Disorders of WBCs and lymphoid tissues Flashcards
Leukocyte development begins with the myeloid and lymphoid stem cells in the
bone marrow
_________, which are the granulocytic precursor cells, have round to oval nuclei, with delicate chromatin and a blue to gray cytoplasm.
myeloblasts
During the next stage of development, the myeloblasts are transformed into ___________ with similar nuclei, but with a cytoplasm containing many primary granules. In the subsequent metamyelocyte stage, the nuclei distort and become arclike, producing the band developmental stage.
promyelocytes
The number of leukocytes, or white blood cells, in the peripheral circulation normally ranges from _____ to ______
5000-10000
_________ describes a decrease in the absolute number of leukocytes in the blood.
leukopenia
Although leukopenia may affect any of the specific types of white blood cells, it most often affects the
neutrophils
________ constitute the majority of blood leukocytes and play a critical role in host defense mechanisms against infection. They migrate to sites of infection and engulf, digest, and destroy microorganisms. Thus a decrease in the number of these places a person at risk for infection.
neutrophils
The ANC is supposed to be 1000/µL, and if the ANC is less than ____ cells/mm3, the person is generally put on neutropenic precautions in the hospital to protect him or her from the environment.
500
____________ denotes a virtual absence of neutrophils
agranulocytosis
__________ ______ neutropenia is neutropenia that occurs after transplacental transfer of maternal alloantibodies directed at an infant’s neutrophils, analogous to Rh hemolytic disease. The disorder usually involves phagocytic destruction of antibody-coated neutrophils by splenic macrophages. Affected infants may present with delayed separation of the umbilical cord, mild skin infection, fever, and pneumonia within the first 2 weeks of life.
alloimmune neonatal
______ or ______ neutropenia is a rare autosomal dominant disorder with variable expression that begins in infancy and persists for decades. The disorder arises from a regulatory abnormality involving early hematopoietic precursors and is associated with the neutrophil elastase (a protease that degrades virulent factors in bacteria) gene.7 It is characterized by regular, periodic oscillations of peripheral neutrophils from normal to neutropenic values every 18 to 24 days.
periodic or cyclic
Severe congenital neutropenia, or __________ ________, is characterized by an arrest in myeloid maturation at the promyelocyte stage of development that can be inherited as either an autosomal dominant or autosomal recessive trait.
Kostmann syndrome
The disorder is characterized by severe bacterial infections. Before the GM-CSFs became available for clinical use, two thirds of children died of fatal infections before reaching adolescence. Approximately 20% of people with the disorder develop acute myelogenous leukemia (AML)
lost mann syndrome (severe congenital neurropenia)
Primary autoimmune neutropenia is a rare disorder of early childhood, during which a moderate to severe neutropenia is observed. The condition is usually benign, with mild to moderate infections for children. The disorder is rare in adults. Because primary autoimmune neutropenia is self-limiting, treatment is usually restricted to the use of
antimicrobial agents to reduce risk of recurrent infections
Secondary immune-associated neutropenia is often associated with systemic autoimmune disorders, mainly (2)
RA and SLE
______ _______ a variant of RA, is a triad of splenomegaly, recurrent pulmonary infections, and neutropenia.11 The neutropenia is the result of antineutrophil antibodies and high levels of circulating immune complexes, which induce neutrophil apoptosis. Coupled with the end-organ manifestations of RA, the majority of people with this disorder are susceptible to serious bacterial infection that sometimes leads to sepsis and poor clinical outcome.
felty syndrome
Several antibody-mediated mechanisms are believed to be responsible for the neutropenia seen in people with _____. These include the development of antineutrophil antibodies, along with increased neutrophil apoptosis and decreased neutrophil production by the bone marrow.
SLE
Many different types of infectious diseases, including viral, bacterial, rickettsial, and parasitic, may cause neutropenia, the most common being
viral
Infections may produce neutropenia in multiple ways such as decreased neutrophil production, loss of neutrophils by toxins, or problems resulting in neutrophil sequestration in the
spleen
Neutropenia is also a common manifestation of ____, in which a virus-induced suppression of marrow cell proliferation is often aggravated by infectious consumption of neutrophils and by antiviral drugs
AIDS
The risk for development of chemotherapy-induced neutropenia is influenced by patient- and treatment-related disease, oncological disease, and other comorbidities. One of the most important patient-related factors is
age
Older adults are at greater risk than younger people because of age-related cellular changes in neutrophils.
Neutrophils provide the first line of defense against organisms that inhabit the ___ and ____ _____. Thus early signs of infection due to neutropenia, particularly those associated with a mild to moderate decrease in neutrophils, include mild skin lesions, stomatitis, pharyngitis, and diarrhea. Signs and symptoms of more severe neutropenia include malaise, chills, and fever, followed in sequence by marked weakness and fatigability
skin and GI tract
________ _______ is a self-limiting lymphoproliferative disorder. Eighty-five percent of the time it is caused by the Epstein-Barr virus (EBV), a member of the herpesvirus family.
infectious mononucleosis
Infectious mononucleosis may occur at any age but occurs principally in _______ and young adults in developed countries. In areas of the world where children often live in crowded conditions, asymptomatic infection with EBV occurs in childhood and infectious mononucleosis is not encountered.
adolescents
The B cells that harbor the EBV genome proliferate in the circulation and produce the well-known _______ antibodies that are used for the diagnosis of infectious mononeucleosis
heterophil
Most important in controlling the proliferation of EBV-infected B cells are the CD8+ cytotoxic T cells and NK cells. These virus-specific ____ cells appear as large, atypical lymphocytes that are characteristic of the infection
T
Although infected B cells and free virions disappear from the blood after recovery from mono, the virus remains in a few transformed B cells in the oropharyngeal region and is shed in the ______. Once infected with the virus, people remain asymptomatically infected for life, and a few such people intermittently shed EBV.
saliva
The incubation period lasts 4 to 8 weeks. A prodromal period, which lasts for several days, follows and is characterized by malaise, anorexia, and chills. The prodromal period precedes the onset of fever, pharyngitis, and lymphadenopathy. Occasionally, the disorder comes on abruptly with a high fever. Most people seek medical attention for severe pharyngitis, which usually is most severe on days 5 to 7 and persists for ___ to ___ days
7-14
Hepatitis and splenomegaly are common manifestations of the disease and are thought to be immune mediated. Hepatitis is characterized by hepatomegaly, nausea, anorexia, and jaundice. Although discomforting, it usually is a benign condition that resolves without causing permanent liver damage. The spleen may be enlarged two to three times its normal size, and rupture of the spleen is an infrequent complication in what condition
mono
There is evidence of ____ infection in essentially all people with Burkitt lymphoma, which is endemic to some parts of Africa.4 A second virus, the human T-cell lymphotropic virus (HTLV-1), which is endemic in the southwestern islands of Japan, has been associated with adult T-cell leukemia/lymphoma
EBV
The NHLs are also seen with increased frequency in people infected with ____, in those who have received chronic immunosuppressive therapy after organ transplantation, and in people with acquired or congenital immunodeficiencies.
HIV
Although the NHLs can originate in any of the lymphoid tissues, they most commonly originate in the
lymphnodes
Follicular lymphomas are derived from germinal center B cells and consist of a mixture of centroblasts and centrocytes (Fig. 28.7). Follicular lymphomas are a particularly common neoplasm in the United States, where they constitute about one third of all adult NHLs, with a peak incidence at ___ years of age
60
Diffuse large B-cell lymphomas are a heterogeneous group of aggressive germinal or postgerminal center neoplasms. The disease occurs in all age groups but is most prevalent between ___ and ___ years of age. The cause of diffuse large B-cell lymphoma is unknown but may involve EBV or HIV infections.
60 and 70
_______ _________ one of the most rapidly growing tumors of the NHLs, is also a disorder of germinal center B cells. it is the most common childhood cancer (peak age 3 to 7 years) in Central Africa, often beginning in the jaw. It occurs in regions of Africa where both EBV and malarial infection are common. Virtually 100% of people with African Burkitt lymphoma have evidence of previous EBV infection.
burkitt lymphoma
Hodgkin lymphoma is a specialized form of lymphoma that features the presence of an abnormal cell called a
reed-sternberg cell
The ____ are multicentric in origin and spread early to various lymphoid tissues throughout the body, especially the liver, spleen, and bone marrow.
NHLs
____ is characterized by painless and progressive enlargement of a single node or group of nodes. It is believed to originate in one area of the lymphatic system and, if unchecked, spreads throughout the lymphatic network.
HL
The leukemias are malignant neoplasms of the hematopoietic ______ cells that originate in the bone marrow. They are classified according to cell type (i.e., lymphocytic or myelocytic) and whether the disease is acute or chronic
precursor
The _________ leukemias involve immature lymphocytes and their progenitors that originate in the bone marrow but infiltrate the spleen, lymph nodes, CNS, and other tissues.
lymphocytic
The _________ leukemias involve the pluripotent myeloid stem cells in bone marrow and interfere with the maturation of all blood cells, including the granulocytes, erythrocytes, and thrombocytes.
myelogenous
this type of leukemia most commonly effects children
ALL
this type of leukemia primarily effects adults
AML
The _____ leukemias have a sudden onset with symptoms of depressed bone marrow function (anemia, fatigue, bleeding, and infections); bone pain; and generalized lymphadenopathy, splenomegaly, and hepatomegaly.
acute
The ______ leukemias, which largely affect adults, have a more insidious onset.
chronic
this type of chronic leukemia often has the most favorable clinical course, with many persons living long enough to die of other, unrelated causes.
CLL
________ ________ is 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.
multiple myeloma
The main sites involved in multiple myeloma are the (2)
bone and bone marrow
In addition to the abnormal proliferation of marrow plasma cells, there is proliferation and activation of osteoclasts that leads to bone resorption and destruction, and predisposes to increased risk for pathologic fractures and development of hypercalcemia. Paraproteins secreted by the plasma cells may cause hyperviscosity of body fluids and may break down into amyloid, a proteinaceous substance deposited between cells that can cause heart failure and neuropathy. Bone marrow involvement leads to increased risk of infection due to suppressed humoral and cell-mediated immunity and anemia due to impaired red cell production.
what condition?
multiple myeloma
