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Psychosocial Aspects Of Hematologic Disorders

Major advances in cancer diagnostics and therapeutics have led to increased survival and improved health outcomes for people diagnosed with hematologic malignancies. However, high levels of psychological distress (e.g., depression) and diminished positive psychological well-being factors (e.g., optimism) exist in this population for myriad reasons, including the cancer diagnosis itself, uncertain prognosis, prolonged treatment course that entails numerous hospitalizations with extended periods of isolation, and potential…

Identification and Management of Checkpoint Inhibition Toxicity

Checkpoint inhibitors revolutionized cancer therapy and the field of oncology when the first drug of this class was introduced for melanoma treatment. But their therapeutic benefit comes at a cost of immunotherapy-related adverse events (IrAEs). Checkpoint inhibitors are immunomodulatory antibodies that are used to activate the immune system to detect and destroy malignant cells. Several immune checkpoint receptors are targeted by different immunotherapy agents, such as…

Grading and Toxicity Management after Immune Effector Therapy

The growing use of novel immunotherapies has revolutionized the management of hematologic malignancies over the past several years. While historically cytotoxic chemotherapy has been the standard treatment approach, newer cellular therapies have demonstrated promising activity in patients with relapsed and refractory hematologic malignancies and have significantly improved outcomes in this setting. The increased use of these innovative therapies has highlighted a set of unique toxicities which…

Principles of Radiation Therapy for Hematologic Disease

The approach and utilization of radiation therapy (RT) for the treatment of hematologic malignancies have seen many changes throughout the years. Advances in imaging and technology have allowed for more precise delineation and targeting of disease. This, along with a reduction in radiation doses and treatment volume, have resulted in increased sparing of normal tissues and dramatically reduced short- and long-term toxicities previously associated with RT.…

Key Considerations for Managing Infections in the Compromised Host

Advances in the supportive care during the treatment of hematologic malignancies have improved the life expectancy of afflicted patients. However, this progress is increasingly at the expense of developing a wider range of infectious complications caused by drug-resistant organisms. The clinical approach to infections occurring among hematology patients involves consideration of host immune system defects, and skin or mucosal barrier disruptions that predispose patients to infection…

Immunoglobulin Light-Chain Amyloidosis (Primary Amyloidosis)

Amyloidosis is defined as the clinical syndrome associated with deposition of amyloid. Amyloid in tissue is defined by its tinctorial properties of a homogeneous, eosinophilic, hyaline material when viewed by hematoxylin and eosin staining. Amyloid stains specifically with Congo red, demonstrating a deep pink amorphous composition. Although Congo red staining is the sine qua non for the diagnosis of amyloid, many pathology laboratories prefer the use…

Waldenström Macroglobulinemia/Lymphoplasmacytic Lymphoma

Waldenström macroglobulinemia (WM) is a lymphoid neoplasm resulting from the accumulation, predominantly in the bone marrow (BM), of a clonal population of lymphocytes, lymphoplasmacytic cells, and plasma cells, which secrete a monoclonal immunoglobulin (Ig)M. WM corresponds to lymphoplasmacytic lymphoma (LPL) as defined in the World Health Organization classification system. Most cases of LPL are WM; less than 5% of cases secrete IgA, IgG, kappa, lambda, or…

Multiple Myeloma

Clonal plasma cell (PC) neoplasms are comprised of a number of interrelated and partially overlapping entities, ranging from benign to fully malignant, which are variously defined and categorized by burden of disease, presence of organ injury, anatomic location, and in some cases, unique constellations of signs, symptoms, and pathologic findings (see box on Rare Disorders Associated With Monoclonal Proteins and/or Plasma Cells , and also Chapter…

Monoclonal Gammopathy of Undetermined Significance and Smoldering Multiple Myeloma

Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant clonal plasma cell disorder. It is characterized by the presence of a monoclonal (M) protein in the serum or urine that is commonly discovered during laboratory work-up of a variety disorders. MGUS is a precursor to various hematologic disorders including multiple myeloma (MM), Waldenström macroglobulinemia (WM), solitary plasmacytoma, and immunoglobulin light chain amyloidosis (AL). Smoldering MM (SMM)…

T-Cell Lymphomas

T-cell lymphomas are a highly heterogeneous group of diseases sharing common origin from mature T-lymphocytes. Heterogeneity is appreciated on biologic and clinical grounds, both in terms of presentation (leukemic, nodal, extranodal, and cutaneous forms) and clinical behavior (aggressive versus indolent). Historically, peripheral T-cell lymphomas (PTCL) have always been separated by cutaneous T-cell lymphomas (CTCL), mostly because the former has a nodal and aggressive presentation, while the…

Malignant Lymphomas in Childhood

Malignant lymphomas are the third most common malignancy among children and adolescents. Among children less than 15 years of age, non-Hodgkin Lymphoma (NHL) is more frequent; however, in patients up to 18 years of age, Hodgkin lymphoma (HL) is predominant. NHLs in children are usually extranodal diffuse high-grade tumors, whereas low- and intermediate-grade nodal lymphomas predominate in adults. These differences are speculated to reflect maturational changes…

Virus-Associated Lymphoma

There are five well-characterized human viruses that are generally accepted as important in lymphomagenesis ( Table 87.1 ). These viruses may infect tumor cells (or their progenitors) or may act at a distance. The genomes of Epstein-Barr virus (EBV), Kaposi sarcoma–associated herpesvirus (KSHV, also known as human herpesvirus 8 [HHV-8]), and human T-lymphotropic virus-1 (HTLV-1) are present in tumor cells. The viral genes expressed in tumor…

Mantle Cell Lymphoma

Mantle cell lymphoma (MCL) is a distinct subtype of mature B-cell non-Hodgkin lymphoma (NHL) that accounts for 5% to 10% of all NHL. Although a subset of patients with MCL may have an indolent course, MCL is generally an aggressive NHL. In the 1970s, the Kiel classification used the terminology centrocytic lymphoma to describe MCL, whereas Berard and Dorfman categorized it as lymphocytic lymphoma of intermediate…

High-Grade B-Cell Lymphomas

High-grade lymphomas are aggressive hematopoietic malignancies derived predominantly from transformed B cells. Notable subtypes include diffuse large B-cell lymphoma (DLBCL), high-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements (HGBCL), and Burkitt lymphoma (BL). Although these entities share many clinical and biologic features, the approach to their management is different; therefore an accurate histologic diagnosis is of utmost importance. There have been several recent therapeutic…

Diffuse Large B-Cell Lymphoma of the Central Nervous System

Diffuse large B-cell lymphoma (DLBCL) of the central nervous system (CNS) accounts for an overwhelming majority (95%) of cases of primary CNS lymphoma (PCNSL). According to the 2016 report of the World Health Organization (WHO), it is defined as DLBCL arising within the brain, spinal cord, leptomeninges, or eye. The WHO definition excludes lymphomas of the dura, intravascular large B-cell lymphomas (IVLBL), secondary CNS lymphomas (SCNSLs),…

Marginal Zone Lymphomas (Extranodal/Malt, Splenic, and Nodal)

The term marginal zone refers to a histologic compartment located at the periphery of lymphoid follicles immediately outside their mantle zone. The marginal zone is especially evident in the spleen, although identical areas have been observed in other lymphoid structures, including mesenteric lymph nodes and mucosa-associated lymphoid tissue (MALT) ( Fig. 83.1 ). Ordinarily, it is composed predominantly of B cells that are slightly larger than…

Clinical Manifestations, Staging, and Treatment of Follicular Lymphoma

Non-Hodgkin lymphoma (NHL) refers to all malignancies of the lymphoid system with the exception of Hodgkin lymphoma. Development of the lymphoid system is a highly regulated process, characterized by differential expression of a number of cell-surface and intracytoplasmic proteins and antigen receptor gene rearrangements, somatic hypermutation, and class switching. Dysregulation of this orderly process can result in humoral deficiency, autoimmunity, or malignancy. The indolent B-cell lymphomas…

Origin of Non-Hodgkin Lymphoma and Therapeutic Targets

Over the past two decades, discoveries in basic immunology and the pathogenesis of malignancies have significantly advanced our understanding of the origin of lymphoid neoplasms. These diseases have been reexamined and grouped based on recurrent chromosomal rearrangements, histologic patterns, gene expression profiles, and more recently by patterns of gene mutations, gene fusions, and epigenetic alterations. The multiple revisions to the World Health Organization’s classification schemes for…

Hodgkin Lymphoma

Hodgkin lymphoma (HL) is an uncommon lymphoproliferative malignancy arising from B cells. It can affect all age groups but is most common in young adults. HL is the first adult malignancy to demonstrate the curative potential of combination chemotherapy. Today, more than 80% of patients with newly diagnosed HL can now expect to be cured of their disease. The challenge now, particularly since many affected patients…

Origin of Hodgkin Lymphoma and Therapeutic Targets

More than 150 years ago, Thomas Hodgkin described several cases of a lymphoproliferative disease, later named Hodgkin disease. For a long time this malignancy, now called Hodgkin lymphoma (HL), has been one of the most enigmatic forms of lymphomas. This is due to several key features of the disease: First, the pathognomonic and suspected tumor cells of HL, the mononuclear Hodgkin and the bi- or multinucleated…