WBC Disorders Flashcards
(176 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 transport?
A
Bloodstream
3
Q
What is the site of WBC production?
A
Bone marrow
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
Chronic infections
A
Monocytotic
6
Q
Decreased serum level of leukocytes
A
Leukopenia
7
Q
Elevated serum level of leukocytes, mostly neutrophils
A
Leukocytosis
8
Q
Seen in the lymph node that is draining a region of infection
A
Painful lymphadenopathy
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
9
Q
Chronic infections and some viral infections
A
Lymphocytotic
9
Q
What do lymph nodes look like in lymphadenopathy?
A
Firm, enlarged
9
Q
When you evaluate lymph nodes, what should they normally look like?
A
Small and non-palpable
10
Q
Allergies (asthma, hay fever), parasitic infections, drug rxns
A
Eosinophilic
11
Q
Seen with chronic inflammation. metastatic cancer, or lymphoma
A
Non-painful lymphadenopathy
12
Q
Which disease?
Often localized but if generalized, there is often an underlying systemic disease
A
Lymphadenopathy
13
Q
Most cases are self-limited and benign, particularly in children
A
Lymphadenopathy
14
Q
Which disease?
<2 weeks or >1 year without size change is unlikely to be a neoplasm
A
Lymphadenopathy
15
Q
What are the Lymphadenopathy etiologies?
A
MIAMI - malignancy, infectious, autoimmune, miscellaneous, iatrogenic
15
Q
Which disease?
Risk for cancer: >6 weeks and not better by 12 weeks
A
Lymphadenopathy
16
Q
Which disease?
Workup includes serology, imaging, possible biopsy
A
Lymphadenopathy
17
Q
What type of Lymphadenopathy etiology?
Fever, drenching night sweats, unexplained weight loss >10% of body weight
A
Malignancy
18
Q
What type of Lymphadenopathy etiology?
Fever, chills, fatigue, malaise
A
Infectious
18
What type of Lymphadenopathy etiology?
Supraclavicular lymphadenopathy adults or children - up to 50% have intraabdominal malignancy
Malignancy
18
Decreased neutrophils in blood
Neutropenia
18
What is the role of a dentist in the evaluation and management of lymphadenopathy?
Dx + tx oral infections (bacterial, viral)
Dx oral malignancies and refer
Assess risk, help dx and refer for systemic malignancy, systemic infection, autoimmune disease
Assess for skin infection/malignancy and refer
19
What type of Lymphadenopathy etiology?
Arthralgias, muscle weakness, rash
Autoimmune
19
What type of Lymphadenopathy etiology?
Other specific findings of each condition
Miscellaneous
19
What type of Lymphadenopathy etiology?
History of new meds
Iatrogenic
20
Decreased granulocytes (neutrophils, basophils, eosinophils) in blood
Agranulocytosis
20
Which disease?
Pathogenesis = decreased production in bone marrow or increased destruction in the peripheral blood
Neutropenia
Agranulocytosis
20
Which disease?
Most common sequela is infection
Neutropenia
Agranulocytosis
21
Which disease?
Symptoms are malaise, fever, chills, weakness, ulceration (oral, gingival) that is deep and punched out
Neutropenia
Agranulocytosis
21
Which lymph node?
Drain scalp, skin
Preauricular
21
Which lymph node?
Drain scalp, neck, upper thoracic skin
Posterior cervical
22
Which lymph node?
Differential diagnosis is scalp infection, mycobacterial infection
Preauricular
Posterior cervical
22
Which lymph node?
Malignancies are skin neoplasm, lymphomas, head and neck squamous cell carcinomas
Preauricular
23
Which lymph node?
Drain GI tract, genitourinary tract, pulmonary
Supraclavicular
24
Which lymph node?
Differential diagnosis is thyroid/laryngeal disease, mycobacterial/fungal infections
Supraclavicular
25
Which disease?
Causes of increased destruction of peripheral cells causing hypercellular marrow include: immune-mediated injury (drugs), overwhelming infections, splenomegaly
Neutropenia
Agranulocytosis
25
Which lymph node?
Malignancies are abdominal and thoracic
Supraclavicular
26
What causes this in neutropenia and agranulocytosis?
Chronic marrow hypoplasia
Aplastic anemia
26
Which lymph node?
Differential diagnosis is mononucleosis, URI, mycobacterial infection, toxoplasma, cytomegalovirus, dental disease, rubella
Submandibular
Anterior cervical
26
Which lymph node?
Drain oral cavity
Submandibular
26
What causes this in neutropenia and agranulocytosis?
Uses up peripheral cells
Overwhelming infections
27
Which lymph node?
Drain larynx, tongue, oropharynx, anterior neck
Anterior cervical
27
What causes this in neutropenia and agranulocytosis?
Transient marrow hyoplasia, decreasing neutrophil production
Chemo
27
Which lymph node?
Squamous cell carcinoma of head and neck, lymphoma, leukemia
Submandibular
28
Which disease?
Causes of decreased production in bone marrow include: chemo, aplastic anemia, leukemia
Neutropenia
Agranulocytosis
29
What causes this in neutropenia and agranulocytosis?
Replacement of normal marrow
Leukemia
30
What causes this in neutropenia and agranulocytosis?
Accelerates removal of granulocytes
Splenomegaly
31
Which disease?
Tx is removal of offending agent, control infection, give granulocyte colony-stimulating factor to stimulate granulocyte production
Neutropenia
Agranulocytosis
31
What are WBC neoplasm classifications based on?
Morphologic and molecular criteria
(lineage-specific protein markers and genetic changes)
31
What are the 3 broad categories of WBC neoplasms that are based on origin/differentiation?
Lymphoid neoplasms
Myeloid neoplasms
Histiocytic neoplasms
32
What is the cause of lymphoid neoplasms?
Increased risk for translocations/transformation in B cells
33
Involvement of bone marrow and peripheral blood
Leukemia
33
Why is there an increased risk for translocations/transformation in B cells?
B cells undergo somatic hypermutation and class switching
33
Increases AB affinity
Somatic hypermutation (B cells)
33
Produces multiple AB types to the same antigen
Class switching (B cells)
34
Which cells are genomically stable, making them an UNCOMMON cause of lymphomas?
T cells
35
Tumor masses in lymph nodes or other tissues
Lymphoma
36
All lymphoid neoplasms can spread where?
To lymph nodes and other tissues
(ex: spleen, bone marrow, peripheral blood)
36
Lymphoid neoplasm classification focuses on ___________ and _________ characteristics of the tumor cells due to overlap in clinical behavior and location
morphology; molecular
37
What are the 2 main mechanisms that lead to the formation of B cell lymphomas?
Somatic hypermutation
Class switching
37
What is meant by surface markers?
Molecular characteristics
37
What tumors come from cells arrested at or derived from a specific stage of normal lymphocyte differentiation?
B and T cell tumors
37
What is meant by shape and size?
Morphology
38
What cancers account for the largest proportion of cancers in children by tumor type?
Leukemia and lymphoma
38
What is the classification criteria for lymphoid neoplasms?
Morphology
Cell origin
Clinical features
Genotype
39
Which disease?
Group of hematologic malignancies characterized by tumor cells that originate in bone marrow and spill over into blood
Leukemia
39
Which disease?
Derived from single transformed cell exhibiting clonal growth
Leukemia
40
As B and T cell tumors progress through each stage of differentiation, what changes?
Morphology
Surface markers
41
Which disease?
Diffuse infiltration into lymph nodes, spleen, liver, and gingiva, causing general enlargment
Leukemia
41
Which disease?
Causes are ionizing radiation, toxins, antineoplastic chemo drugs, chromosomal abnormalities
Acute leukemia
41
Which disease?
Pathophysiology is myelophthisic anemia, neutropenia, anemia, thrombocytopenia
Acute leukemia
41
Replacement of normal hematopoietic cells by neoplastic “blasts” (myeloblasts, erythroblasts, and megakaryocytes) in bone marrow
Myelophthisic anemia
41
Which disease?
Typically, all of the clonal cell population have the same surface markers
Leukemia
41
What does Myelophthisic anemia cause?
Neutropenia
Anemia
Thrombocytopenia
41
What is the MOST COMMON cancer in children?
ALL (acute lymphoblastic leukemia)
42
Leads to hypoxia causing fatigue, shortness of breath, and pallor (decreased RBCs)
Anemia
42
Leads to bacterial, viral, and fungal infections
Neutropenia
42
Leads to bleeding and petechiae
Thrombocytopenia
42
What do the pathophysiologic changes (myelophthisic anemia, neutropenia, anemia, and thrombocytopenia) in acute leukemia lead to?
Extramedullary hematopoiesis
42
Does ALL have a good outcome/cure rate?
Yes!
42
Which disease?
Neoplasm of mature circulating lymphocytes, causing high WBC count
Chronic leukemia
43
Which acute leukemia primarily affects older adults?
AML (acute myeloid leukemia)
43
Does AML have a good outcome/cure rate?
No :(
44
Which disease?
Either lymphocytic or myeloid
Chronic leukemia
45
Which disease?
Slow onset in adult pts with common symptoms like weakness/fatigue, weight loss, night sweats, swollen abdomen, infections, easy bruising
Chronic leukemia
46
Which disease?
High WBC - neutrophils
CML (chronic myeloid leukemia)
47
Which disease?
High WBC - lymphocytes
CLL (chronic lymphocytic leukemia)
47
What is the MOST COMMON leukemia of ADULTS in the western world?
CLL
48
Which chronic leukemia?
Indolent, slow growing tumor, often asymptomatic
CLL
48
Which chronic leukemia?
Increased BCL 2
CLL
49
What is BCL 2?
Anti-apoptotic protein
50
Which chronic leukemia?
If it involves lymph nodes, it is called Small Lymphocytic Lymphoma (SLL)
CLL
50
Which chronic leukemia?
Cure only achieved with hematopoietic stem cell transplant
CLL
51
Which chronic leukemia?
Some cases undergo transformation into an acute leukemia (“blast crisis”)
CML
52
Which chronic leukemia?
Some tumors transform to more aggressive diffuse large B cell lymphoma, and pts die within 1 year
CLL
52
Which chronic leukemia?
Philadelphia chromosome: BCR-ABL t(9:22) translocation causing a fusion protein
CML
52
Which chronic leukemia?
A myeloproliferative disorder that particularly shows an increase in granulocytes
CML
53
Which chronic leukemia?
Tx is tyrosine kinase inhibitors induce sustained remissions and prevent progression to blast crisis
CML
54
Name an example of a tyrosine kinase inhibitor
Imatinib (Glecvec)
54
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)
54
Which disease?
Usually idiopathic, but can develop after chemo or exposure to ionizing radiation
MDS
55
Which disease?
High risk of transformation to AML
MDS
55
What are the 3 types of B cell lymphomas?
Hodgkin lymphoma
Non-hodgkin lymphoma
Plasma cell disorders (multiple myeloma)
55
Which disease?
Marrow is hypercellular/normocellular, but the peripheral blood shows cytopenias
MDS
55
Which B cell lymphoma?
Typified by the Reed-Sternberg (RS) cell which is a germinal center B cell
Hodgkin lymphoma
56
Which B cell lymphoma?
Some have EBV infection
Hodgkin lymphoma
56
Which B cell lymphoma?
Affects adolescents/young adults or patients >50 yrs
Hodgkin lymphoma
56
Which B cell lymphoma?
Painless LAD: single node spreads to contiguous nodes (lower cervical, supraclavicular, mediastinal)
Hodgkin lymphoma
57
What are the “B symptoms”?
Fever, weight loss, night sweats
58
Which B cell lymphoma?
“B symptoms,” pruritus and anemia occur with more advanced disease
Hodgkin lymphoma
59
What are the contiguous nodes?
Lower cervical
Supraclavicular
Mediastinal
59
Which B cell lymphoma?
Tx is chemo, radiotherapy; immunotherapy for refractory disease
Hodgkin lymphoma
60
Which B cell lymphoma?
Prognosis: 5 year survival is > 90%
Hodgkin lymphoma
60
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
60
Which B cell lymphoma?
Most have the characteristic t(14;18) translocation, causing overexpression of BCL-2 protein
Follicular lymphoma
61
Which B cell lymphoma?
Incurable, indolent lymphoma; tx is reserved for bulky or symptomatic disease
Follicular lymphoma
61
Which B cell lymphoma?
Painless, generalized lymphadenopathy
Follicular lymphoma
62
Which B cell lymphoma?
Affects adults > 50 years old
Follicular lymphoma
62
Which B cell lymphoma?
30-40% progress to diffuse large B cell lymphoma
Follicular lymphoma
63
Which B cell lymphoma?
Arise in MALT associated with epithelium (stomach, salivary glands, etc) and can cause swelling
Marginal zone lymphoma
63
What % of follicular lymphoma cases will progress to diffuse large B cell lymphoma?
30-40%
64
Which B cell lymphoma?
Sustained by chronic inflammation triggered by autoimmune disorders or sites of chronic infection
Marginal zone lymphoma
65
What is the most common lymphoma in adults?
Diffuse large B cell lymphoma
65
Which B cell lymphoma?
When localized, cured by simple excision and radiotherapy
Marginal zone lymphoma
66
Which B cell lymphoma?
Often symptomatic; rapidly enlarging mass within a lymph node, or extranodal in any organ/tissue
Diffuse large B cell lymphoma
66
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
67
Which B cell lymphoma?
Aggressive tumor; requires intensive combo of chemo and anti-CD20 drugs w/ 60-80% complete remission
Diffuse large B cell lymphoma
67
Which B cell lymphoma?
In North America, typically presents as an abdominal mass
Burkitt lymphoma
67
Which B cell lymphoma?
t(8;14) translocation of MYC and IgH
Burkitt lymphoma
68
What is the fastest growing human tumor?
Burkitt lymphoma
68
Which B cell lymphoma?
Effects mainly children and young adults at extranodal sites
Burkitt lymphoma
68
Which B cell lymphoma?
Highly aggressive, but with intensive chemo, most pts are cured
Burkitt lymphoma
69
Which B cell lymphoma?
Jaw masses associated with EBV occur in Africa and in ~20% of other cases
Burkitt lymphoma
70
Which B cell lymphoma?
“Starry sky” histologic appearance
Burkitt lymphoma
71
Which B cell lymphoma?
Median age is 70 yrs
Multiple myeloma
71
What do neoplastic plasma cells secrete?
Monoclonal immunoglobulin (M protein)
Immunoglobulin fragments with pathologic consequences
71
Are M proteins large or small?
Large
71
Where are M proteins restricted to?
Plasma
72
Name the conditions with abnormal immunoglobulins
Multiple myeloma
Monoclonal gammopathy of undetermined significance (MGUS)
Amyloidosis
Waldenstrom’s macroglobulinemia
72
Which B cell lymphoma?
A common lymphoid malignancy
Multiple myeloma
72
If immunoglobulin light chains are also made by neoplastic plasma cells, where can they be detected?
Urine and blood
72
Increased IgM blood levels in association with lymphoplasmacytic lymphoma
Waldenstrom’s macroglobulinemia
72
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
72
Light chain deposition with or without multiple myeloma
Amyloidosis
72
Which B cell lymphoma?
Involves bone marrow associated with lytic lesions (often “punched out” radiolucencies) throughout the skeleton
Multiple myeloma
73
Which B cell lymphoma?
Most frequent M protein is IgG
Multiple myeloma
73
Multiple myeloma Which B cell lymphoma?
Defective production of normal B cells -> high risk for bacterial infections
Multiple myeloma
73
Name 3 examples of obstructive proteinaceous casts causing renal dysfunction in multiple myeloma
Bence-Jones proteins
Complete Ig
Albumin
73
Which B cell lymphoma?
“Punched out” radiolucencies in bone
Multiple myeloma
73
Which B cell lymphoma?
Bone resorption causing chronic pain and pathologic fracture
Multiple myeloma
73
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
74
Which B cell lymphoma?
Hypercalcemia causes confusion, weakness, lethargy
Multiple myeloma
75
Which B cell lymphoma?
Recurrent bacterial infections
Multiple myeloma
76
Which B cell lymphoma?
Renal insufficiency & failure
Multiple myeloma
77
Which B cell lymphoma?
Definitive diagnosis requires bone marrow exam
Multiple myeloma
78
Which B cell lymphoma?
Tx is chemo and bisphosphonates
Multiple myeloma
79
Drugs that inhibit bone resorption and reduce fractures/hypercalcemia
Bisphosphonates
80
Which B cell lymphoma?
Death occurs within 1 year if untreated
Multiple myeloma
81
Which B cell lymphoma?
Some patients have “smoldering myeloma” that may be asymptomatic for many years
Multiple myeloma
82
Which B cell lymphoma?
Median survival is 4-7 years
Multiple myeloma
83
What is the median survival for multiple myeloma?
4-7 years
84
Variety of proliferative disorders of dendritic cells or macrophages
Histiocytosis
85
Range from highly malignant rare neoplasms to benign/reactive proliferations in lymph nodes
Histiocytic neoplasms
86
What disease lies between the range of highly malignant rare neoplasms to benign/reactive proliferations in lymph nodes?
Histiocytosis X or Langerhans cell histocytosis
87
Immature dendritic cells that capture antigens and present them through MHC class II to T cells
Langerhans cells
88
What disease?
Neoplastic cells look more like tissue macrophages than dendritic cells; eosinophils also present
Langerhans cell histocytosis
89
What disease?
Acute/chronic presentations that affect skin, viscera, and bone
Langerhans cell histocytosis
90
What disease?
Eosinophilic granuloma of bone is the most common clinical manifestation
Langerhans cell histocytosis
91
What parts of the body are commonly affected in Langerhans cell histocytosis?
Skull, ribs, vertebrae, mandible
92
What disease?
Dull pain and tenderness often present
Langerhans cell histocytosis
93
What disease?
Punched out or ill-defined radiolucency on X-Ray
Langerhans cell histocytosis
