Disorders of WBCs and lymphoid tissues

Question 1

Leukocyte development begins with the myeloid and lymphoid stem cells in the

Answer 1

bone marrow

Question 2

_________, which are the granulocytic precursor cells, have round to oval nuclei, with delicate chromatin and a blue to gray cytoplasm.

Answer 2

myeloblasts

Question 3

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.

Answer 3

promyelocytes

Question 4

The number of leukocytes, or white blood cells, in the peripheral circulation normally ranges from _____ to ______

Answer 4

5000-10000

Question 5

_________ describes a decrease in the absolute number of leukocytes in the blood.

Answer 5

leukopenia

Question 6

Although leukopenia may affect any of the specific types of white blood cells, it most often affects the

Answer 6

neutrophils

Question 7

________ 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.

Answer 7

neutrophils

Question 8

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.

Answer 8

500

Question 9

____________ denotes a virtual absence of neutrophils

Answer 9

agranulocytosis

Question 10

__________ ______ 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.

Answer 10

alloimmune neonatal

Question 11

______ 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.

Answer 11

periodic or cyclic

Question 12

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.

Answer 12

Kostmann syndrome

Question 13

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)

Answer 13

lost mann syndrome (severe congenital neurropenia)

Question 14

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

Answer 14

antimicrobial agents to reduce risk of recurrent infections

Question 15

Secondary immune-associated neutropenia is often associated with systemic autoimmune disorders, mainly (2)

Answer 15

RA and SLE

Question 16

______ _______ 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.

Answer 16

felty syndrome

Question 17

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.

Answer 17

SLE

Question 18

Many different types of infectious diseases, including viral, bacterial, rickettsial, and parasitic, may cause neutropenia, the most common being

Answer 18

viral

Question 19

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

Answer 19

spleen

Question 20

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

Answer 20

AIDS

Question 21

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

Answer 21

age

Older adults are at greater risk than younger people because of age-related cellular changes in neutrophils.

Question 22

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

Answer 22

skin and GI tract

Question 23

________ _______ 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.

Answer 23

infectious mononucleosis

Question 24

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.

Answer 24

adolescents

Question 25

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

Answer 25

heterophil

Question 26

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

Answer 26

T

Question 27

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.

Answer 27

saliva

Question 28

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

Answer 28

7-14

Question 29

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

Answer 29

mono

Question 30

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

Answer 30

EBV

Question 31

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.

Answer 31

HIV

Question 32

Although the NHLs can originate in any of the lymphoid tissues, they most commonly originate in the

Answer 32

lymphnodes

Question 33

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

Answer 33

60

Question 34

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.

Answer 34

60 and 70

Question 35

_______ _________ 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.

Answer 35

burkitt lymphoma

Question 36

Hodgkin lymphoma is a specialized form of lymphoma that features the presence of an abnormal cell called a

Answer 36

reed-sternberg cell

Question 37

The ____ are multicentric in origin and spread early to various lymphoid tissues throughout the body, especially the liver, spleen, and bone marrow.

Answer 37

NHLs

Question 38

____ 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.

Answer 38

HL

Question 39

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

Answer 39

precursor

Question 40

The _________ leukemias involve immature lymphocytes and their progenitors that originate in the bone marrow but infiltrate the spleen, lymph nodes, CNS, and other tissues.

Answer 40

lymphocytic

Question 41

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.

Answer 41

myelogenous

Question 42

this type of leukemia most commonly effects children

Answer 42

ALL

Question 43

this type of leukemia primarily effects adults

Answer 43

AML

Question 44

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.

Answer 44

acute

Question 45

The ______ leukemias, which largely affect adults, have a more insidious onset.

Answer 45

chronic

Question 46

this type of chronic leukemia often has the most favorable clinical course, with many persons living long enough to die of other, unrelated causes.

Answer 46

CLL

Question 47

________ ________ 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.

Answer 47

multiple myeloma

Question 48

The main sites involved in multiple myeloma are the (2)

Answer 48

bone and bone marrow

Question 49

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?

Answer 49

multiple myeloma