Gen Path Exam 2 - WBC Disorders Flashcards
(138 cards)
1
Q
What are the 4 main compartments WBCs occupy in the body?
A
Bone marrow
Bloodstream
Lymph nodes
Site of infection or immune stimulation
2
Q
What is the site of WBC production?
A
Bone marrow
3
Q
What is the site of WBC transport?
A
Bloodstream
4
Q
What is the site of WBC immune activation?
A
Lymph nodes
5
Q
What is the site of WBC infection or immune stimulation?
A
Any organ or soft tissue
(what you see clinically)
6
Q
Decreased serum level of leukocytes
A
Leukopenia
7
Q
Elevated serum level of leukocytes, mostly neutrophils
A
Leukocytosis
8
Q
What is the normal level of neutrophils? What about during leukocytosis?
A
Normal = 4-10,000 ul
Leukocytosis = 15-20,000 ul
9
Q
Bacterial infections or when there is tissue necrosis (burns, MI)
A
Neutrophilic
10
Q
Chronic infections and some viral infections
A
Lymphocytotic
11
Q
Chronic infections
A
Monocytotic
12
Q
Allergies (asthma, hay fever), parasitic infections, drug rxns
A
Eosinophilic
13
Q
When you evaluate lymph nodes, what should they normally look like?
A
Small and non-palpable
14
Q
What do lymph nodes look like in lymphadenopathy?
A
Firm, enlarged
15
Q
Seen in the lymph node that is draining a region of infection
A
Painful lymphadenopathy
16
Q
Seen with chronic inflammation. metastatic cancer, or lymphoma
A
Non-painful lymphadenopathy
17
Q
Which disease?
Most cases are self-limited and benign, particularly in children
A
Lymphadenopathy
18
Q
Which disease?
Often localized but if generalized, there is often an underlying systemic disease
A
Lymphadenopathy
19
Q
Which disease?
<2 weeks or >1 year without size change is unlikely to be a neoplasm
A
Lymphadenopathy
20
Q
Which disease?
Risk for cancer: >6 weeks and not better by 12 weeks
A
Lymphadenopathy
21
Q
Which disease?
Workup includes serology, imaging, possible biopsy
A
Lymphadenopathy
22
Q
What are the Lymphadenopathy etiologies?
A
MIAMI - malignancy, infectious, autoimmune, miscellaneous, iatrogenic
23
Q
What type of Lymphadenopathy etiology?
Fever, drenching night sweats, unexplained weight loss >10% of body weight
A
Malignancy
24
Q
What type of Lymphadenopathy etiology?
Supraclavicular lymphadenopathy adults or children - up to 50% have intraabdominal malignancy
A
Malignancy
25
What type of Lymphadenopathy etiology?
Fever, chills, fatigue, malaise
Infectious
26
What type of Lymphadenopathy etiology?
Arthralgias, muscle weakness, rash
Autoimmune
27
What type of Lymphadenopathy etiology?
Other specific findings of each condition
Miscellaneous
28
What type of Lymphadenopathy etiology?
History of new meds
Iatrogenic
29
Decreased neutrophils in blood
Neutropenia
30
Decreased granulocytes (neutrophils, basophils, eosinophils) in blood
Agranulocytosis
31
Which disease?
Pathogenesis = decreased production in bone marrow or increased destruction in the peripheral blood
Neutropenia
Agranulocytosis
32
Which disease?
Most common sequela is infection
Neutropenia
Agranulocytosis
33
Which disease?
Symptoms are malaise, fever, chills, weakness, ulceration (oral, gingival) that is deep and punched out
Neutropenia
Agranulocytosis
34
Which disease?
Causes of decreased production in bone marrow include: chemo, aplastic anemia, leukemia
Neutropenia
Agranulocytosis
35
Which disease?
Causes of increased destruction of peripheral cells causing hypercellular marrow include: immune-mediated injury (drugs), overwhelming infections, splenomegaly
Neutropenia
Agranulocytosis
36
What causes this in neutropenia and agranulocytosis?
Transient marrow hyoplasia, decreasing neutrophil production
Chemo
37
What causes this in neutropenia and agranulocytosis?
Chronic marrow hypoplasia
Aplastic anemia
38
What causes this in neutropenia and agranulocytosis?
Replacement of normal marrow
Leukemia
39
What causes this in neutropenia and agranulocytosis?
Uses up peripheral cells
Overwhelming infections
40
What causes this in neutropenia and agranulocytosis?
Accelerates removal of granulocytes
Splenomegaly
41
Which disease?
Tx is removal of offending agent, control infection, give granulocyte colony-stimulating factor to stimulate granulocyte production
Neutropenia
Agranulocytosis
42
What is the cause of lymphoid neoplasms?
Increased risk for translocations/transformation in B cells
43
Why is there an increased risk for translocations/transformation in B cells?
B cells undergo somatic hypermutation and class switching
44
Increases AB affinity
Somatic hypermutation (B cells)
45
Produces multiple AB types to the same antigen
Class switching (B cells)
46
Which cells are genomically stable, making them an UNCOMMON cause of lymphomas?
T cells
47
Involvement of bone marrow and peripheral blood
Leukemia
48
Tumor masses in lymph nodes or other tissues
Lymphoma
49
All lymphoid neoplasms can spread where?
To lymph nodes and other tissues
(ex: spleen, bone marrow, peripheral blood)
50
What tumors come from cells arrested at or derived from a specific stage of normal lymphocyte differentiation?
B and T cell tumors
51
What cancers account for the largest proportion of cancers in children by tumor type?
Leukemia and lymphoma
52
Which disease?
Group of hematologic malignancies characterized by tumor cells that originate in bone marrow and spill over into blood
Leukemia
53
What are the 2 main mechanisms that lead to the formation of B cell lymphomas?
Somatic hypermutation
Class switching
54
Which disease?
Diffuse infiltration into lymph nodes, spleen, liver, and gingiva, causing general enlargment
Leukemia
55
Which disease?
Derived from single transformed cell exhibiting clonal growth
Leukemia
56
Which disease?
Typically, all of the clonal cell population have the same surface markers
Leukemia
57
Which disease?
Causes are ionizing radiation, toxins, antineoplastic chemo drugs, chromosomal abnormalities
Acute leukemia
58
Which disease?
Pathophysiology is myelophthisic anemia, neutropenia, anemia, thrombocytopenia
Acute leukemia
59
Replacement of normal hematopoietic cells by neoplastic "blasts" (myeloblasts, erythroblasts, and megakaryocytes) in bone marrow
Myelophthisic anemia
60
What does Myelophthisic anemia cause?
Neutropenia
Anemia
Thrombocytopenia
61
Leads to bacterial, viral, and fungal infections
Neutropenia
62
Leads to hypoxia causing fatigue, shortness of breath, and pallor (decreased RBCs)
Anemia
63
Leads to bleeding and petechiae
Thrombocytopenia
64
What do the pathophysiologic changes (myelophthisic anemia, neutropenia, anemia, and thrombocytopenia) in acute leukemia lead to?
Extramedullary hematopoiesis
65
What is the MOST COMMON cancer in children?
ALL (acute lymphoblastic leukemia)
66
Does ALL have a good outcome/cure rate?
Yes!
67
Which acute leukemia primarily affects older adults?
AML (acute myeloid leukemia)
68
Does AML have a good outcome/cure rate?
No :(
69
Which disease?
Neoplasm of mature circulating lymphocytes, causing high WBC count
Chronic leukemia
70
Which disease?
Either lymphocytic or myeloid
Chronic leukemia
71
Which disease?
Slow onset in adult pts with common symptoms like weakness/fatigue, weight loss, night sweats, swollen abdomen, infections, easy bruising
Chronic leukemia
72
Which disease?
High WBC - neutrophils
CML (chronic myeloid leukemia)
73
Which disease?
High WBC - lymphocytes
CLL (chronic lymphocytic leukemia)
74
What is the MOST COMMON leukemia of ADULTS in the western world?
CLL
75
Which chronic leukemia?
Indolent, slow growing tumor, often asymptomatic
CLL
76
Which chronic leukemia?
Increased BCL 2
CLL
77
What is BCL 2?
Anti-apoptotic protein
78
Which chronic leukemia?
If it involves lymph nodes, it is called Small Lymphocytic Lymphoma (SLL)
CLL
79
Which chronic leukemia?
Cure only achieved with hematopoietic stem cell transplant
CLL
80
Which chronic leukemia?
Some tumors transform to more aggressive diffuse large B cell lymphoma, and pts die within 1 year
CLL
81
Which chronic leukemia?
A myeloproliferative disorder that particularly shows an increase in granulocytes
CML
82
Which chronic leukemia?
Philadelphia chromosome: BCR-ABL t(9:22) translocation causing a fusion protein
CML
83
Which chronic leukemia?
Some cases undergo transformation into an acute leukemia ("blast crisis")
CML
84
Which chronic leukemia?
Tx is tyrosine kinase inhibitors induce sustained remissions and prevent progression to blast crisis
CML
85
Name an example of a tyrosine kinase inhibitor
Imatinib (Glecvec)
86
Which disease?
Disordered maturation defect where bone marrow is replaced by clonal, multipotent stem cells with capacity for differentiation into red cells (erythroid precursors), granulocytes, and platelets
Myelodysplastic syndrome (MDS)
87
Which disease?
Usually idiopathic, but can develop after chemo or exposure to ionizing radiation
MDS
88
Which disease?
Marrow is hypercellular/normocellular, but the peripheral blood shows cytopenias
MDS
89
Which disease?
High risk of transformation to AML
MDS
90
What are the 3 types of B cell lymphomas?
Hodgkin lymphoma
Non-hodgkin lymphoma
Plasma cell disorders (multiple myeloma)
91
Which B cell lymphoma?
Typified by the Reed-Sternberg (RS) cell which is a germinal center B cell
Hodgkin lymphoma
92
Which B cell lymphoma?
Affects adolescents/young adults or patients >50 yrs
Some have EBV infection
Hodgkin lymphoma
93
Which B cell lymphoma?
Painless LAD: single node spreads to contiguous nodes
"B symptoms," pruritus, and anemia occur with more advanced disease
Hodgkin lymphoma
94
What are the "B symptoms"?
Fever, weight loss, night sweats
95
Which B cell lymphoma?
Tx is chemo, radiotherapy; immunotherapy for refractory disease;
Hodgkin lymphoma
96
Which B cell lymphoma?
Most have the characteristic t(14;18) translocation, causing overexpression of BCL-2 protein
Follicular lymphoma
97
Which B cell lymphoma?
Affects adults > 50 years old
Painless, generalized lymphadenopathy
Follicular lymphoma
98
Which B cell lymphoma?
Incurable, indolent lymphoma; tx is reserved for bulky or symptomatic disease
Follicular lymphoma
99
Which B cell lymphoma?
30-40% progress to diffuse large B cell lymphoma
Follicular lymphoma
100
Which B cell lymphoma?
Arise in MALT associated with epithelium (stomach, salivary glands, etc) and can cause swelling
Marginal zone lymphoma
101
Which B cell lymphoma?
Sustained by chronic inflammation triggered by autoimmune disorders or sites of chronic infection
Marginal zone lymphoma
102
Which B cell lymphoma?
H pylori-specific T cells drive growth and survival of B cells. If you kill the bug, the tumor will shrink, but polyclonal B cell growth can evolve into monoclonal change and spread to distant sites
Marginal zone lymphoma
103
What is the most common lymphoma in adults?
Diffuse large B cell lymphoma
104
Which B cell lymphoma?
Some have a t(14;18) translocation of BCL-2, and others have translocations of MYC (oncogene)
Diffuse large B cell lymphoma
105
Which B cell lymphoma?
Often symptomatic; rapidly enlarging mass within a lymph node, or extranodal in any organ/tissue
Diffuse large B cell lymphoma
106
What is the fastest growing human tumor?
Burkitt lymphoma
107
Which B cell lymphoma?
t(8;14) translocation of MYC and IgH
Effects mainly children and young adults at extranodal sites
Burkitt lymphoma
108
Which B cell lymphoma?
Jaw masses associated with EBV occur in Africa and in ~20% of other cases
Burkitt lymphoma
109
Which B cell lymphoma?
In North America, typically presents as an abdominal mass
Burkitt lymphoma
110
Which B cell lymphoma?
Highly aggressive, but with intensive chemo, most pts are cured
"Starry sky" histologic appearance
Burkitt lymphoma
111
What do neoplastic plasma cells secrete?
Monoclonal immunoglobulin (M protein)
Immunoglobulin fragments with pathologic consequences
112
Are M proteins large or small?
Large
113
Where are M proteins restricted to?
Plasma
114
If immunoglobulin light chains are also made by neoplastic plasma cells, where can they be detected?
Urine and blood
115
Name the conditions with abnormal immunoglobulins
Multiple myeloma
Monoclonal gammopathy of undetermined significance (MGUS)
Amyloidosis
Waldenstrom's macroglobulinemia
116
Light chain deposition with or without multiple myeloma
Amyloidosis
117
Increased IgM blood levels in association with lymphoplasmacytic lymphoma
Waldenstrom's macroglobulinemia
118
Which B cell lymphoma?
Involves bone marrow associated with lytic lesions (often "punched out" radiolucencies) throughout the skeleton
Multiple myeloma
119
Which B cell lymphoma?
Most frequent M protein is IgG
Multiple myeloma
120
Which B cell lymphoma?
If kappa or lambda light chains are produced, their small size allows excretion in the urine (Bence-Jones proteins)
Multiple myeloma
121
Which B cell lymphoma?
Defective production of normal B cells -> high risk for bacterial infections
Multiple myeloma
122
Which B cell lymphoma?
Renal dysfunction due to:
Obstructive proteinaceous casts
Light chain deposition in glomerulus or interstitial
Hypercalcemia leads to dehydration/renal stones
Bacterial pyelonephritis due to hypogammaglobulinemia
Multiple myeloma
123
Name 3 examples of obstructive proteinaceous casts causing renal dysfunction in multiple myeloma
Bence-Jones proteins
Complete Ig
Albumin
124
Which B cell lymphoma?
Bone resorption causing chronic pain and pathologic fracture
Multiple myeloma
125
Which B cell lymphoma?
"Punched out" radiolucencies in bone
Multiple myeloma
126
Which B cell lymphoma?
Hypercalcemia causes confusion, weakness, lethargy
Recurrent bacterial infections
Renal insufficiency & failure
Definitive diagnosis requires bone marrow exam
Tx is chemo and bisphosphonates
Multiple myeloma
127
Drugs that inhibit bone resorption and reduce fractures/hypercalcemia
Bisphosphonates
128
Which B cell lymphoma?
Some patients have "smoldering myeloma" that may be asymptomatic for many years
Multiple myeloma
129
Variety of proliferative disorders of dendritic cells or macrophages
Histiocytosis
130
Range from highly malignant rare neoplasms to benign/reactive proliferations in lymph nodes
Histiocytic neoplasms
131
What disease lies between the range of highly malignant rare neoplasms to benign/reactive proliferations in lymph nodes?
Histiocytosis X or Langerhans cell histocytosis
132
Immature dendritic cells that capture antigens and present them through MHC class II to T cells
Langerhans cells
133
What disease?
Neoplastic cells look more like tissue macrophages than dendritic cells; eosinophils also present
Langerhans cell histocytosis
134
What disease?
Acute/chronic presentations that affect skin, viscera, and bone
Langerhans cell histocytosis
135
What disease?
Eosinophilic granuloma of bone is the most common clinical manifestation
Langerhans cell histocytosis
136
What parts of the body are commonly affected in Langerhans cell histocytosis?
Skull, ribs, vertebrae, mandible
137
What disease?
Dull pain and tenderness often present
Langerhans cell histocytosis
138
What disease?
Punched out or ill-defined radiolucency on X-Ray
Langerhans cell histocytosis
