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23 General Pathology > 11/9 - Hematopoetic and Lymphoid System > Flashcards

11/9 - Hematopoetic and Lymphoid System Flashcards

1
Q

what are plasma cell tumors

A
  1. multiple myeloma
  2. plasmacytoma
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2
Q

what do B-cell proliferations in plasma cell neoplasma contain

A

neoplastic plasma cells that virtually always secrete monoclonal Ig or Ig fragments which serve as tumor markers and often have pathologic consequences

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3
Q

what is the most common and deadly of plasma cell neoplasms

A

multiple myeloma

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4
Q

difference between normal plasma and myeloma

A

normal (polyclonal) - lots of different types of whole antibodies
intact Ig myeloma (monoclonal) - whole Ab and excess light chains and too many of both

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5
Q

what makes up heavy chain of normal antibody

A

G, A, M, E, D

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6
Q

what makes up light chain of normal antibody

A

kappa, lambda

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7
Q

M proteins are also called what

A

Bence Jones

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8
Q

what is the monoconal Ig identified in the blood referred to as

A

M protieins (in reference to multiple myeloma)

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9
Q

where are M proteins restricted

A

plasma and extracellular fluid

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10
Q

when and where is bence jones/m proteins excreted

A

in urine in abscnce of glomerular damage

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11
Q

neoplastic cells synthesize excess what

A

light chains (kappa or lambda) along with complete Igs

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12
Q

light chains also excreted in urine also called what

A

bence jones proteins

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13
Q

what is plasma cell neoplasm commonly associated with lytic bone lesions, hypercalcemia,renal failure, and acquired immuneabnormalities

A

multiple myeloma

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14
Q

what is most dominated disease in multiple myeloma? where else seen

A

dominated: bone disease:
seen in lymph nodes and extranodal sites

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15
Q

who gets multiple myeloma

A

Incidence higher in men and people of African
descent
Peak age is 65-70 years

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16
Q

is multiple myeloma homo or heretrogenous

A

heterogenous

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17
Q

proliferation and survival of myseloma cells are dependent on what

A

dependent upon several cytokines, most notable IL-6, which is an important growth factor for plasma cells

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18
Q

what produces multiple myeloma

A

produced by tumor cells themselves and
by resident marrow stromal cells

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19
Q

[High OR low] serum levels of IL-6 in patients with active disease are associated with poor prognosis

A

HIGH

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20
Q

what cytokine is important in multiple myeloma

A

IL-6

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21
Q
  • Usually present as destructive plasma cell
    tumors involving the axial skeleton
  • Bones most affected are the vertebral
    column, ribs, skull pelvis, femur, clavicle,
    and scapula
A

multiple. myeloma

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22
Q

multiple myeloma bone lesions radiographically

A

Bone lesions appear radiographically as
punched-out defects, usually |-4 cm in
diameter

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23
Q

high levels of what in the blood of multiple myeloma patients

A

M protein

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24
Q

most characteristic symptom of multiple myeloma

A

Bone pain, particularly in the lumbar spine, is
the most characteristic presenting feature

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25
Q

symptoms of multiple myeloma

A

Hypercalcemia (C)
Renal dysfunction (R)
Anemia (A)
Bone pain with lytic lesions punched out lesions”(B)

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26
Q

common cause of death of multiple myeloma patients

A

Renal insufficiencies and failure are a common cause of death - kidneys become overburdened with the excess circulating light chain proteins of the tumor cells

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27
Q

what do multiple myeloma cells produce

A

produce abnormal clonal, complete or
incomplete, immunoglobulins which can be
detected in the blood and in the urine

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28
Q

in multiple myeloma, abnormal immunoglobulin, usually a light chain, was
previously referred to as ___

A

Bence-Jones protein

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29
Q

what suppress normal humoral immunity in multiple myeloma

A

neoplastic cells

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30
Q

oral manifestations of mulptiple myeloma

A
  • Jaws involved in 30% of cases
  • Jawbone pain and paresthesia
  • Petechial hemorrhage of oral mucosa
  • Bleeding tendency
  • Infections
  • Patients often on IV bisphosphonates- results in medication related osteonecrosis of bone (MRONJ)
  • Prognosis depends on stage of disease
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31
Q

multiple myeloma treatment

A
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32
Q

what is unifocal, monoclonal, neoplastic proliferation of plasma cells that usually arise within bone?

A

plasmacytoma

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33
Q

are extramedullary lesions in plasmacytoma COMMON or UNCOMMON

A

uncommon

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34
Q

who gets plasmacytoma? where?

A

> . males; average age at diagnosis is 55 years
in spine

35
Q

symptoms of plasmacytoma? what does it look like in xray

A

swelling or bone pain
well-defined, unilocular radiolucency

36
Q

how is plasmacytoma treated

A

radiation therapy

37
Q

virtually all patients with plasmacytoma develop ___ by 15 years following their diagnosis

A

MM (multiple myeloma)

38
Q

what displays a mixture of properties, which they share with macrophages such as self-maintenance and ontogeny

A

langerhan cells

39
Q

what is capable of presenting antigen and actively presenting antigen and actively migrate to the draning lymph nodes, which qualifies them as DCs

A

langerhans cells

40
Q

what is an umbrella term designated for variety of prolierative disorders of dendritis cells or macrophages

A

Histiocytosis

41
Q

what is a clonal (genetically identical) proliferation of a special type of immature dendritic cell

A

langerhans cell histiocytosis

42
Q

LBH lies between when spectrum

A

malignant and benign spectrum

43
Q

in LCH, majorit have driver mutations in what genes

A

cancer associated genes

44
Q

neoplastic cells in LCH express what

A

chemokine receptors

45
Q

normal LC expression vs LCH cells

A

Normal LCs express CCR6
neoplastic express CCR6 and CCR7

46
Q

what do LCH neoplastic cells with CCR6 and CCR7 allow

A

allowing the neoplastic cells to migrate into tissues that express the relevant chemokines- CCL20 in skin and bones and CCL19 and CCL21 in lymphoid organs

47
Q

what has abundant, often vacuolated cytoplasm and vesicular nuclei containing linear grooves or folds

A

proliferating LC

48
Q

characteristic morphology of LCH

A

Birbeck granules in cytoplasm and kidney bean shaped cells

49
Q

who gets LCH

A
  1. Rare; 1to 2 newborns per million per year
  2. May occur at any age but is more likely to occur in those <15 years of age
50
Q

LCH clinical features

A
51
Q

what are the LCH prognostic category classifications

A
  1. single organ involvement
  2. multiorgan involvement
52
Q

what is part of LCH single organ involvement

A
  1. unifocal disease
  2. mutifocal disease
53
Q

what is part of LCH multiorgan involvement

A
  1. no organ dysfunction
  2. organ dysfunction (low risk and high risk)

low risk - skin, LN, bone and or pit gland
high risk - lung, liver, spleen and or BM

54
Q

traditional clinical classifications of LCH

A
  1. Monostotic or polyostotic eosinophilic granuloma
  2. Chronic disseminated histiocytosis
    (Hand-Schuller-Christian disease)
  3. Acute disseminated histiocytosis (letterer-Siwe disease)
55
Q

what is LCH that involves solitary or multiple bone lesions without visceral involvement

A

Monostotic or polyostotic eosinophilic granuloma

56
Q

what is LCH that involves skin, bone and viscera

A

Chronic disseminated histiocytosis
(Hand-Schuller-Christian disease)

57
Q

what is LCH that has Prominent cutaneous, visceral, and bone marrow involvement mainly in infants

A

Acute disseminated histiocytosis (letterer-Siwe disease)

58
Q

in LCH, ___ is affected in 10-20% of all cases; dull pain and tenderness often accompany ___ lesions

A

jaw; jaw

59
Q

what disease:

Ulcerative or proliferative mucosal lesions;
proliferative gingival mass develops if it breaks
out of bone

A

LCH oral manifestation

60
Q

what disease:

Radiographically, lesions often appear as sharply punched-out radiolucencies without a corticated rim

A

LCH oral manifestation

61
Q

what disease:

Bone involvement in the mandible is seen in the posterior areas; scooped out appearance is evident causing the teeth to appear as if they are floating in air

A

LCH oral manigestation

62
Q

LCH tx

A
  1. Accessible bone lesions, such as those in the maxilla and mandible are treated by curettage
  2. Low dose of irradiation may be used for less accessible lesions
  3. Multiple chemotherapeutic agents are given in high-risk situations
63
Q

what are tests for evaluating hemostasis

A
  1. prothrombin time (PT)
  2. partial thromboplastin time (PTT)
  3. test for platelet count
  4. test or platelet function
64
Q

what does prothrombin time test

A

this test assesses the extrinsic
and common coagulation pathways

(PT = Play Tennis OUTSIDE)

65
Q

prolonged PT can result from deficiency or dysfunction of what?

A

V, VII, X, prothrombin and fibrinogen

66
Q

what does partial thromboplastin time test

A

this test assesses the intrinsic and common pathway

(PTT = Play Table Tennis INSIDE)

67
Q

prolonged PTT can be due to deficiencies or dysfunction of what

A

V, VIII, IX, X, XI, XII, prothrombin or fibrinogen

68
Q

what is reference range for testing platelet count to evaluate for hemostasis

A

Reference range is 150 x to 350 X 10^3/ μL

69
Q

how to test for platelet function to evaluate for hemostasis

A
  • At present no single test provides an adequate assessment of the complex function of platelets
  • In clinical setting platelet aggregation measures the ability of platelets to adhere to one another in response to thrombin
70
Q

what can excessive bleeding result from

A
  1. Increased fragility of vessels
  2. Platelet deficiency or dysfunction
  3. Derangement of coagulation
71
Q

abnormal hemorrhage is due to what

A
  1. increase fragility of blood vessels
  2. inadequate hemostatic response due to either platelet deficiency or dysfunction
  3. derangement in clotting mechanism
72
Q

what are clinical conditions in which vessel wall abnormalities cause bleeding disorders

A

Hereditary hemorrhagic telangiectasia
Perivascular amyloidosis
Drug reactions
Infections
Ehlers Danlos syndrome
Scurvy

(Harry Potter DIES)

73
Q

what is bleeding related to reduced platelet number called

A

thrombocytopenia

74
Q

reduction in platelet number constitutes an important cause of what

A

generalized bleeding

75
Q

how many counts of platelets constitute THROMBOCYTOPENIA

A

Count <150,000 platelets /µL constitutes
thrombocytopenia

76
Q

how many counts of platelets can aggravate POSTTRAUMATIC BLEEDING

A

Counts in range of 20,000 to 50,000
platelets/ µL can aggravate posttraumatic
bleeding

77
Q

how many counts of platelets is associated with SPONTANEOUS BLEEDING

A

Count <20,000 platelets/ µL may be
associated with spontaneous bleeding

78
Q

causes of thrombocytopenia

A
  1. decreased platelet production
  2. decreased platelet survivala
  3. sequestration
  4. dilution
79
Q

what cause decreased platelet production

A
  • Drug induced: alcohol, thiazides, cytotoxic drugs
  • Infections: measles, HIV
  • Nutritional deficiencies: B 1 2, folate,
  • Leukemia, disseminated cancer, granulomatous diseases
80
Q

what causes decreased platelet survival

A
  • Immunologic destruction
  • nonimune destruction
81
Q

what diseases cause immunologic destruction that decrease platelet survival

A

- Chronic immune thrombocytopenic purpura
- acute immune thrombocytopenic purpura

82
Q

what causes nonimmune destruction that decreases platelet survival

A

disseminated intravascular coagulation

83
Q

what is characteristic in thrombocytopenia

A

small vessel bleeding

84
Q

what does thrombocytopenia produce

A

petechia, purpura, and ecchymosis

23 General Pathology (24 decks)

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