplasm (MPN) overlap syndromes are a group of rare myeloid malignancies that paradoxically exhibit both dysplastic and proliferative hematopoietic cell characteristics. They share phenotypic features of both MDS, such as dysplasia and cytopenias, and of MPNs, including…

Primary Myelofibrosis and Chronic Neutrophilic Leukemia

Primary myelofibrosis (PMF) is a Philadelphia chromosome-negative, clonal, myeloproliferative neoplasm (MPN) that is often but not always accompanied by the driver mutations JAK2V617F , calreticulin exon 9 ( CALR ), and the thrombopoietin receptor, MPL515L/K . PMF is clinically characterized…

Essential Thrombocythemia

Essential thrombocythemia (ET) is a chronic myeloproliferative neoplasm (MPN) characterized by platelet counts in excess of 450 × 10 9 /L, profound bone marrow (BM) megakaryocyte hyperplasia, leukocytosis, splenomegaly, a clinical course punctuated by hemorrhagic and/ or thrombotic episodes, and…

The Polycythemias

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…

Chronic Myeloid Leukemia

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…