Disorders of WBC & Lymphoid tissues

Question 1

Hematopoiesis

Answer 1

*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

Question 2

RBCs and platelets are formed in…

Answer 2

the marrow and released into circulation

Question 3

Growth and reproduction of WBCs

Answer 3

*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

Question 4

Diseases of deficient blood cell production

Answer 4

*Leukopenia deficiency of leukocytes
-neutropenia
-granulocytopenia

*Aplastic anemia
-anemia, thrombocytopenia, and agranulocytosis

Question 5

Causes of neutropenia

Answer 5

-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

Question 6

Signs and symptoms of neutropenia

Answer 6

*Initially, those of bacterial or fungal infections
-malaise
-chills
-fever
-extreme weakness and fatigue
*reduced WBC count

Question 7

Infectious mononucleosis

Answer 7

self-limited lymphoproliferative disorder

Question 8

Causes of infectious mononucleosis

Answer 8

*caused by the B-lymphoproliferative EBV, a member of the herpes virus family ; transmitted in saliva

Question 9

characteristics of infectious mononucleosis

Answer 9

*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

Question 10

Neoplastic disorder of hematopoietic and lymphoid origin

Answer 10

*represent the most important of white cell disorders
include somewhat overlapping categories

*Lymphomas
*Leukemia
*Plasma cell dyscrasias (multiple myeloma)

Question 11

clinical features of neoplasms

Answer 11

*largely determined by
-their site origin
-the progenitor cell from which they originated
-the molecular events involved in their transformation into a malignant neoplasm

Question 12

Chromosomal aberrations

Answer 12

deletions, polyploidy, translocations, hyper ploidy, inversions

Question 13

Hodgkin disease

Answer 13

*lymphoma arises in a single node or chain of nodes
-Reed-Sternberg cells are present (cells derived from B lymphocytes)

Question 14

Non-Hodgkin disease

Answer 14

*lymphoma originates at extranodal sites and spread to anatomically contiguous nodes
-Reed-Sternberg cells are not present

Question 15

Symptoms of Hodgkin disease

Answer 15

*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

Question 16

Categories of Non-Hodgkin lymphomas

Answer 16

*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

Question 17

Diagnosis of Hodgkin Disease

Answer 17

*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

Question 18

Stages of Non-Hodgkin lymphomas disease

Answer 18

*Bone marrow biopsy
*Blood studies
*Chest and abdominal CT scans
*Nuclear medicine studies
*Cytologic examination of the cerebrospinal fluid

Question 19

Treatment for Hodgkin and NHL

Answer 19

*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)

Question 20

Leukemias

Answer 20

malignant neoplasms arising from the transformation of a single blood cell line derived from hematopoietic stem cells

Question 21

Classifications of leukemias according to cell lineage

Answer 21

*lymphocytic (lymphocytes)
*Myelocytic (granulocytes, monocytes)

Question 22

Chronic leukemias

Answer 22

*Malignancies involving the proliferation of well-differentiated myeloid and lymphoid cells

Types:
Chronic lymphocytic leukemia (CLL)
Chronic myelogenous leukemia (CML)

Question 23

Classification of Leukemia types

Answer 23

*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

Question 24

Goals of treatment for CML

Answer 24

*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

Question 25

Criteria for remission of ALL and AML

Answer 25

*Less than 5% blasts in the bone marrow
*Normal peripheral blood counts
*Absence of cytogenetic abnormalities
*Return to preillness performance status

Question 26

Factors affecting the likelihood of achieving remission

Answer 26

*Age (most significant prognostic variable)
*Type of leukemia
*Stages of the disease at time of presentation

Question 27

Leukemia cells

Answer 27

*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

Question 28

Signs of complications of acute leukemia

Answer 28

Fatigue, pallor, weight loss, repeated infections, easy bruising, nosebleeds, other types of hemorrhage

Question 29

complications of acute leukemia

Answer 29

leukostasis, tumor lysis syndrome, hyperuricemia, blast crisis

Question 30

Multiple myeloma

Answer 30

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

Question 31

Main sites involved in multiple myeloma

Answer 31

*The bones and bone marrow
*Proliferation and activation of osteoclasts that lead to bone resorption and destruction
*Pathologic fractures
*Hypercalcemia