Diseases of White Blood Cells and Lymph

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Chapter 15Disorders of White Blood Cells and Lymphoid Tissues : 

Chapter 15Disorders of White Blood Cells and Lymphoid Tissues

Objectives : 

Objectives 1. Describe the different types of white blood cells and structures of the lymphoid system where they circulate and mature 2. Trace the development of the different white blood cells from their origin in the pluripotent bone marrow stem cell to their circulation in the bloodstream 3. Define the terms leukopenia, neutropenia, granulocytopenia, and aplastic anemia 4. Cite two general causes of neutropenia 5. Describe the mechanism of symptom production in neutropenia

Objectives : 

Objectives 6. Use the concepts regarding the central and peripheral lymphoid tissues to describe the site of origin of the malignant lymphomas, leukemias, and plasma cell dyscrasias. 7. Explain how changes in chromosomal structure and gene function can contribute to the development of malignant lymphomas, leukemias, and plasma cell dyscrasias. 8. Contrast and compare the signs and symptoms of non-Hodgkin and Hodgkin lymphomas 9. Describe the measures used in treatment of non-Hodgkin and Hodgkin lymphomas

Objectives : 

Objectives 10. Use the predominant white blood cell type and classification of acute or chronic to describe the four general types of leukemia 11. Explain the manifestations of leukemia in terms of altered cell differentiation 12. Describe the following complications of acute leukemia and its treatment: leukostasis, tumor lysis syndrome, hyperuricemia, and blast crisis 13. Relate the clonal expansion of immunoglobulin-producing plasma cells and accompanying destructive skeletal changes that occur with multiple myeloma in terms of manifestations and clinical course of the disorder.

Formed Elements of Blood : 

Formed Elements of Blood Red blood cells Platelets (megakaryocytes) Granulocytes (i.e., neutrophils, eosinophils, and basophils) Monocyte and macrophage lineage Both are derived from the myeloid stem cell in the bone marrow and circulate in the blood Lymphocytes T lymphocytes (T cells) and B lymphocytes (B cells) originate in the bone marrow and migrate between the blood and the lymph Learning objective 1

Hematopoietic Tissue : 

Hematopoietic Tissue Myeloid tissue Bone marrow Lymphoid tissue Nodes Thymus Spleen Learning objective 1

Hematopoiesis : 

Hematopoiesis White blood cells are formed partially in the bone marrow and partially in the lymph system. They are formed from hematopoietic stem cells that differentiate into committed progenitor cells. These in turn develop into the myelocytic and lymphocytic lineages needed to form white blood cells. RBCs and platelets are formed in the marrow and released into circulation.

Growth and Reproduction of White Blood Cells : 

Growth and Reproduction of White Blood Cells The growth and reproduction of different stem cells is controlled by multiple hematopoietic growth factors or inducers. The life span of WBCs is relatively short; constant renewal is necessary to maintain normal blood levels. Conditions that decrease availability of stem cells or hematopoietic growth factors produce a decrease in WBCs. Learning objective 3

Diseases of Deficient Blood Cell Production : 

Diseases of Deficient Blood Cell Production Leukopenia deficiency of leukocytes Neutropenia Granulocytopenia Aplastic anemia Anemia, thrombocytopenia, and agranulocytosis Learning objective 3

Causes of Neutropenia : 

Causes of Neutropenia Accelerated removal Drug-induced granulocytopenia Periodic or cyclic neutropenia Neoplasms involving bone marrow Idiopathic neutropenia occurring in the absence of other disease or provoking influence Felty syndrome Learning objective 4

Signs and Symptoms of Neutropenia : 

Signs and Symptoms of Neutropenia Initially, those of bacterial or fungal infections Malaise Chills Fever Extreme weakness and fatigue Reduced white blood cell count Learning objective 5

Infectious Mononucleosis : 

Infectious Mononucleosis Definition Self-limited lymphoproliferative disorder Causes and characteristics Caused by the B-lymphocytotropic Epstein-Barr virus, a member of the herpes virus family; transmitted in saliva Characterized by fever, generalized lymphadenopathy, sore throat, and the appearance in the blood of atypical lymphocytes and several antibodies Highest incidence in adolescents and young adults Treatment is symptomatic and supportive

Question : 

Question The suffix –penia refers to a _____________ in number. Increase Stasis Decrease

Answer : 

Answer Increase Stasis Decrease: In hematology, neutropenia, granulocytopenia, and other terms with this suffix refer to decreased circulating cells.

Neoplastic Disorders of Hematopoietic and Lymphoid Origin : 

Neoplastic Disorders of Hematopoietic and Lymphoid Origin Represent the most important of the white cell disorders Include somewhat overlapping categories Lymphomas (Hodgkin disease and non-Hodgkin lymphoma) Leukemias Plasma cell dyscrasias (multiple myeloma) Learning objective 6

Clinical Features of Neoplasms : 

Clinical Features of Neoplasms Largely determined by Their site of origin The progenitor cell from which they originated The molecular events involved in their transformation into a malignant neoplasm Chromosomal aberrations Deletions Polyploidy Translocations Hyperploidy Inversions Learning objective 7 & 8

Hodgkin Disease and Non-Hodgkin Disease : 

Hodgkin Disease and Non-Hodgkin Disease Hodgkin disease Lymphoma arises in a single node or chain of nodes Reed-Sternberg cells are present Non-Hodgkin disease Lymphoma originates at extranodal sites and spreads to anatomically contiguous nodes Reed-Sternberg cells are not present Reed-Sternberg cells are derived from B lymphocytes. Learning objective 8

Symptoms of Hodgkin Disease : 

Symptoms of Hodgkin Disease Stage A Lacks constitutional symptoms Stage B (40% of persons with Hodgkin disease) Significant weight loss, fevers, pruritus, or night sweats Advanced stages Fatigue and anemia Liver, lungs, digestive tract, and CNS may be involved

Diagnosis of Hodgkin Disease : 

Diagnosis of Hodgkin Disease Reed-Sternberg cells present in a biopsy specimen of lymph node tissue Computed tomography (CT) scans of the chest and abdomen to assess for involvement of mediastinal, abdominal, and pelvic lymph nodes Bipedal lymphangiogram to detect structural changes in the lymph nodes too small to visualize on CT scan Positron emission tomography (PET) imaging Bilateral bone marrow biopsy may be performed if disease is disseminated.

Categories of Non-Hodgkin Lymphomas : 

Categories of Non-Hodgkin Lymphomas Low-grade lymphomas Predominantly B cell tumors Intermediate-grade lymphomas Include B cell and some T cell lymphomas High-grade lymphomas Largely immunoblastic (B cell), lymphoblastic (T cell), Burkitt and non-Burkitt lymphomas Learning objective 9

Staging of Non-Hodgkin Lymphomas : 

Staging of Non-Hodgkin Lymphomas Bone marrow biopsy Blood studies Chest and abdominal CT scans Nuclear medicine studies Cytologic examination of the cerebrospinal fluid Learning objective 9

Treatment for Hodgkin Disease and NHL : 

Treatment for Hodgkin Disease and NHL Depends on the histologic type, stage of the disease, and clinical status of the person Types Radiation Combination chemotherapy For NHL only Adjuvant radiation therapy Monoclonal antibodies Learning objective 9

Leukemias : 

Leukemias Definition Malignant neoplasms arising from the transformation of a single blood-cell line derived from hematopoietic stem cells Classification according to cell lineage Lymphocytic (lymphocytes) Myelocytic (granulocytes, monocytes) Learning objective 10

Leukemic Cells : 

Leukemic Cells Immature and poorly differentiated Proliferate rapidly and have a long life span Do not function normally Interfere with the maturation of normal blood cells Circulate in the bloodstream Cross the blood–brain barrier Infiltrate many body organs Learning objective 10 & 11

Classification of Leukemia Types : 

Classification of Leukemia Types Acute lymphocytic (lymphoblastic) leukemia (ALL) Chronic lymphocytic leukemia (CLL) Both involve immature lymphocytes and their progenitors in the bone marrow, the spleen, lymph nodes, CNS, and other tissue Acute myelogenous (myeloblastic) leukemia (AML) Chronic myelogenous leukemia (CML) Both involve pluripotent myeloid stem cells in bone marrow and interfere with the maturation of all blood cells Learning objective 10 & 11

Warning Signs and Complications of Acute Leukemia : 

Warning Signs and Complications of Acute Leukemia Signs Fatigue Pallor Weight loss Repeated infections Easy bruising Nosebleeds Other types of hemorrhage Complications Leukostasis Tumor lysis syndrome Hyperuricemia Blast crisis Learning objective 12

Criteria for Remission of ALL and AML : 

Criteria for Remission of ALL and AML Less than 5% blasts in the bone marrow Normal peripheral blood counts Absence of cytogenetic abnormalities Return to pre-illness performance status

Factors Affecting the Likelihood of Achieving Remission : 

Factors Affecting the Likelihood of Achieving Remission Age (most significant prognostic variable) Type of leukemia Stage of the disease at time of presentation

Chronic Leukemias : 

Chronic Leukemias Definition Malignancies involving the proliferation of well-differentiated myeloid and lymphoid cells Types of chronic leukemia Chronic lymphocytic leukemia (CLL) Chronic myelogenous leukemia (CML)

Goals of Treatment for CML : 

Goals of Treatment for CML Hematological response characterized by normalized blood counts Cytogenetic response demonstrated by the reduction or elimination of the Ph chromosome from the bone marrow Molecular response confirmed by the elimination of the BCR-ABL fusion protein

Multiple Myeloma : 

Multiple Myeloma Definition: a plasma cell dyscrasia characterized by expansion of a single clone of immunoglobulin-producing plasma cells and a resultant increase in serum levels of a single monoclonal immunoglobulin or its fragments Main sites involved: bones and bone marrow Proliferation and activation of osteoclasts that leads to bone resorption and destruction - Pathologic fractures - Hypercalcemia Learning objective13

Question : 

Question Which of the following is not a chromosomal aberration? Deletions Polyploidy Translocations Regression Hyperploidy

Answer : 

Answer Deletions Polyploidy Translocations Regression: Regression is not a type of chromosomal change. Hyperploidy

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