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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
Q

What are the characteristics of CLL?

A

Median age 60

More common in men

Lymph >5000 cells/uL

Transformation common

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26
Q

What is another name for CLL?

A

Small lymphocytic lymphoma

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27
Q

What is the most common adult leukemia?

A

CLL

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28
Q

What translocation is associated with follicular lymphoma?

A

BCL2 (14;18)

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29
Q

What is the function of BCL2?

A

Antagonizes apoptosis and promotes cell survival (follicular lymphoma)

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30
Q

What are the characteristics of follicular lymphoma?

A

Painless, generalized lymphadenopathy in middle aged adults

Spleen shows expanded white pulp with prominent nodules

Transformation can occur

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31
Q

What are the characteristics of diffuse large B-cell lymphoma?

A

Diffuse pattern obliterates the nodal architecture

> 60

Rapidly enlarging mass

Remission with chemo 60-80%

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32
Q

What gene translocation is associated with Burkitt lymphoma?

A

C-MYC

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33
Q

What is the characteristic pattern associated with Burkitt lymphoma?

A

Microscopic starry sky due to the phagocytosis of apoptosis bodies

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34
Q

What are the three forms of Burkitt lymphoma?

A

African/endemic (EBV)

Sporadic/nonendemic

Patients with HIV

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35
Q

What cells are involved in plasma cell neoplasms?

A

Terminally differentiated B-cells

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36
Q

What is the term for he monoclonal Ig in the blood of patients with plasma cell neoplasm?

A

M component

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37
Q

What are Bence Jones proteins?

A

Free light chains (excreted in urine in plasma cell neoplasms)

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38
Q

What are two common forms of plasma cell neoplasms and their characteristics?

A

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

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39
Q

What mutation is associated with lymphoplasmacytic lymphoma?

A

MYD88

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40
Q

What are the characteristics of lymphoplasmacytic lymphoma?

A

B-cell neoplasm

Older adults

Cryoglobulinemia

No bone lesions or kidney failure

41
Q

What translocation is associated with mantle cell lymphoma?

A

11;14

42
Q

What are the characteristics of mantle cell lymphoma?

A

Mantle zone B-cells surround germinal centres

Male predominance

50-60

Extranodal disease is common

43
Q

What are the characteristics of marginal zone lymphomas?

A

Heterogenous B-cell tumours

Arise in nodes and spleen

MALTomas

Occur at sites of chronic immune or inflammatory reactions

44
Q

When may marginal zone lymphomas regress?

A

If the inflammatory stimulus is removed

45
Q

What are the characteristics of hairy cell leukemias?

A

Plasma cell tumour

Hair like projections on cells

Splenomegaly (red pulp infiltrated)

Males more affected

Often give dry tap (cells trapped in ECM)

46
Q

What is the point mutation associated with hairy cell leukemia?

A

BRAF

47
Q

What are the types of peripheral T and NK cell neoplasms?

A
  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
Q

Chromosomal rearrangements in anaplastic large cell lymphoma involve what?

A

ALK

49
Q

What age does anaplastic large cell lymphoma occur in?

A

Children and young adults

50
Q

What do the nuclei of cells involved with anaplastic large cell lymphoma look like?

A

Large, primitive, horseshoe shaped

51
Q

What virus is associated with adult T-cell leukemia/lymphoma?

A

HTLV-1

52
Q

What is the morphology of CD4 cells in adult T-cell leukemia/lymphoma?

A

Multilobar clover leaf nuclei

53
Q

What is the pathology of mycosis fungoides and Sezary syndrome?

A

CD4 cells home to skin

54
Q

What is the difference between mycosis fungoides and Sezary syndrome?

A

MF- involves epidermis and upper dermis, can spread outside of skin

SS- exfoliating erythroderma, localized to skin

55
Q

What are the characteristics of large granular lymphocytic leukemia?

A

Large lymphocytes with blue cytoplasm and azurophilic granules

Neutropenia and anemia

Increased incidence of rheumatologic disorders

56
Q

What syndrome is associated with large granular lymphocytic leukemia and what are its characteristics?

A

Felty syndrome

RA, splenomegaly and neutropenia

57
Q

What virus is associated with extranodal NK or T-cell lymphoma?

A

EBV

58
Q

What are the characteristics of extranodal NK or T-cell lymphoma?

A

Invaded small vessels and causes necrosis

Destructive nasopharyngeal masses

59
Q

How is Hodgkin lymphoma differentiated from NHL?

A

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
Q

What are Reed-Sternburg cells?

A

B-cells from germinal centres that release factors inducing the accumulation of active lymphs, macs and grans

61
Q

What are the subtypes of Hodgkin lymphoma?

A
  1. Nodular sclerosing
  2. Mixed cellularity
  3. Lymph rich
  4. Lymph depletion
  5. Lymph predominance
62
Q

What are the most and least common types of Hodgkin lymphoma?

A

Most- nodular sclerosing

Least- lymph depletion

63
Q

What are the categories of myeloid neoplasms?

A
  1. Acute myeloid leukemia (AML)
  2. Myelodyplastic syndrome (MDS)
  3. Myeloproliferative disorders (MPD)
64
Q

What is the pathology of myeloid disorders?

A

Feedback mechanisms that modulate cell production in the marrow are deranged

Cells escape normal homeostatic controls

65
Q

What are the characteristics of AML?

A

Accumulation of immature myeloid blasts

> 60

Anemia, neutropenia, thrombocytopenia (pan but mostly WBC)

Can acquire DIC

66
Q

What are the characteristics of MDS?

A

Maturation defects with ineffective hematopoiesis

Dysplasic differentiation in all three lineages

Pseudo-Pelger-Huet bodies

67
Q

What can MDS transform to?

A

AML

68
Q

What mutation is associated with MPD?

A

Constitutively active tyr kinases

69
Q

What are the characteristics of MPD?

A

Increased marrow proliferation

Extramedullary hematopoiesis

70
Q

What are the different MPD?

A
  1. Chronic myelogenous leukemia (CML)
  2. Polycythemia Vera (PV)
  3. Essential thrombocytosis (ET)
  4. Primary myelofibrosis (PMF)
71
Q

What mutation is associated with CML?

A

BCR-ABL fusion on Philadelphia chromosome (t(9:22))

72
Q

What are the characteristics of CML?

A

Leukocytosis

Fatigue, weakness, weight loss, anorexia

50-60

73
Q

What mutation is associated with PV?

A

JAK2

74
Q

What are the characteristics of PV?

A

Erythrocytosis

Increased erythroblast, granulocyte and platelet production

Platelet dysfunction and abnormal blood flow

Organmegaly (extramedullary hematopoiesis)

75
Q

What is the treatment for PV?

A

Phlebotomy

76
Q

What are the characteristics of ET?

A

Increased proliferation and production of megakaryocytes

Thrombosis

Giant platelets

Erythromelalgia

77
Q

What is erythromelalgia?

A

Throbbing/burning of hands and feet due to platelet aggregates in arterioles

78
Q

What mutation is associated with PMF?

A

JAK2

79
Q

What are the characteristics of PMF?

A

Obliterative marrow fibrosis

Leukoerythroblastosis

Extramedullary hematopoiesis and organmegally

Anemia, splenomegaly, bleeding

80
Q

What is Langerhans cell histiocytosis?

A

Monoclonal, neoplastic proliferation of immature dendritic cells

81
Q

What is characteristic of Langerhans cell histiocytosis?

A

Birbeck granules in cytoplasm

Tennis racket appearance of cells- pentalaminar tubules with dilated ends

82
Q

What are the types of Langerhans cell histiocytosis?

A
  1. Multifocal-multisystem/Letterer Siwe disease
  2. Unifocal and multifocal-unisystem/eosinophilic granuloma
  3. Pulmonary
83
Q

What are the functions of the spleen?

A
  1. Phagocytosis of blood cells and particulate matter
  2. Ab production
  3. Hematopoiesis
  4. Sequestration of formed blood elements
84
Q

What is considered splenomegaly?

A

> 150g

85
Q

What does splenectomy increase?

A

Susceptibility to sepsis by encapsulated bacteria

86
Q

What can cause nonspecific acute splenitis?

A

Any blood-borne infection

87
Q

What is the morphology of the spleen in nonspecific acute splenitis?

A

Red and soft (susceptible to rupture)

88
Q

What can cause congestive splenomegaly?

A

Right sided heart failure

Impairment of portal venous drainage

89
Q

Why does congestive splenomegaly protect the spleen from rupture?

A

Capsule becomes thickened and fibrous, harder to rupture

90
Q

When are splenic infarcts seen?

A

Any condition causing splenomegaly

Emboli infarcts

Severe atherosclerosis

91
Q

What types of neoplasms are common in the spleen?

A

Myeloid and lymphoid tumours

92
Q

What congenital abnormality of the spleen is relatively common?

A

Accessory spleens

93
Q

What is splenic rupture normally due to?

A

Blunt trauma

94
Q

What is the function of the thymus?

A

T-cell maturation

T-cell production (until adulthood)

95
Q

What developmental disorders of the thymus as common?

A

Hypoplasia/aplasia (DiGeorge)

Cysts

Hyperplasia- reactive B-cell follicles

96
Q

What disorder is thymic hyperplasia seen?

A

Myasthenia gravis

97
Q

Where do most thyomas occur?

A

Anterior mediastinum

98
Q

What are the characteristics of thyomas?

A

Thymic epithelial cells

Lobulated, form, grey-white masses

Cystic necrosis and calcification

Most are encapsulated

Pathology (25 decks)

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