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WBC 0503 Flashcards

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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
mantle cell lymphoma
B NHL in older MALES. poor prognosis. CD5+
26
mantle cell lymphoma genetics
t(11;14) cyclin D overexpression inactivates regulatory genes
27
adult T cell lymphoma
T NHL in adults
28
what virus causes adult T cell lymphoma?
HTLV-1 (retrovirus)
29
adult T cell lymphoma presentation
CUTANEOUS lesions. Japan, W Africa, Caribbean. very aggressive.
30
mycosis fungoides
T NHL in adults. | indolent CD4+.
31
mycosis fungoides presentation
cutaneous patches/nodules. | Pautrier microabscesses in epidermis
32
Sezary syndrome
mycosis fungoides spread to blood
33
multiple myeloma
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
multiple myeloma histo
FRIED EGG appearance. | numerous plasma cells with "clock face chromatin" and intracytoplasmic inclusions of Ig.
35
multiple myeloma presentation**
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
multiple myeloma labs**
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
Waldenstrom's macroglobulinemia
M spike = IgM (pentameric) hyperviscosity sx: HA, vision problems, fatigue, aches. no lytic bone lesions.
38
MGUS
monoclonal gammopathy of undetermined significance - monoclonal plasma cell expansion WITHOUT sx of multiple myeloma. *only see M spike
39
acute lymphoblastic leukemia (ALL)
INCREASED LYMPHOBLASTS in bm. TdT+ (marker for pre-B and pre-T cells). CALLA+
40
ALL sx
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
ALL prognosis
most responsive to therapy. may spread to CNS and testes (drugs can't cross BBB and BTB). better prognosis with t(12;21).
42
chronic lymphocytic leukemia (CLL) | and small lymphocytic lymphoma (SLL)
INCREASED LYMPHOCYTES. | SMUDGE CELLS.
43
CLL/SLL sx
affects > 60yo. often asymptomatic. warm AIHA. CLL has increased peripheral bld lymphocytosis. SLL involves nodes - generalized lymphadenopathy.
44
hairy cell leukemia
chronic leuk. | INCREASED MATURE B CELLS with HAIRY CYTOPLASMIC PROCESSES.
45
HCL presentation (sx, lab)
elderly. splenomegaly. pancytopenia (marrow fibrosis). NO LYMPHADENOPATHY. tartrate-resistant acid phosphatase (TRAP).
46
acute myelogenous leukemia (AML)
INCREASED MYELOBLASTS in bm. | myeloperoxidase (MPO) positive - crystallize as AUER RODS.
47
AML presentation
median onset 60 yo. | DIC due to granule contents of promyelocytes.
48
AML M3 subtype
t(15;17) ACUTE PROMYELOCYTIC LEUK. responds to all-trans retinoic acid (vit A).
49
ATRA tx
induces differentiation of myeloblasts
50
auer rods
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
chronic myelogenous leukemia (CML)
MYELOID STEM CELL PROLIF. | increased neutrophils, metamyelocytes, basophils**
52
CML genetics
t(9;22) bcr-abl fusion gene (Philadelphia chromosome)
53
CML presentation
age 30-60. splenomegaly. very low leukocyte alkaline phosphatase due to immature granulocytes - vs. leukemoid rxn of mature cells
54
CML progression
may accelerate and transform into AML or ALL (BLAST CRISIS)
55
CML tx
imatinib: small molecule inhibitor of bcr-abl tyrosine kinase
56
Langerhans cell histiocytosis
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
myeloproliferative disorders
1. CML 2. polycythemia vera 3. essential thrombocytosis 4. myelofibrosis
58
polycythemia vera
abnormal clone of HSCs that are increasingly sensitive to GFs, esp RBC. JAK2 kinase mutation.
59
polycythemia vera presentation
INCREASE IN RBC, WBC, PLT. esp RBC. sx of hyper viscosity: HA, blurry vision, venous thrombosis, splenomegaly, pruritus esp after bathing
60
essential thrombocytosis
INCREASED MEGAKARYOCYTES. | JAK2 kinase mutation.
61
myelofibrosis
fibrotic obliteration of BM. TEAR DROP CELLS. decrease RBC, variable WBC and PLT. JAK2 kinase mutation.
62
which myeloproliferative d/o is NOT assoc. with JAK2 kinase mutation?
CML
63
what is JAK2?
tyrosine kinase. involved in hematopoietic growth factor signaling. V617F mutation = constitutive activity. activate STAT transcription factors.
64
CML cell counts
decrease RBC | increase WBC, PLT
65
relative polycythemia
due to decreased plasma volume. no change in RBC mass or O2 sat. assoc. with dehydration or excessive diuresis.
66
reactive (secondary) polycythemia - appropriate absolute
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
reactive (secondary) polycythemia - inappropriate absolute
due to ECTOPIC EPO (RCC, HCC, Wilm's tumor, hydronephrosis). increase RBC mass only.
68
(primary) polycythemia vera
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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