Disorders of WBC & Lymphoid tissues Flashcards
Hematopoiesis
*WBCs are formed partially in the bone marrow and partially in the lymph system
-formed from hematopoietic stem cells that differentiate into committed progenitor cells -> turns into myelocytic and lymphocytic lineages needed to form WBC
RBCs and platelets are formed in…
the marrow and released into circulation
Growth and reproduction of WBCs
*controlled by multiple hematopoietic growth factors or inducers
*life span is short, constant renewal is necessary to maintain normal blood levels
*conditions that decrease stem cells or hematopoietic growth factors produce a decrease in WBCs
Diseases of deficient blood cell production
*Leukopenia deficiency of leukocytes
-neutropenia
-granulocytopenia
*Aplastic anemia
-anemia, thrombocytopenia, and agranulocytosis
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
Signs and symptoms of neutropenia
*Initially, those of bacterial or fungal infections
-malaise
-chills
-fever
-extreme weakness and fatigue
*reduced WBC count
Infectious mononucleosis
self-limited lymphoproliferative disorder
Causes of infectious mononucleosis
*caused by the B-lymphoproliferative EBV, a member of the herpes virus family ; transmitted in saliva
characteristics of infectious mononucleosis
*Characterized by fever, genialized lymphadenopathy, sore throat, and the appearance in the blood of atypical lymphocytes and several antibodies
-highest incidence in adolescent children and young adults
-treatment is symptomatic and supportive
Neoplastic disorder of hematopoietic and lymphoid origin
*represent the most important of white cell disorders
include somewhat overlapping categories
*Lymphomas
*Leukemia
*Plasma cell dyscrasias (multiple myeloma)
clinical features of neoplasms
*largely determined by
-their site origin
-the progenitor cell from which they originated
-the molecular events involved in their transformation into a malignant neoplasm
Chromosomal aberrations
deletions, polyploidy, translocations, hyper ploidy, inversions
Hodgkin disease
*lymphoma arises in a single node or chain of nodes
-Reed-Sternberg cells are present (cells derived from B lymphocytes)
Non-Hodgkin disease
*lymphoma originates at extranodal sites and spread to anatomically contiguous nodes
-Reed-Sternberg cells are not present
Symptoms of Hodgkin disease
*Stage A- lack of constitutional symptoms
*Stage B- (40% of persons with Hodgins disease) significant weight loss, fevers, pruritis, or night sweats
*Advanced stages- fatigue and anemia;
liver, lungs, digestive tract , and CNS may be involved
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
Diagnosis of Hodgkin Disease
*Reed-Sternberg cell present in a biopsy specimen of lymph node tissue
*CT scans of the chest and abdomen to assess for involvement of mediastinal, abd, and pelvic lymph nodes
*A bipedal lymphangiogram to detect structural changes in the lymph nodes too small to visualize on CT scan
*A PET imaging
*A bilateral bone marrow biopsy may be performed if disease is disseminated
Stages of Non-Hodgkin lymphomas disease
*Bone marrow biopsy
*Blood studies
*Chest and abdominal CT scans
*Nuclear medicine studies
*Cytologic examination of the cerebrospinal fluid
Treatment for Hodgkin and NHL
*Depends on the histologic type, stage of the disease, and clinical status of the person
*Types
-radiation
-combo chemo
-for NHL only (adjuvant radiation therapy, monoclonal antibodies)
Leukemias
malignant neoplasms arising from the transformation of a single blood cell line derived from hematopoietic stem cells
Classifications of leukemias according to cell lineage
*lymphocytic (lymphocytes)
*Myelocytic (granulocytes, monocytes)
Chronic leukemias
*Malignancies involving the proliferation of well-differentiated myeloid and lymphoid cells
Types:
Chronic lymphocytic leukemia (CLL)
Chronic myelogenous leukemia (CML)
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 the pluripotent myeloid stem cells in bone marrow and interfere with the maturation of all blood cells
Goals of treatment for CML
*A hematological response characterized by normalized blood counts
*A cytogenetic response demonstrated by the reduction or elimination of the Ph chromosome from the bone marrow
*A molecular response confirmed by the elimination of the BCR-ABL fusion protein
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 preillness performance status
Factors affecting the likelihood of achieving remission
*Age (most significant prognostic variable)
*Type of leukemia
*Stages of the disease at time of presentation
Leukemia cells
*Are immature and poorly differentiated
*Proliferate rapidly and have a long life span
*Do not function normally
*Interfere with the mutation of normal blood cells
*Circulate in the blood stem
*Cross the blood-brain barrier
*Infiltrate many body organs
Signs of complications of acute leukemia
Fatigue, pallor, weight loss, repeated infections, easy bruising, nosebleeds, other types of hemorrhage
complications of acute leukemia
leukostasis, tumor lysis syndrome, hyperuricemia, blast crisis
Multiple myeloma
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 in multiple myeloma
*The bones and bone marrow
*Proliferation and activation of osteoclasts that lead to bone resorption and destruction
*Pathologic fractures
*Hypercalcemia
