The treatment of patients with hematologic malignancies has been revolutionized over the past decades as new therapeutic targets continue to be identified through cellular and molecular studies of these conditions. These investigations have spawned the discovery, clinical evaluation, and US…
Dedicated to the loving memory of Eta Najfeld, MD, a Holocaust survivor and an amazing mother . Over the past 66 years the cytogenetic analysis of hematologic malignancies has been an area of prolific growth. Chromosome studies and karyotype analysis…
Defining and classifying tumors of the hematopoietic and lymphoid tissue accurately is critical for providing optimal treatment to patients with hematologic malignancies. Explicit definitions and terminologies are prerequisites for the precise classification of hematologic malignancies. A reproducible classification that is…
The initial clinical descriptions of primary Epstein-Barr virus (EBV) infections are credited to Filatov and Pfeiffer at the end of the 19th century. Pfeiffer coined the term glandular fever , which described an illness consisting of fever, malaise, sore throat,…
Lysosomal Storage Diseases Lysosomal storage diseases (LSDs) are genetic disorders caused by deficiencies in single lysosomal hydrolases. Deficiencies result in cellular and organ damage due to subsequent accumulation of a specific substrate for that particular enzyme, hence the term “storage”…
The histiocytic disorders comprise a broad grouping of hematologic and immunologic diseases united by aberrant function, differentiation, and/or proliferation of cells of the mononuclear phagocyte system. The term histiocyte historically referred to tissue phagocytes but now more precisely defines cells…
Over 350 molecular defects that result in primary immune deficiency are known to date. Many of these gene defects have been identified in recent years with the advent of whole-exome and whole-genome sequencing. The study of patients with primary immune…
Phagocytic leukocytes are an essential component of the innate immune system that has evolved to rapidly respond to the presence of invading bacteria, fungi, and parasites. This first line of host defense also includes natural killer (NK) lymphocytes, complement, and…
Any discussion of quantitative abnormalities of lymphocytes and immunoglobulins is necessarily linked because the B-cell compartment is responsible for immunoglobulin production and the T-cell compartment helps to provide the stimulus. Nevertheless, clinicians are often consulted when a quantitative disorder of…
Abnormalities of leukocyte number are commonly encountered in medical practice. The clinical significance of leukocytosis or leukopenia varies from none at all to being an early clue to a life-threatening process, whether that is a primary hematologic or secondary reactive…