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Heme and Lymph > WBC 2 > Flashcards

WBC 2 Flashcards

1
Q

Precursor or primitive B and T cell neoplasms are called what

A

Acute lymphoblastic leukemia (ALLs)

-B-ALL and T-ALL

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2
Q

Most ALLs are what type?

manifest as what?

A

B-ALLs

-acute childhood leukemias

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3
Q

T-ALLs tend to present in who?

as what?

A

Adolescent males

-Thymic “lymphomas”

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4
Q

What is the most common cancer of children

A

ALL

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5
Q

Epidemiology of ALLs

A
  • Most younger than 15 . . .peak at age 3
  • 3x as common in whites than blacks
  • slightly more in boys
  • Hispanics have highest frequency
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6
Q

How should follicles normally be in the cortex?

A

nicely arranged

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7
Q

What are the 3 broad types of lymphomas

A
  • NHL
  • HL
  • plasma cell neoplasia
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8
Q

2/3 of NHL and virtually all Hodgkin lymphomas present as what?
other 1/3 of NHL?

A

enlarged non-tender lymph nodes (often >2cm)

-symptoms related to involvement of or in extranodal sites (skin, stomach, brain)

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9
Q

General clinical presentations: Lymphocytic leukemias?
Plasma cell neoplasia - multiple myeloma?
Hodgkin Disease?

A
  • Bone Marrow suppression
  • Lytic lesions of bone/secretion of light chains or entire immunoglobulins
  • Fever
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10
Q
  • M3
  • Aggressive course
  • DIC
A

Acute Promyelocytic Leukemia (APL)

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11
Q

2 year old comes in lethargic and fever . . thinking maybe infections. . you run a CBC with platelet count and it is 10,000 (low)

A
  • it is Not infection; doesn’t suppress platelet count

- Child has Acute lymphocytic leukemia until proven otherwise

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12
Q

Chronic leukemia and platelet counts

A

do NOT tend to be low

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13
Q

in HL and NHL . . is platelet count at presentation clinically useful

A

usually not

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14
Q

What tumors tend to produce cyclic fevers

A

B cell

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15
Q

What are the neoplasms of immature B and T cells

A

B ALL and T ALL

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16
Q

What are the neoplasms of MATURE B cells

A
  • Burkitt lymphoma
  • DLBCL
  • Extranodal Margin Zone Lymphoma
  • Follicular Lymphoma
  • hairy Cell leukemia
  • Mantle Cell lymphoma
  • Multiple myeloma/solitary plasmacytoma
  • SLL/CLL
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17
Q

What are the Neoplasms of MATURE T or NK cells

A
  • Adult T cell leukemia/lymphoma
  • peripheral T cell lymphoma, unspecified
  • Anaplastic Large cell lymphoma
  • Extra-nodal NK/T cell lymphoma
  • Mycosis fungoides/Sezary syndrome
  • Large/granular Lymphocytic leukemia (NK cell lineage)
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18
Q

What are the Classic subtypes of Hodgkin disease?

What is the one Non classic subtype?

A
  • nodular sclerosis
  • mixed cellularity
  • lymphocyte-rich
  • lymphocyte-depleted

Lymphocyte predominant

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19
Q

Reactive and hyperplastic cells in the background of Hodgkin disease that aren’t neoplastic but elaborate all kinds of cytokines leading to fever

A

Reed-Sternburg cells

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20
Q

The most common site of primary extranodal lymphoma is where?
Almost all of these are what?
what is next most frequent site?

A

GI tract
NHL
Skin

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21
Q

When a NHL occurs only in skin, it is called what?

A

cutaneous lymphoma

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22
Q

What is a common tissue manifestation of ALL

A

testicle in boys

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23
Q

age and presentation of B ALL

A
  • children
  • Pancytopenia
  • Aggressive
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24
Q

Age and presentation of T ALL

A
  • Adolescent males
  • thymic masses
  • aggressive
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25
Q

Big cells
alot of cytoplasm
alot of nuclei

A
  • Peripheral T cell lymphoma

- B cells don’t have alot of cytoplasm or alot of nuclei

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26
Q
  • very little cytoplasm . . High N:C ratio
  • chromatin nicely dispursed
  • nucleoli and no granules
  • These are blasts in peripheral blood
A
  • Don’t know if its B or T cell

- if it’s a 3 year old then more than likely B ALL

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27
Q

Approximately 90% of ALLs have numerical or structural chromosomal changes . . most common being what?

A

hyperdiploidy

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28
Q

Pre B markers

A
  • TdT
  • CD10
  • CD19
  • CD22
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29
Q

Onset of ALL

A

Abrupt stormy onset within days to a few weeks of first symptoms

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30
Q

symptoms of ALL related to what?

A

depression of marrow function

  • fatigue due to anemia
  • fever reflecting infections secondary to neutropenia
  • bleeding due to thrombocytopenia
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31
Q

Mass effects of ALL

A
  • Bone pain from marrow expansion and infiltration of subperiosteum
  • Generalized lymphadenopathy, splenomegaly, and hepatomegaly
  • Testicular enlargement
  • in T ALL complications related to compression of large vessels and airways in mediastinum
32
Q

CNS manifestations of ALL

A

headache, vomiting, nerve palsies resulting from meningeal spread . . all more common in ALL in contrast to AML

33
Q

Favorable prognostic factors for ALL?

A
  • Age 2-10
  • low WBC count
  • Hyperdiploidy
  • Trisomies 4, 7, and 10
  • t(12;21)
34
Q

Unfavorable prognostic factors for ALL

A
  • under age 2
  • in adolescent or adult
  • peripheral blasts >100,000
  • t(9;22) “philadelphia chromosome” . . often BCR-ABL fusion
35
Q

Auer rods

A

AML

36
Q

Peripheral B cell neoplasma are typically what age?

What are the 2 that sometimes are found in children?

A

Adults

  • Burkitt lymphoma
  • Marginal Zone Lymphoma
37
Q

CLL and SLL differ only in degree of what?

A

degree of peripheral blood lymphocytosis

38
Q

What is the diagnostic absolute lymphocyte count requirement for CLL

A

> 5000 per mm^3

39
Q

What is the most common leukemia of adults in the western world?

A

CLL

40
Q

median age at diagnosis of CLL?

gender?

A
  • 60

- 2:1 male predominance

41
Q

explain difference b/t CLL and SLL

A
  • CLL has very high PB WC count . . may be higher than 20,000
  • SLL just tends to have lymphadenopathy so you need to biopsy lymph nodes
42
Q
  • Hepatosplenomegaly
  • Pancytopenia (50%)
  • infections
  • “dry tap”
A

Hairy cell leukemia

43
Q

describe the typical lymph node appearance of CLL

A

architecture is obliterated and monomorphic . .can’t delineate anything

44
Q

CLL is often called a pre leukemia. . most adults with it will die of something else because we can treat effectively and they aren’t aggressive . . what will most die from?

A

infection

45
Q

If all you have was lymph node involvement. No lymphocytosis, no big white cell counts peripherally and you looked for immunophenotype and it comes up like CLL . . what do you call this?

A

SLL

46
Q

Surface markers for CLL/SLL?

Hairy cell leukemia?

A
  • IgM or IgM and IgD

- IgG

47
Q

CLL/SLL Ig spike

A

A small monoclonal Ig “spike is present in blood of some patients

48
Q

describe how CLL/SLL disrupts normal immune mechanism

A
  • HYPOGAMMAGLOBULINEMIA is common and contributes to an increased susceptibility to infections particularly bacterial
  • 10-15% of patients develop hemolytic anemia or thrombocytopenia due to AUTOANTIBODIES made by nonneoplastic B cells
49
Q

If we see nucleoli in a lymphocyte, it is called what

A

Prolymphocyte

50
Q

Smudge cells and scattered sperocytes

A

CLL

51
Q

Proliferations centers . . pathognomonic for what?

A

CLL/SLL

52
Q

sperocytes in CLL are caused by what?

A

making antibodies to RBC and causing autoimmune hemolytic anemia

53
Q

Very aggressive phase that some CLL patients go into . .often an ALL like manifestations as in they will start producing blasts in peripheral blood or they can go into a diffuse large B cell lymphoma . . much more lymphadenopathy and organomegaly

A

Richter syndrome

54
Q

What sites are almost always involved with the leukemic infiltrated of CLL

A
  • Bone Marrow

- Splenic white and red pulp and hepatic portal tracts

55
Q

prognosis with CLL transformation to diffuse large B cell lymphoma . . Richter Syndrome

A

less than 1 year

56
Q

What is the most common for of INDOLENT NHL in the US?

A

Follicular Lymphoma

57
Q

Epidemiology for follicular Lymphoma

A
  • M=F
  • Less common in Europe
  • Rare in asians
58
Q

2 principal cell types in follicular lymphoma

A
  • Centrocytes

- Centroblasts

59
Q

Describe the lymph node in follicular lymphoma

A

Obliteration of architecture . . follicles filling up entire compartment of lymph node

60
Q

immunoperoxidase and tagged with antiBCL2 shoes follicle filled with cells tagged with BCL2

A

follicular lymphoma

61
Q

PB in follicular lymphoma?
Bone marrow?
Spleen?

A
  • lymphocytosis (less than 20,000) in about 10%
  • paratrebecular lymphoid aggregates in 85%
  • Splenic white pulp and hepatic portal triads
62
Q

Clinical features of follicular lymphoma

A
  • painless, generalized lymphadenopathy

- although incurable, is usually follows an indolent waxing and waning course

63
Q

median survival for follicular lymphoma?

Does it improve by aggressive therapy?

A

7 to 9 years

-No

64
Q

What is the usual approach to treating a symptomatic follicular lymphoma patient?

A

low dose chemo or anti-CD20 antibody

65
Q

Follicular lymphoma and transformation?

A
  • most go to DLBCL

- some go to tumors resembling Burkitt lymphoma emerge associated with translocations involving MYC

66
Q

What are the special subtypes of DLBCL and what viruses?

A
  • Immunodeficiency-associated Large B cell lymphoma (EBV)

- Primary Effusion Lymphoma (KSHV/HHV-8, in ALL cases)

67
Q

What is the most common for of NHL?

A

DLBCL

68
Q

Clinical presentation of DLBCL

A
  • Aggressive
  • rapidly fatal without treatment
  • Usually a rapidly enlarging mass that can arise virtually anywhere
  • WALDEYER ring involved frequently (posterior Pharynx)
  • Primary of secondary involvement of the liver and spleen may result in large destructive masses
  • Extranodal sites are common and BM involvement Uncommon in contrast to follicular lymphoma
69
Q

Categories of Burkitt lymphoma? and what each involve?

A
  • African (endemic): EBV and face or mandible
  • Sporadic (nonendemic): viscera
  • HIV (aggressive): viscera
70
Q

Starry night morphology

A

Burkitt

71
Q
  • Low power wouldn’t see lymphoid follicles or germinal centers . .just diffuse infiltrate of cells
  • Higher power: lots of mitosis and alot of nucleoli and some look like macrophages . . don’t have tingible bodies
  • lacunar cells
  • these cells stain for fat in cytoplasma
A

Burkitt lymphoma

72
Q

Epidemiology of Burkitt

A

-mainly children or young adults

73
Q

where do most Burkitt manifest

A

extranodal sites
Endemic: kidneys, ovaries, adrenal glands especially
SPoradic: ileocecum and peritoneum

74
Q

Warburg effect . . fastest growing human tumor

A

Burkitt lymphoma

75
Q

nature of Burkitt?

A

Aggressive

76
Q

Burkitt response to intensive chemo

A

responds well

  • most children and young adults can be cured
  • outcome more guarded in older adults

Heme and Lymph (27 decks)

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