Chapter 14 - Lymphoid Tissue Disorders Flashcards

0
Q

Where in the lymphoid tissues are T cells located?

A

T cells: paracortex, thymus

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1
Q

Where in the lymphoid tissues are B cells located?

A

B cells: germinal follicles

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2
Q

Where in the lymphoid tissues are histiocytes located?

A

Histiocytes: sinuses, skin (Langerhans cell)

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3
Q

What age ranges are affected by benign or malignant nodal enlargement?

A

Nodal enlargement: 30 usually malignant

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4
Q

What is the most common cause of nodal malignancy?

A

Metastasis MC nodal malignancy

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5
Q

What does painful lymphadenopathy imply?

A

Painful lymphadenopathy: inflammation

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6
Q

What are the most common sites of localized painful lymphadenopathy?

A

Anterior cervical nodes/inguinal nodes MC site localized painful adenopathy

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7
Q

Name two causes of generalized painful lymphadenopathy.

A

Generalized painful adenopathy: SLE, infectious mono

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8
Q

What are the causes of painless lymphadenopathy?

A

Painless lymphadenopathy: metastasis or primary malignant lymphoma

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9
Q

What is a possible cause of painless axillary nodes in a woman?

A

Painless axillary nodes woman: metastatic breast cancer

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10
Q

What are the malignant causes of generalized painless lymphadenopathy?

A

Generalized painless adenopathy: acute/chronic leukemia; follicular B-cell lymphoma

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11
Q

What does left supraclavicular node metastasis indicate?

A

Left supraclavicular node metastasis: stomach/pancreatic carcinoma

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12
Q

What does hilar node metastasis indicate?

A

Hilar node metastasis: lung cancer

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13
Q

What is a cause of pre-aortic node metastasis in men?

A

Pre-aortic node metastasis: testicular cancer

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14
Q

What are causes of inguinal node metastasis?

A

Inguinal node metastasis men/women: penis/vulvar squamous cancer

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15
Q

Describe follicular hyperplasia.

A

Follicular hyperplasia: prominent germinal follicles

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16
Q

What is a key finding in dermatopathic lymphadenitis?

A

Dermatopathic lymphadenitis: melanin pigment in macrophages

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17
Q

What are the characteristics of cat scratch disease?

A

Cat-scratch disease: B. henselae; granulomatous microabscesses

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18
Q

What is toxoplasmosis?

A

Toxoplasmosis: mononucleosis-like syndrome with painful cervical adenopathy

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19
Q

Describe the characteristics of tularemia.

A

Tularemia: zoonosis (rabbits); F. tularensis; ulceroglandular MC type

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20
Q

What causes the plague?

A

Plague: Y. pestis; Yop gene protein products inhibit phagocytosis and kill phagocytes

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21
Q

Describe the transmission of the plague.

A

Transmission: bite of infected fleas that have bitten infected rodents (reservoir of bacterium)

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22
Q

Where does the bubonic plague mainly occur?

A

Bubonic plague mainly in Western U.S.

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23
Q

What are the three main presentations of the plague?

A

Bubonic plaque (MC), septicemic plague, pneumonic plague

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24
Q

If a flea transmitting Y. pestis bites a human leg, which nodes will be involved?

A

Bubonic plague: bite on leg; inguinal node involvement

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25
Q

What occurs during inguinal enlargement in the plague?

A

Inguinal node enlargement: bacteria proliferate, cause edema but few inflammatory cells present; nodes rupture

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26
Q

What are the characteristics of the septicemic plague?

A

Septicemic plague: endotoxemia; massive organ involvement; septicemia

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27
Q

What is a good prognostic sign in breast cancer?

A

Sinus histiocytosis in axillary nodes—good prognostic sign in breast cancer

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28
Q

Describe the origin of the majority of non-Hodgkin lymphomas.

A

NHL: majority B-cell origin

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29
Q

What are the extranodal sites in NHL?

A

Extranodal sites: stomach (MC), CNS, Peyer patches

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30
Q

What is the most common malignant lymphoma in adults and children?

A

NHL: MC malignant lymphoma adults/children

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31
Q

Which viruses are risk factors for NHL?

A

EBV: Burkitt lymphoma, primary CNS lymphoma (HIV)
HTLV-1: adult T-cell leukemia/lymphoma
HCV: B cell lymphoma

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32
Q

H. pylori is a risk factor for which type of lymphoma?

A

H. pylori: low-grade malignant lymphoma in stomach

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33
Q

Which autoimmune diseases are risk factors for NHL?

A

NHL autoimmune disease: Sjögren syndrome, Hashimoto thyroiditis

34
Q

Describe the pathogenesis of NHL.

A

NHL: mutation blocks B/T cells at specific stage of development

35
Q

Precursor T-cell lymphoblastic lymphoma/leukemia is common in which age group?

A

Precursor T-cell lymphoblastic lymphoma/leukemia: common in children

36
Q

Describe the characteristics of mycosis fungoides.

A

MF: CD4 TH cell neoplasm; skin involvement (Pautrier microabscesses)

37
Q

What is Sézary syndrome?

A

Sézary syndrome: MF in leukemic phase

38
Q

Which gender is more affected by nodular sclerosing Hodgkin lymphoma?

A

Nodular sclerosing HL: female dominant

39
Q

Describe the bimodal age distribution of HL.

A

Bimodal age distribution: 15–34 years old and >50 years old

40
Q

EBV is associated with which type of HL?

A

EBV: association with mixed cellularity/lymphocyte depletion/lymphocyte rich HL

41
Q

What kind of immunity is affected in HL?

A

HL: defects in CMI; anergy

42
Q

What is the most common type of HL?

A

Nodular sclerosing classical HL: MC type of HL

43
Q

What is important in HL pathogenesis?

A

Activated NF-κB important in HL pathogenesis

44
Q

What is the neoplastic cell of HL?

A

RS cell neoplastic cell of HL

45
Q

What is required to diagnose HL?

A

RS cell required to diagnose HL

46
Q

What are the clinical findings in HL?

A

HL: fever, weight loss, night sweats; pruritus

47
Q

What is important in determining the prognosis in HL?

A

Prognosis: stage more important than type of HL

48
Q

What are the clinical findings in nodular sclerosis HL?

A

Nodular sclerosis HL: anterior mediastinal mass + single group of nodes above diaphragm

49
Q

What are the risks of treatment in HL?

A

Rx HL: ↑risk 2nd malignancies (AML, NHL)

50
Q

What are the characteristics of histiocytes?

A

Histiocytes: CD1+; Birbeck granules

51
Q

What are the characteristics of Letterer-Siwe disease?

A

Letterer-Siwe: malignant; skin involvement; lytic bone lesions

52
Q

What is the classic triad of Hand-Schüller-Christian disease?

A

HSC: lytic skull lesions, CDI, exophthalmos

53
Q

What are the chracteristics of eosinophilic granuloma?

A

Eosinophilic granuloma: benign histiocytosis; unifocal lytic lesions in bone

54
Q

What are the signs and symptoms of mast cell disorders?

A

Mast cell disease: pruritus, swelling, hyperpigmentation

55
Q

What are the characteristics of urticaria pigmentosum?

A

UP: dermatographism; lesions remain hyperpigmented

56
Q

What type of granules do mast cells have?

A

Mast cells: metachromatic granules

57
Q

What is increased in monoclonal gammopathies?

A

MG: single M protein + light chain

58
Q

What type of M protein is present in the majority of MGs?

A

Majority have IgG M protein

59
Q

What are Bence Jones proteins?

A

BJ protein: light chains in urine

60
Q

What does the serum protein electrophoresis show in MGs?

A

SPE shows monoclonal spike

61
Q

Testing the serum for free light chains is more sensitive than which type of tests?

A

Serum free light chains: more sensitive than urine tests

62
Q

Which age groups and races are affected by multiple myeloma?

A

MM: rare whites

63
Q

List the top three most common types of multiple myeloma in descending order.

A

MM: IgG > IgA > light chain myeloma

64
Q

What does evidence of end-organ damage include in multiple myeloma?

A

CRAB: calcium elevation, renal insufficiency, anemia, and bone lesions

65
Q

Describe the evolution of normal plasma cells to MM.

A

MM: MM: normal plasma cell → MGUS → myeloma

66
Q

What are the pathologic findings and skeletal system findings in MM?

A

MM: lytic lesions, plasma cells >10%; pathologic fractures, hypercalcemia

67
Q

What is the most common site of bone involvement in MM?

A

MM: vertebrae MC bone site

68
Q

Describe the renal disease caused by Bence Jones proteins in MM.

A

BJ renal disease in MM: proteinaceous casts with multinucleated giant cell reaction

69
Q

MM is associated with which type of amyloidosis?

A

MM: association with primary (AL) amyloidosis

70
Q

What are the hematologic findings in MM?

A

MM: anemia with rouleaux, ↑bleeding time (platelet aggregation defect)

71
Q

What are the common causes of death in MM?

A

MM: sepsis/renal failure common causes of death

72
Q

What is the most common MG?

A

MGUS: MC monoclonal gammopathy

73
Q

What is present in the red and white pulp of the spleen?

A

Red pulp: fixed macrophages

White pulp: B and T cells

74
Q

What are the functions of the spleen?

A

Functions: blood filtration (encapsulated bacteria), antigen trapping/processing, platelet reservoir, EMH

75
Q

What are the characteristics of Gaucher disease?

A

Gaucher disease: ↓glucocerebrosidase, ↑glucocerebroside, macrophages fibrillary appearance

76
Q

What are the characteristics of Niemann-Pick disease?

A

Niemann-Pick: ↓sphingomyelinase, ↑sphingomyelin, macrophages soap bubble appearance

77
Q

What are the clinical findings in massive splenomegaly?

A

Massive splenomegaly: infarctions with pain, friction rub, and left-sided pleural effusion

78
Q

What is the gross finding in splenomegaly in cirrhosis with portal hypertension?

A

Splenomegaly in cirrhosis with portal hypertension: sugar-coated spleen

79
Q

What is hypersplenism?

A

Hypersplenism: destruction of hematopoietic cells produces cytopenias

80
Q

What is the most common cause of hypersplenism?

A

PH MCC hypersplenism

81
Q

Splenic dysfunction increases the risk for what?

A

Splenic dysfunction: ↑risk for S. pneumoniae sepsis; HJ bodies

82
Q

Describe the mechanisms by which encapsulated pathogens cause infection in splenic dysfunction.

A

Mechanisms: ↓IgM, ↓tuftsin, ↓splenic macrophages