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Tumors Derived from Hematopoietic Cells and Tissue

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Tumors Derived from Hematopoietic Cells and Tissue CHAPTER 34 Tumors Derived from Hematopoietic Cells and Tissue John B. Kerrison ERYTHROCYTOSIS AND POLYCYTHEMIA VERA Non-Hodgkin’s Lymphomas General Clinical Manifestations Mycosis Fungoides (Cutaneous T-Cell Lymphoma) Diagnosis MULTIPLE MYELOMA, PLASMACYTOMAS, AND RELATED Prognosis and Treatment DISORDERS (MONOCLONAL GAMMOPATHIES) ESSENTIALTHROMBOCYTHEMIA Multiple Myeloma and Plasmacytomas AGNOGENIC MYELOID METAPLASIA (MYELOID Plasma-Cell Granulomas METAPLASIA WITH MYELOFIBROSIS, IDIOPATHIC POEMS Syndrome (Crow-Fukase Syndrome) MYELOFIBROSIS) Waldenstro¨m’s Macroglobulinemia General Considerations Heavy Chain Diseases (HCDs) Diagnosis Primary and Myeloma-Related Forms of Generalized (Systemic) Prognosis Amyloidosis LEUKEMIAS DISEASES OF THE MONONUCLEAR-PHAGOCYTIC General Considerations (RETICULOENDOTHELIAL) SYSTEM: HISTIOCYTOSIS Pathology Origin, Components, and Functions of the Cells of the Symptoms and Signs Mononuclear-Phagocytic System Therapy and Prognosis Histiocytoses LYMPHOMAS Hodgkin’s Disease Hematopoiesis is the orderly process of blood cell prolif- Megakaryocytes undergo a process of nuclear endoredupli- eration and maturation. It is a continuum characterized by cation, cytoplasmic maturation, and fragmentation to yield constant turnover of cells responsive to various stimuli in- platelets (4). Polymorphonuclear leukocytes or granulocytes cluding hypoxia, bleeding, and infection, and controlled by (neutrophils, basophils, and eosinophils) share similar pro- a number of humoral and cellular mediators (1). By such cesses of nuclear configuration and the formation of cyto- means the normal concentration of cellular elements in the plasmic granules (5,6). peripheral blood is maintained. Monocytes, macrophages, and their precursors comprise Hematopoiesis begins with the pluripotential stem cells the mononuclear phagocyte system (7,8). These cells were that are capable of differentiating into myeloid stem cells once thought to arise from reticular networks within the en- or lymphoid stem cells (Fig. 34.1) (2). Myeloid stem cells dothelium of blood vessels in several tissues (e.g., lungs, subsequently form three cell lines of colony forming units liver, spleen, and bone marrow) and were considered to con- (CFU) which, in turn, transform into the precursors of eryth- stitute the reticuloendothelial system. In fact, these cells rocytes, megakaryocytes, polymorphonuclear leukocytes, originate in the bone marrow from committed progenitors, basophils, eosinophils, and monocytes-macrophages. are released into the blood as monocytes, and migrate to Lymphoid stem cells give rise to T-lymphocytes, B-lympho- tissues where they differentiate into macrophages. Macro- cytes, and plasma cells. phages are designated by their location when fixed; they are The process of erythropoiesis, by which erythrocytes called ‘‘histiocytes’’ in the tissues, ‘‘Kupffer cells’’ in the are formed, occurs in stages, involving the humoral regu- liver, ‘‘lining cells’’ in the spleen and lymph nodes, ‘‘mesan- lator erythropoietin and the biosynthesis of hemoglobin (3). gial cells’’ in the kidney, ‘‘microglial cells’’ in the central 1607 1608 CLINICAL NEURO-OPHTHALMOLOGY Figure 34.1. Schematic outline of the progenitor basis for hematopoiesis disease. (From Nathan DG. Approach to the patient with hematologic disease. In Bennett JC, Plum F, eds. Cecil Textbook of Medicine. Philadelphia: WB Saunders, 1996Ϻ817–821.) nervous system (CNS), and ‘‘osteoclasts’’ in the bone. They tain leukemias (1). Throughout the hematopoietic process, are called ‘‘wandering macrophages’’ when they are in the from progenitor cells to mature blood cells, glycoproteins pleural, peritoneal, and pericardial cavities or the alveoli of known as hematopoietic growth factors or colony stimulat- the lung. ing factors, influence proliferation, differentiation, and sur- The lymphoid system is the other major derivative of the vival (13). The actions of human growth factors are complex pluripotential stem cell and is comprised of B-lymphocytes, and overlapping. Several human growth factor genes have T-lymphocytes, and plasma cells. Terminology was previ- ously based on cell surface morphology (9). One type of lymphocyte had a smooth surface and was thymus-derived: Table 34.1 the T-lymphocyte; the other lymphocyte had a ‘‘hairy’’ sur- Classification of the Acute Leukemias face that was covered with microvilli and was derived from the bone marrow: the B-lymphocyte. In fact, both types have Acute lymphocytic leukemia (ALL) microvilli when they are in the peripheral circulation, and Early pre-B-cell ALL both are smooth in their final tissue sites (10). Pre-B-cell ALL Lymphocyte differentiation is complex. Precursors of B- B-cell ALL lymphocytes originate in the bone marrow, spleen, and T-cell ALL lymph nodes whereas T-lymphocytes arise in the bone mar- Acute nonlymphocytic leukemia (ANLL) Acute myelocytic leukemia, minimally differentiated (AML-MO) row, travel to the thymus for further differentiation, and then Acute myelocytic leukemia without maturation (AML-M1) migrate to the marrow, spleen, and lymph nodes. Once Acute myelocytic leukemia with maturation (AML-M2) thought to develop independently, plasma cells derive from Acute promyelocytic leukemia (APL, AML-M3) lymphocytes (10,11). Acute myelomonocytic leukemia (AMMoL, AML-M4) In the embryo and fetus, hematopoietic stem cells arise Acute monocytic leukemia (AMoL, AML-M5) from the mesoderm (12), and hematopoiesis is both skeletal Acute erythroleukemia (AEL, AML-M6) and extraskeletal (1). From birth, bone marrow provides the Acute megakaryocytic leukemia (AMegL, AML-M7) principal hematopoietic microenvironment. Medullary he- Biphenotypic (mixed lineage) leukemia. matopoiesis is the rule, although normal marrow occasion- From Kinney MC, Lukens JN. Classification and differentiation of the acute ally develops outside the skeleton. Usually, however, such leukemias. In: Lee RG, Foerster J, Lukens J, et al, eds. Wintrobe’s Clinical extramedullary hematopoiesis occurs in disease, such as cer- Hematology. Baltimore: Williams & Wilkins, 1999; 2209–2240. TUMORS DERIVED FROM HEMATOPOIETIC CELLS AND TISSUE 1609 Table 34.2 Table 34.3 Chronic Myeloproliferative Disorders REAL/WHO Classification of Non-Hodgkin’s Lymphoma Polycythemia vera Indolent Essential thrombocythemia B-cell CLL/small lymphocytic lymphoma Agnogenic myeloid metaplasia Marginal zone lymphoma Chronic myelogenous leukemia MALT Splenic marginal zone lymphoma Nodal marginal zone lymphoma Lymphoplasmacytoid lymphoma/immunocytoma been cloned, their actions analyzed, and their potential thera- Follicle center lymphoma, follicular type peutic applications demonstrated (14,15). Grade I (0–5 centroblasts/hpf) Any cell involved in hematopoiesis, regardless of its de- Grade II (6–15 centroblasts/hpf) gree of differentiation, may proliferate excessively. In this Grade III (Ͼ15 centroblasts/hpf) chapter, we consider the neuro-ophthalmologic manifes- Aggressive tations of hematologic proliferative disorders, including Diffuse, large cell lymphoma Mediastinal large cell lymphoma polycythemia vera, erythrocytosis, essential thrombocyto- Primary effusion lymphoma sis, agnogenic myeloid metaplasia, leukemia, lymphoma, Mantle cell lymphoma Hodgkin’s disease, plasma cell dyscrasias, and the histio- Burkitt’s lymphoma/high-grade Burkitt’s-like cytoses. Classification of these disorders requires diagnostic Precursor B-cell leukemia/lymphoma accuracy, reproducibility, and clinical relevance, and classi- fication schemes continue to evolve due to a progressive understanding of tumor-cell physiology, immunotyping, and molecular genetic analyses. A variety of schemes have been (20). Classification of the acute leukemias is presented in used to classify hematologic malignancies including the Table 34.1, chronic myeloproliferative disorders in Table French-American-British (FAB) and World Health Organi- 34.2, and non-Hodgkin’s lymphomas in Table 34.3. It is zation (WHO) classification of acute leukemias (16,17) and beyond the scope of this text to describe all aspects of these the Working Formulation 1982 (18), Revised European- diseases in detail. The reader interested in pursuing this sub- American Classification of Lymphoid Neoplasms (REAL) ject in more detail is advised to read one of the latest texts 1994 (19), and the WHO classification of lymphomas 1999 or monographs concerning hematologic malignancies. ERYTHROCYTOSIS AND POLYCYTHEMIA VERA Erythrocytosis is characterized by elevation of the red sence of normal negative regulation of primitive progenitors blood cell count, hematocrit, and hemoglobin concentration; (27). Polycythemia is one of the chronic myeloprolifera- it may be absolute or apparent. Absolute erythrocytosis tive disorders. The others in this category include essen- results from an increase in total red cell mass and may be tial thrombocythemia, agnogenic myeloid metaplasia, and further categorized as clonal and nonclonal. Clonal erythro- chronic myelogenous leukemia (Table 34.2). cytosis results from a disorder intrinsic to the erythroid pro- Polycythemia usually occurs in later life with a median genitor cells of the bone marrow, independent of erythropoi- age at presentation of 60 years (5). Rarely, the disorder is etin, and is called polycythemia (rubra) vera. Nonclonal or seen in children (28). There is a slight male preponderance secondary erythrocytosis results from an otherwise normal and a propensity for the disease to occur with increased fre- bone marrow excessively stimulated
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