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Pathology > Chapter 13- WBCs, Lymph Nodes, Spleen And Thymus > Flashcards

Chapter 13- WBCs, Lymph Nodes, Spleen And Thymus Flashcards

1
Q

What are the two divisions of blood cell types?

A

Myeloid (bone marrow)

Lymphoid (thymus, lymph nodes, spleen)

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2
Q

What is leukopenia?

A

WBC deficiency

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3
Q

What is the most common type of leukopenia?

A

Neutropenia

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4
Q

What are the possible causes of neutropenia?

A

Inadequate/ineffective granulopoiesis

Increased cell destruction or removal in the periphery

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5
Q

What state does hypocellularity occur in?

A

Suppression of granulocyte progenitor cell growth and survival

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6
Q

What state(s) does hypercellularity occur in?

A

Ineffective granulopoiesis or increased peripheral destruction

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7
Q

What are the clinical features of neutropenia?

A

Ulcerating, necrotizing lesions of the oral mucosa

Ulcerative lesions of the skin, vagina, anus or GI tract are less common

Life threatening infections (compromised immune system)

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8
Q

What is leukocytosis?

A

Increased proliferation of WBC

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9
Q

What are the two types of leukocytosis?

A
  1. Reactive

2. Neoplastic

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10
Q

What are potential causes of reactive leukocytosis?

A

Increased marrow production

Increased release from stores

Decreased margination

Decreased extravasation

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11
Q

What are the types of reactive leukocytosis and their causes?

A

Neutrophilia- acute bacterial infections

Eosinophilia- allergies, parasitic infections

Basophilia- myeloproliferative disease

Monocytosis- chronic infections, autoimmune

Lymphocytosis- chronic infections, viral infections

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12
Q

What is lymphadenitis?

A

Lymph node infection

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13
Q

How can lymphadenitis contribute to reactive leukocytosis?

A

Activation causes hyperplasia of follicles and paracortex (increased lymphocyte production and release)

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14
Q

What are the different types of lymphadenitis?

A

Acute nonspecific- tender, localized or systemic

Chronic nonspecific- nontender, follicular or paracortical hyperplasia

Hemophagocytic lymphohistiocytosis- activated macs engulf other blood cell elements, cytokine storm (inflammatory response)

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15
Q

What cells are associated with which part of the lymph node?

A

T-cells- paracortex

B-cells- follicles

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16
Q

What is sinus histiocytosis?

A

Macrophages aggregate within sinuses of lymph nodes draining tissue with epithelial cancers (eg. breast)

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17
Q

What etiological and pathogenic factors influence neoplastic leukocytosis?

A

Chromosomal translocations and other mutations

Genetic factors

Viruses

Chronic inflammation

Iatrogenic factors and smoking

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18
Q

What is the difference between leukemias and lymphoma?

A

Leukemia- BM and blood cells

Lymphoma- discrete tissue masses

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19
Q

What are the categories of neoplastic leukocytosis?

A

Precursor B-cell

Peripheral B-cell

Precursor T-cell

Peripheral T-cell

Hodgkin lymphoma

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20
Q

What is the difference between precursor and peripheral lymphoid neoplasms?

A

Precursor- immature

Peripheral- mature

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21
Q

What are the peripheral B-cell neoplasms?

A

Peripheral B-cell (CLL)

Follicular lymphoma

Diffuse large B-cell lymphoma (DLBCL)

Burkitt lymphoma

Plasma cell neoplasm

Lymphoplasmacytic lymphoma

Mantle cell lymphoma

Marginal zone lymphoma

Hairy cell leukemia

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22
Q

What type of leukemia/lymphoma characterizes precursor B and T-cell neoplasms?

A

Acute lymphoblastic (ALL)

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23
Q

What are the characteristics of ALL?

A

Most common childhood leukemia

Lymphoblasts fill marrow (depressed function)

Anemia, infections, bleeding

Bone pain

CNS manifestations

Lymphadenopathy, hepatosplenomegaly, testicular enlargement

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24
Q

How does pre-T ALL present in adolescent boys?

A

Thymic lymphoma

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25
What are the characteristics of CLL?
Median age 60 More common in men Lymph >5000 cells/uL Transformation common
26
What is another name for CLL?
Small lymphocytic lymphoma
27
What is the most common adult leukemia?
CLL
28
What translocation is associated with follicular lymphoma?
BCL2 (14;18)
29
What is the function of BCL2?
Antagonizes apoptosis and promotes cell survival (follicular lymphoma)
30
What are the characteristics of follicular lymphoma?
Painless, generalized lymphadenopathy in middle aged adults Spleen shows expanded white pulp with prominent nodules Transformation can occur
31
What are the characteristics of diffuse large B-cell lymphoma?
Diffuse pattern obliterates the nodal architecture >60 Rapidly enlarging mass Remission with chemo 60-80%
32
What gene translocation is associated with Burkitt lymphoma?
C-MYC
33
What is the characteristic pattern associated with Burkitt lymphoma?
Microscopic starry sky due to the phagocytosis of apoptosis bodies
34
What are the three forms of Burkitt lymphoma?
African/endemic (EBV) Sporadic/nonendemic Patients with HIV
35
What cells are involved in plasma cell neoplasms?
Terminally differentiated B-cells
36
What is the term for he monoclonal Ig in the blood of patients with plasma cell neoplasm?
M component
37
What are Bence Jones proteins?
Free light chains (excreted in urine in plasma cell neoplasms)
38
What are two common forms of plasma cell neoplasms and their characteristics?
Multiple myeloma- plasma cell tumours in axial skeleton, renal failure and infection Waldenstrom macroglobinemia- high levels of IgM lead to increased blood viscosity Monoclonal gammopathy of undetermined significance
39
What mutation is associated with lymphoplasmacytic lymphoma?
MYD88
40
What are the characteristics of lymphoplasmacytic lymphoma?
B-cell neoplasm Older adults Cryoglobulinemia No bone lesions or kidney failure
41
What translocation is associated with mantle cell lymphoma?
11;14
42
What are the characteristics of mantle cell lymphoma?
Mantle zone B-cells surround germinal centres Male predominance 50-60 Extranodal disease is common
43
What are the characteristics of marginal zone lymphomas?
Heterogenous B-cell tumours Arise in nodes and spleen MALTomas Occur at sites of chronic immune or inflammatory reactions
44
When may marginal zone lymphomas regress?
If the inflammatory stimulus is removed
45
What are the characteristics of hairy cell leukemias?
Plasma cell tumour Hair like projections on cells Splenomegaly (red pulp infiltrated) Males more affected Often give dry tap (cells trapped in ECM)
46
What is the point mutation associated with hairy cell leukemia?
BRAF
47
What are the types of peripheral T and NK cell neoplasms?
1. Unspecified 2. Anaplastic large cell lymphoma 3. Adult T-cell leukemia/lymphoma 4. Mycosis fungoides and Sezary syndrome 5. Large granular lymphocytic leukemia 6. Extranodal NK or T-cell lymphoma
48
Chromosomal rearrangements in anaplastic large cell lymphoma involve what?
ALK
49
What age does anaplastic large cell lymphoma occur in?
Children and young adults
50
What do the nuclei of cells involved with anaplastic large cell lymphoma look like?
Large, primitive, horseshoe shaped
51
What virus is associated with adult T-cell leukemia/lymphoma?
HTLV-1
52
What is the morphology of CD4 cells in adult T-cell leukemia/lymphoma?
Multilobar clover leaf nuclei
53
What is the pathology of mycosis fungoides and Sezary syndrome?
CD4 cells home to skin
54
What is the difference between mycosis fungoides and Sezary syndrome?
MF- involves epidermis and upper dermis, can spread outside of skin SS- exfoliating erythroderma, localized to skin
55
What are the characteristics of large granular lymphocytic leukemia?
Large lymphocytes with blue cytoplasm and azurophilic granules Neutropenia and anemia Increased incidence of rheumatologic disorders
56
What syndrome is associated with large granular lymphocytic leukemia and what are its characteristics?
Felty syndrome RA, splenomegaly and neutropenia
57
What virus is associated with extranodal NK or T-cell lymphoma?
EBV
58
What are the characteristics of extranodal NK or T-cell lymphoma?
Invaded small vessels and causes necrosis Destructive nasopharyngeal masses
59
How is Hodgkin lymphoma differentiated from NHL?
Localized Orderly spread/contiguous Rare mesenteric node and Waldeyr ring involvement Rare extranodal presentation Can’t be diagnosed with flow cytometry Reed-Sternburg cells are necessary for diagnosis
60
What are Reed-Sternburg cells?
B-cells from germinal centres that release factors inducing the accumulation of active lymphs, macs and grans
61
What are the subtypes of Hodgkin lymphoma?
1. Nodular sclerosing 2. Mixed cellularity 3. Lymph rich 4. Lymph depletion 5. Lymph predominance
62
What are the most and least common types of Hodgkin lymphoma?
Most- nodular sclerosing Least- lymph depletion
63
What are the categories of myeloid neoplasms?
1. Acute myeloid leukemia (AML) 2. Myelodyplastic syndrome (MDS) 3. Myeloproliferative disorders (MPD)
64
What is the pathology of myeloid disorders?
Feedback mechanisms that modulate cell production in the marrow are deranged Cells escape normal homeostatic controls
65
What are the characteristics of AML?
Accumulation of immature myeloid blasts >60 Anemia, neutropenia, thrombocytopenia (pan but mostly WBC) Can acquire DIC
66
What are the characteristics of MDS?
Maturation defects with ineffective hematopoiesis Dysplasic differentiation in all three lineages Pseudo-Pelger-Huet bodies
67
What can MDS transform to?
AML
68
What mutation is associated with MPD?
Constitutively active tyr kinases
69
What are the characteristics of MPD?
Increased marrow proliferation Extramedullary hematopoiesis
70
What are the different MPD?
1. Chronic myelogenous leukemia (CML) 2. Polycythemia Vera (PV) 3. Essential thrombocytosis (ET) 4. Primary myelofibrosis (PMF)
71
What mutation is associated with CML?
BCR-ABL fusion on Philadelphia chromosome (t(9:22))
72
What are the characteristics of CML?
Leukocytosis Fatigue, weakness, weight loss, anorexia 50-60
73
What mutation is associated with PV?
JAK2
74
What are the characteristics of PV?
Erythrocytosis Increased erythroblast, granulocyte and platelet production Platelet dysfunction and abnormal blood flow Organmegaly (extramedullary hematopoiesis)
75
What is the treatment for PV?
Phlebotomy
76
What are the characteristics of ET?
Increased proliferation and production of megakaryocytes Thrombosis Giant platelets Erythromelalgia
77
What is erythromelalgia?
Throbbing/burning of hands and feet due to platelet aggregates in arterioles
78
What mutation is associated with PMF?
JAK2
79
What are the characteristics of PMF?
Obliterative marrow fibrosis Leukoerythroblastosis Extramedullary hematopoiesis and organmegally Anemia, splenomegaly, bleeding
80
What is Langerhans cell histiocytosis?
Monoclonal, neoplastic proliferation of immature dendritic cells
81
What is characteristic of Langerhans cell histiocytosis?
Birbeck granules in cytoplasm Tennis racket appearance of cells- pentalaminar tubules with dilated ends
82
What are the types of Langerhans cell histiocytosis?
1. Multifocal-multisystem/Letterer Siwe disease 2. Unifocal and multifocal-unisystem/eosinophilic granuloma 3. Pulmonary
83
What are the functions of the spleen?
1. Phagocytosis of blood cells and particulate matter 2. Ab production 3. Hematopoiesis 4. Sequestration of formed blood elements
84
What is considered splenomegaly?
>150g
85
What does splenectomy increase?
Susceptibility to sepsis by encapsulated bacteria
86
What can cause nonspecific acute splenitis?
Any blood-borne infection
87
What is the morphology of the spleen in nonspecific acute splenitis?
Red and soft (susceptible to rupture)
88
What can cause congestive splenomegaly?
Right sided heart failure Impairment of portal venous drainage
89
Why does congestive splenomegaly protect the spleen from rupture?
Capsule becomes thickened and fibrous, harder to rupture
90
When are splenic infarcts seen?
Any condition causing splenomegaly Emboli infarcts Severe atherosclerosis
91
What types of neoplasms are common in the spleen?
Myeloid and lymphoid tumours
92
What congenital abnormality of the spleen is relatively common?
Accessory spleens
93
What is splenic rupture normally due to?
Blunt trauma
94
What is the function of the thymus?
T-cell maturation T-cell production (until adulthood)
95
What developmental disorders of the thymus as common?
Hypoplasia/aplasia (DiGeorge) Cysts Hyperplasia- reactive B-cell follicles
96
What disorder is thymic hyperplasia seen?
Myasthenia gravis
97
Where do most thyomas occur?
Anterior mediastinum
98
What are the characteristics of thyomas?
Thymic epithelial cells Lobulated, form, grey-white masses Cystic necrosis and calcification Most are encapsulated

Pathology (25 decks)

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