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WBC 0503 Flashcards

1
Q

leukemoid reaction

A

usually due to INFX.
increase WBC with left shift.
increase leukocyte all phis.
*confused with leukemia

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2
Q

Hodgkin lymphoma

A

Reed-Sternberg cells.
localized single group of nodes.
contiguous spread.

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3
Q

Hodgkin sx

A

constitutional “B” sx: low grade fever, night sweats, weight loss

mediastinal lymphadenopathy

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4
Q

Hodgkin distribution

A

bimodal - young and old.
more common in men (except nodular sclerosing type).

50% assoc. with EBV.

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5
Q

Hodgkin prognosis

A

strongest predictor = stage.

better if increased lymphocytes, decreased RS cells.

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6
Q

Reed-Sternberg cells

A

distinctive tumor giant cell.
binucleate or bilobed with 2 halves as mirror images (OWL’S EYES).
lots of cytoplasm.
inclusion-like nucleoli.

CD30+ and CD15+ B cell origin.

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7
Q

nodular sclerosing HL (65-75%)

A

RS: + (lacunar)
lympho: +++
collagen banding

women > men
young adults
excellent prognosis

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8
Q

mixed cellularity HL (25%)

A

RS: ++++
lympho: +++

interm prognosis

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9
Q

lymphocyte predominant HL (6%)

A

RS: +
lympho: ++++

males < 35yo
excellent prognosis

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10
Q

lymphocyte depleted HL (rare)

A

RS: ++
lympho: +

older males w/disseminated disease.
poor prognosis.

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11
Q

non-Hodgkin lymphoma

A

affects multiple peripheral nodes.
extranodal involvement.
noncontiguous spread.

most involve B cells.

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12
Q

NHL sx

A

fewer constitutional sx than HL.

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13
Q

Burkitt lymphoma

A

B NHL

adolescents, young adults

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14
Q

Burkitt is assoc. with which virus?

A

EBV

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15
Q

Burkitt genetics

A

t(8;14) c-myc moves next to heavy chain Ig gene

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16
Q

Burkitt subtypes

A

Africa: JAW
sporadic: PELVIS/ABD

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17
Q

Burkitt histo

A

“starry sky” appearance - sheets of dark blue lymphocytes with interspersed macrophages

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18
Q

diffuse large B cell lymphoma

A

most common adult NHL (B)
older adults
20% in kids

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19
Q

DLBL presentation

A

large LN or extranodal mass.

oropharyngeal or GI tract.

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20
Q

DLBL histo

A

diffuse sheets of large lymphocytes.

20% mature T cell origin.

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21
Q

follicular lymphoma

A

B NHL in adults.
indolent course.
difficult to cure.

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22
Q

follicular lymphoma presentation

A

painless WAXING/WANING lymphadenopathy.

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23
Q

follicular lymphoma genetics

A

t(14;18) bcl-2 overexpression inhibits apoptosis

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24
Q

follicular lymphoma histo

A

packed follicles.

lose normal LN architecture.

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25
Q

mantle cell lymphoma

A

B NHL in older MALES.
poor prognosis.
CD5+

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26
Q

mantle cell lymphoma genetics

A

t(11;14) cyclin D overexpression inactivates regulatory genes

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27
Q

adult T cell lymphoma

A

T NHL in adults

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28
Q

what virus causes adult T cell lymphoma?

A

HTLV-1 (retrovirus)

29
Q

adult T cell lymphoma presentation

A

CUTANEOUS lesions.
Japan, W Africa, Caribbean.
very aggressive.

30
Q

mycosis fungoides

A

T NHL in adults.

indolent CD4+.

31
Q

mycosis fungoides presentation

A

cutaneous patches/nodules.

Pautrier microabscesses in epidermis

32
Q

Sezary syndrome

A

mycosis fungoides spread to blood

33
Q

multiple myeloma

A

MONOCLONAL plasma cell cancer in marrow.
produces large amounts of IgG (55%) or IgA (25%).

most common primary tumor arising in bone of elderly (> age 40-50)

34
Q

multiple myeloma histo

A

FRIED EGG appearance.

numerous plasma cells with “clock face chromatin” and intracytoplasmic inclusions of Ig.

35
Q

multiple myeloma presentation**

A
  1. increase susceptibility to infx
  2. primary AL amyloidosis (light chains)
  3. punched out lytic bone lesions
"CRAB"
hyperCalcemia
Renal failure (myeloma kidney)
Anemia - normocytic
Bone lesions, BACK PAIN
36
Q

multiple myeloma labs**

A
  1. M spike on protein electrophoresis
  2. Rouleaux formation (increase ESR)
  3. Ig light chains (BENCE JONES PROT) in urine
  4. hypercalcemia (produce osteoclast-activating factor - increase resorption)
37
Q

Waldenstrom’s macroglobulinemia

A

M spike = IgM (pentameric)
hyperviscosity sx: HA, vision problems, fatigue, aches.
no lytic bone lesions.

38
Q

MGUS

A

monoclonal gammopathy of undetermined significance - monoclonal plasma cell expansion WITHOUT sx of multiple myeloma.

*only see M spike

39
Q

acute lymphoblastic leukemia (ALL)

A

INCREASED LYMPHOBLASTS in bm.
TdT+ (marker for pre-B and pre-T cells).
CALLA+

40
Q

ALL sx

A

affects < 15yo.
BM involvement in kids.
mediastinal mass (lymphoma) in adolescent males (T cell subtype, thymic mass).

bone pain.
may involve pleura or present as diffuse pulmo infiltrates.

41
Q

ALL prognosis

A

most responsive to therapy.
may spread to CNS and testes (drugs can’t cross BBB and BTB).

better prognosis with t(12;21).

42
Q

chronic lymphocytic leukemia (CLL)

and small lymphocytic lymphoma (SLL)

A

INCREASED LYMPHOCYTES.

SMUDGE CELLS.

43
Q

CLL/SLL sx

A

affects > 60yo.
often asymptomatic.
warm AIHA.

CLL has increased peripheral bld lymphocytosis.
SLL involves nodes - generalized lymphadenopathy.

44
Q

hairy cell leukemia

A

chronic leuk.

INCREASED MATURE B CELLS with HAIRY CYTOPLASMIC PROCESSES.

45
Q

HCL presentation (sx, lab)

A

elderly.
splenomegaly.
pancytopenia (marrow fibrosis).
NO LYMPHADENOPATHY.

tartrate-resistant acid phosphatase (TRAP).

46
Q

acute myelogenous leukemia (AML)

A

INCREASED MYELOBLASTS in bm.

myeloperoxidase (MPO) positive - crystallize as AUER RODS.

47
Q

AML presentation

A

median onset 60 yo.

DIC due to granule contents of promyelocytes.

48
Q

AML M3 subtype

A

t(15;17)
ACUTE PROMYELOCYTIC LEUK.
responds to all-trans retinoic acid (vit A).

49
Q

ATRA tx

A

induces differentiation of myeloblasts

50
Q

auer rods

A

myeloperoxidase-positive CYTOPLASMIC inclusions in granulocytes, myeloblasts.

common in M3 acute promyelocytic leak.
tx of M3 APML can release auer rods and cause DIC.

51
Q

chronic myelogenous leukemia (CML)

A

MYELOID STEM CELL PROLIF.

increased neutrophils, metamyelocytes, basophils**

52
Q

CML genetics

A

t(9;22) bcr-abl fusion gene (Philadelphia chromosome)

53
Q

CML presentation

A

age 30-60.
splenomegaly.

very low leukocyte alkaline phosphatase due to immature granulocytes - vs. leukemoid rxn of mature cells

54
Q

CML progression

A

may accelerate and transform into AML or ALL (BLAST CRISIS)

55
Q

CML tx

A

imatinib: small molecule inhibitor of bcr-abl tyrosine kinase

56
Q

Langerhans cell histiocytosis

A

Langerhans cell = special dendritic cells in skin.

monocytic proliferation.
unknown etiology.
functionally immature cells do NOT efficiently stimulate T cells via Ag presentation.

S100+ and CD1a+
Birbeck granules (tennis rackets) on EM.
57
Q

myeloproliferative disorders

A
  1. CML
  2. polycythemia vera
  3. essential thrombocytosis
  4. myelofibrosis
58
Q

polycythemia vera

A

abnormal clone of HSCs that are increasingly sensitive to GFs, esp RBC.

JAK2 kinase mutation.

59
Q

polycythemia vera presentation

A

INCREASE IN RBC, WBC, PLT.
esp RBC.

sx of hyper viscosity: HA, blurry vision, venous thrombosis, splenomegaly, pruritus esp after bathing

60
Q

essential thrombocytosis

A

INCREASED MEGAKARYOCYTES.

JAK2 kinase mutation.

61
Q

myelofibrosis

A

fibrotic obliteration of BM.
TEAR DROP CELLS.
decrease RBC, variable WBC and PLT.

JAK2 kinase mutation.

62
Q

which myeloproliferative d/o is NOT assoc. with JAK2 kinase mutation?

63
Q

what is JAK2?

A

tyrosine kinase.
involved in hematopoietic growth factor signaling.

V617F mutation = constitutive activity. activate STAT transcription factors.

64
Q

CML cell counts

A

decrease RBC

increase WBC, PLT

65
Q

relative polycythemia

A

due to decreased plasma volume.
no change in RBC mass or O2 sat.

assoc. with dehydration or excessive diuresis.

66
Q

reactive (secondary) polycythemia - appropriate absolute

A

due to high altitude, lung disease, or congenital heart disease.

increased EPO.
increase RBC mass, decrease O2 sat (hypoxia). no change in plasma vol.

67
Q

reactive (secondary) polycythemia - inappropriate absolute

A

due to ECTOPIC EPO (RCC, HCC, Wilm’s tumor, hydronephrosis).

increase RBC mass only.

68
Q

(primary) polycythemia vera

A

increase plasma vol.
INCREASE RBC mass.
no change O2 sat.

*EPO levels decreased due to neg fb.

Hem/Onc (9 decks)

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