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Pathology Unit 2 > Ch 20: Lymphoid Disorders 2 > Flashcards

Ch 20: Lymphoid Disorders 2 Flashcards

1
Q
A
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2
Q

What is LPL (Lymphoplastic lymphoma) similar to? (Difference?)

A

MALT (different site)

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3
Q

What kind of cells are involved in LPL? Pattern?

A

Mature B cell neoplasm, small lymphocytes, plasmacytoid lymphocytes, and plasma cells. Diffuse pattern

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4
Q

Where is LPL found?

A

Bone marrow. Sometimes spleen/lymph nodes

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5
Q

LPL can develop into? Associated with?

A

Waldenstrom macroglobulinemia. Hyper-viscosity syndrome

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6
Q

Who gets LPL?

A

Mostly men, 50s-60s

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7
Q

What nuclear pseudoinclusions are often found in LPL?

A

Dutcher bodies (immunoglobulin)

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8
Q

What LPL marker is usually found in the serum

A

IgM

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9
Q

What is the prognosis for LPL?

A

5-10 years

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10
Q

What kind of cells are involved in HCL (Hairy Cell Leukemia)? Where? What breakfast item are they compared to?

A

Clonal B-cells small/medium pale cytoplasm, hair-like cell cytoplasmic protrusions. Bone marrow and blood Fried eggs

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11
Q

Who gets HCL? Common involvement? Not?

A

Old men 5:1. Liver/splee. Not lymph nodes

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12
Q

HCL cells are positive for what marker?

A

TRAP (Tartrate-resistant acid phosphatase)

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13
Q

Drug used for remission of HCL?

A

2-CDA

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14
Q

Plasma cell neoplasias produce what kind of cells?

A

Terminally differentiated plasma cells capable of monoclonal gammopathy

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15
Q

What 5 disorders make up plasma cell neoplasias. Most important being which two?

A

1) MGUS 2) Multiple Myeloma 3) Plasmacytoma 4) (Ig deposition) Amyloidosis/light-chain disease 5) Osteosclerotic Myeloma (POEMS) MGUS and Multiple Myeloma

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16
Q

3 risk factors for Plasma Cell Neoplasia?

A

1) Genetics 2) Ionizing radiation 3) Chronic antigenics

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17
Q

Monoclonal Gammopathy of Undetermined Significance (MGUS) has what main marker?

A

elevated M spike in serum – IgM

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18
Q

What is the most common primary bone cancer?

A

Multiple Myeloma aka Plasma Cell Myeloma.

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19
Q

What defines Multiple Myeloma? (4)

A

Plasma cells in the marrow. Extra Ig in the blood/urine (M spike). Elevated Calcium. Sometimes elevated IL-6

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20
Q

What 2 Ig can be found in excess with Multiple Myeloma?

A

IgG and IgA

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21
Q

Who mostly gets Mult. Myeloma? With first degree relative?

A

Old (black x2) men. 4X risk.

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22
Q

Why does MM elevate Ca?

A

Increased osteoclast activation factor → lytic bone lesions (RANK receptor)

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23
Q

What bone findings with MM?

A

Lytic lesions in mostly spine/skull (bone pain) → fractures

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24
Q

What is the key finding in MM blood smears? Why?

A

Rouleax. Charge is lost on RBCs causing clumping “stack of coins”.

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25
Q

What 3 things do MM plasma cells overproduce?

A

Osteoclast activating factor, Ig, and Light chain.

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26
Q

What is the most common morbitity with MM? Why?

A

Infection. Monoclonal plasma cells → no variety of antigen protection

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27
Q

What does extra light chain production by MM cause, leading to? Where is it found?

A

Light chain disease (monoclonal kappa or lambda) → Amyloidosis – blood, urine, kidney.

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28
Q

MM light chain excess shows up what 2 ways?

A

Urine (Bence-Jones proteins) and Kidney (renal failure-myeloma kidney)

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29
Q

What 2 normal B-Cell CD markers do MM cells lack?

A

CD19/20

30
Q

5 common IgH translocation Oncogenes involved in MM?

A

cyclin D1 and D3, C-MAF, FGFR3/MMSET, and MAFB

31
Q

Symptomatic Myeloma is characterized by what acronym of symptoms?

A

CRAB (hyperCalcemia, Renal insuff., Anemia, Bone lesions)

32
Q

Important negative prognostic indicators for MM? (5)

A

t(4;14), t(14;16), t(14;20), del 17p/TP53, and increased B2 microglobulin

33
Q

Where in the world are T Cell lymphomas common? What pathogens are risk factors?

A

Asia. HTLV-1 (ATLL) and EBV

34
Q

T cell lymphomas attract what 2 cells via cytokines?

A

Eosinophils and benign M0s

35
Q

Mature (CD4) T Cell lymphomas have what markers?

A

CD2-8

36
Q

What type of T cells are found in peripheral lymphomas?

A

Alpha-beta T cells

37
Q

What are 2 main symptoms found with ATLL Why? Also?

A

Rash and hypercalcemia. CD4 goes to the skin and lytic bone lesions. Lymphadenopathy and splenomegaly

38
Q

What are the 4 groupings of T Cell lymphoma? More or less aggressive than B Cell?

A

Leukemic, nodal, extranodal, and cutaneous. More aggressive.

39
Q

How is HTLV transmitted? (2)

A

Breast milk, blood exposure.

40
Q

Most common form of primary (CTCL) Cutaneous T-Cell Lymphoma? What kind of cells?

A

Mycosis Fungoides. Mature CD4

41
Q

What 2 locations is CDCL infiltration characterized by? What helps ID the latter?

A

Skin (pautrier microacsesses) and blood (Sezary cells). Cerebriform nuclei.

42
Q

3 Stages of CDCL?

A

Premycotic/eczematous, plaque, tumor,

43
Q

ALCL (Anaplastic Large Cell Lymphoma) Involves? Interesting fact about who it afflicts?

A

Lymph nodes, skin. Bimodal age distribution

44
Q

Diagnostic (hallmark) cells of ALCL have what 3 components?

A

CD30, abundant eosinophilic cytoplasm, irregularly shaped nuclei

45
Q

What virus is found in T cells of most (AILT/AITL) Angioimmunoblastic T-Cell Lymphoma? Where?

A

EBV. In the B cells

46
Q

Main 3 features of Classical Hodgkin’s lymphoma? How does it arise?

A

No mass, B Cells, and Reed-Sternberg cells. Arises in a young person in a single lymph node or chain, spreading contiguously (nontender)

47
Q

2 Telltale features of Reed-Sternberg cell? But?

A

CD30/15 (not CD20) and Multi-lobed “Owl eyes” nuclei. Can be hard to find – only 1-3% of cells in affected tissue.

48
Q

Reed-Sternberg cells secreting cytokines does what? Creates?

A

Creates B symptoms and attracts reactive inflamm. Cells – lymphocytes/plasma cell/M0s/eosinophils → fibrosis

49
Q

What makes up the bulk of Hodgkin’s tumors?

A

Mixed reactive inflammatory cells.

50
Q

What history predisposes one to Hodgkin’s?

A

Mono from EBV

51
Q

70% of classic Hodgkin’s is what kind? Found where?

A

Nodular Sclerosis. Ladies’ Neck/mediastinal Lymph Nodes

52
Q

Nodular sclerosis has what feature? Where are the Reed-Sternberg cells found?

A

Fibrosis bands in LNs. Lacunar lakes – open areas.

53
Q

Hodgkin’s has the best prognosis when it is? Lacks what 3 features?

A

Lymphocyte rich. Eosinophils/neutrophils/sclerosis.

54
Q

Most frequent subtype of Classic Hodgkin’s with HIV? What kind of cell abounds with attraction by R-S Cells using IL-5?

A

MCHL (Mixed Cellularity). Eosinophils

55
Q

What cells are found with (NLPHL) Nodular Lymphocyte-Predominant HL? Prognosis?

A

L&H “popcorn cells”. Excellent

56
Q

Where does NLPHL originate?

A

Germinal centers, B Cells

57
Q

Lymphoproliferative disorders are associated with?

A

Immune deficiency

58
Q

Bad HLA matching techniques can lead to what kind of disorder? What population is it more common in? Why?

A

Posttransplant Lymphoproliferative. (PTLD) Kids. EBV

59
Q

4 varieties of Posttransplant Lymphoproliferative Disorder (PTLD)?

A

1) Early lesions – plasmacytic hyperplasia 2) Polymorphic – Heterogenous cells (most common) 3) Monomorphic – Monoclonal B cells 4) Classic HL-type – rare, in renal transplants

60
Q

Another Lymphoproliferative disorder similar to PTLD? From?

A

Iatrogenic Immunodeficiency-Associated LD. Immunosuppressive drugs for autoimmune disease.

61
Q

Histiocytic proliferations may be both…?

A

Neoplastic and non.

62
Q

What/how do hemophagocytic disorders cause dysregulation?

A

An immunologic defect results in elevation of cytokines dysregulating T cell and M0 activation.

63
Q

Histiocytosis has what 4 key features?

A

Elevated langerhans cells, CD1a, S100, Birbeck granules (tennis racket shaped)

64
Q

Reminder of what Langerhans cells are/do?

A

Dendritic cells in the skin/etc (from bone monocytes) that are APCs to T Cells

65
Q

3 subtypes of Langerhans Cell Histiocytosis? Named disease means (pathoma)?

A

Eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe Disease. Malignant skin

66
Q

Eosinophilic granuloma 2 key pathoma things? Biopsy shows?

A

Unnamed, so benign and not in skin (bone). Fractures in adolescents. Langerhans/Inflamm. Cells/Eosinophils

67
Q

Hand-Schuller-Christian disease has what 5 pathoma facts?

A

3 names = malignancy affects kids >3, scalp rash, lytic skull, diabetes Insip., expthalmos

68
Q

Letterer-Siwe Disease has what 4 pathoma facts?

A

2 names = malignancy affects kids <2, skin rash, cystic skeletal defects, rapidly fatal.

69
Q

Thymoma affects thymus epithelial cells – It’s associated with what disease? Describe the cells.

A

Myasthenia Gravis. Cells are plump rather than spindle shaped.

70
Q

Waldenstrom Macroglobulinemia is what kind of disease?

A

B cell lymphoma with monoclonal IgM (BIG)

71
Q

What 5 symptoms should you look for in Wald. Macro.? What shouldn’t you see?

A

1) Lymphadenopathy 2) IgM spike in serum 3) bad vision 4) neuropathy 5) bleeds (bad platelet aggragation). Lytic lesions

Pathology Unit 2 (17 decks)

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