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Emma Step 1 > WT - WBC disorders > Flashcards

WT - WBC disorders Flashcards

1
Q

ALL

A
  • child
  • downs (>5y/o)
  • bone pain/BM depression
  • poss tumor lysis syndrome (emergency, tx. w/ rasburicase)

dx: tDt (DNAP, found only in nucleus of pre-B, pre-T)

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2
Q

B cell ALL

A
  • pre-B malig
  • CD10 (pre-B marker)
  • tDt+

tx: high cure rate w/ chemo (tx sanctuary sites!) *better prognosis than T-ALL

translocations: t(9;22) - poor
t(12;21) - good

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3
Q

T cell ALL

A
  • Teenage males
  • Thymic mass (mediastinal)
  • Tracheal obstruction, SVC syndrome
  • tDt+
  • CD 7, CD2, CD3, CD5, CD4, CD8 (blasts)
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4
Q

AML

A
  • myeloblast malignancy
  • adult males (median=65)
  • sx of BM suppression, fatigue, thrombocytopenia, neutropenia
  • AUER RODS (MPO –> DIC)
  • CD33+ (blasts)
  • blasts >20% of cells
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5
Q

APL

A

(key AML subtype)

  • t(15;17)
  • dx: fusion PML-RARA
  • -> abnml RAR (retinoid acid receptor) –> abnml maturation of promyelocytes
  • -> DIC via lots of MPO

tx: ATRA - all trans tetinoic acid (vit A)
- also tx DIC w/ FFP, cryoprecipitate, platelets

-excellent prognosis

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6
Q

acute monocytic leukemia

A

monoblasts
LACK MPO
infiltrate gums

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7
Q

acute megakaryoblastic leukemia

A

lack MPO

assoc w Downs <5 y/o

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8
Q

myelodysplasia

A

via past radiation/chemo

  • ineffective hematopoiesis (isolated anemia)
  • blasts <20% of cells
  • cytopenia with hypERcellular BM
  • dysplasia (abnml morphology)
  • poor prognosis compared to de novo AML

histo: pseudo Pelger-Huet (bilobed nucleus), hypOgranular

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9
Q

CML

A
  • myeloproliferative, at all stages of diffr
  • insidious, asymptomatic
  • OLDER adults
  • CD34 mut
  • BASOPHILS!!!!!!!
  • dx: PHILADELPHIA t(9;22) - BCR-abl fusion –> tyr kinase pro
    tx: imatinib

-can transform to acute leukemia (blast crisis, usu AML)

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10
Q

leukomoid rxn

A

nml response to infec
-inc bands but no inc basophils

dx: leukocyte alkaline phosphatase (LAP) - absent in neutrophils of CML

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11
Q

CLL

A

naive lymphocytes newly prod by BM

  • NOT blasts
  • CD20 AND CD5 (coexpression)
  • lymphocyte count >5000
  • CRUSHED, LITTLE LYMPHOCYTES (smudge)
  • B cells that are mature but not yet diffr into plasma cells
  • often asymptomatic but…
  • B cell disruption
  • AutoAbs
  • feared: DIFFUSE LG B cell LYMPHOMA (richters transform)
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12
Q

small lymphocytic lymphoma

A
  • same malignant cells as CLL, but lymphocyte counts <5000
  • a subtype of indolent NHL
  • nml/mild lymphocytosis
  • CD20+CD5
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13
Q

hairy cell leukemia

A
  • rare, chronic B cell malig
  • CD19, CD20, CD22
  • sensitive –> CD103
  • MASSIVE splenomegaly, red pulp engorged
  • LACK lymphadenopathy
  • dry tap

dx: TRAP (tartare resistant acid phosphatase)

median age= 52 y/o

tx: Cladribine

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14
Q

HL

A
  • localized
  • orderly, sequential spread node–> node
  • RARE extra nodal involvement
  • bimodal age (25, 65)
  • painless nodes
  • B sx
  • reed-sterngerg
  • CD15 and CD30

risk fx: prior EBV

tx: ABVD (adiamycin, bleomycin, decarbazine, vinblastin)

RARE NEOPLASTIC (RS) CELLS

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15
Q

HL subtypes

A
  1. Nodular lymphocyte predominant
    - CD20, popcorn cells
  2. Classical
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16
Q

Classical HL subtypes

A
  1. nodular sclerosing HL (clear space around RS cells), mediastinal mass, most comm, young women
  2. mixed cellularity (older males, IL5, eos)
  3. lymphocyte rich (excellent prognosis
  4. lymphocyte depleted (poor prob, elderly, HIV)

**more lymphocytes=better prognosis

17
Q

NHL

A
  • painless nodes
  • multiple peripheral sites
  • noncontiguous spread
  • rapid spread
  • EXTRANODAL involv (GI, skin)
  • 60 y/o or children
  • CD20+

ENTIRE MASS=malig cells

18
Q

indolent NHL

A
  • follicular
  • marginal zone lymphoma (MALT)
  • small lymphocytic lymphoma
19
Q

aggressive NHL

A
  • diffuse lg B cell
  • mantle cell
  • Burkitt’s
20
Q

diffuse lg B cell NHL

A

via HIV
BCL6, Zn finger

tx: rituximab

21
Q

follicular NHL

A

t(14;18)

  • BCL2 over expression
  • abundant follicles of SIMILAR SIZE
22
Q

mantle cell NHL

A

CD5 + CD20
follicle or germinal center
t(11;14)
-overexpressed CYCLIN D –> G1–>S unregulated

23
Q

marginal zone NHL

A

via inflammation (marginal zone forms/expands due to inflamm)

  • Sjrogrens
  • Hashimoto
  • H pylori (MALToma)
24
Q

small lymphocytic NHL

A

-same ass CLL but <5000 lymphocytes

CD5 and CD20

25
Q

Burkitts NHL

A

via HIV or EBV

  • t(8;14)
  • c-myc –> translocated to Ig heavy chain locus on 14

histo: starry sky morph

26
Q

Burkitts NHL endemic

A

-jaw
-africa/new guinea
-children
males
-mandible mass

27
Q

Burkitts NHL sporadic

A
  • abd

- children, industrialized countries

28
Q

primary CNS lymphoma

A

SINGLE enhancing lesion

via EBV, AIDS

29
Q

cutaneous T-cell lymphoma

A 
seazary (diffuse)
mycosis fungicides (localized)

-malignant T cells

30
Q

mult myeloma

A

IL6
xs IgG, IgA but mostly IgG (M spike)

CRAB
hyperCalcemia
Renal failure (bence jones light chains coming w/ TammHorfsall)
Anemia (normocytic, normochromic)

  • INFEC=COD
  • rouleaux (stacked coins)
31
Q

Waldenstrom macroglbulinemia

A

B cell lymphoma

  • -> IgM (PENTAMER)
  • -> hyper viscosity

NO bone lesions

medical EMERGENCY

32
Q

polycythemia vera

A

inc Hb, Hct
hypERcellular BM
DEC EPO
dec ferritin

need to exclude hypoxemia or EPO tumor (renal cell CA)

sx:
HTN, flushing, thrombosis, facial red/puffy, itchiness post shower), DVT (BUDD-CHIARI * HEPATIC V.)

tx: phlebotomy to avoid death, hydroxyurea

33
Q

essential thrombocytosis

A
  • malig prolif of mature myeloid cells
  • massive prolif of MEGAKARYOCYTES/PLATELETS

exclude CML, PV, reactive thrombocytosis (Fe deficiency)

sx:
inc platelet count but dysfxn (bleeding) or overfxn (thrombosis)

tx: hydroxyurea, aspirin

34
Q

myelofibrosis

A
  • neoplastic prolif of mature myeloid cells, esp MEGAKARYOCUTES, look ABNML
  • obliteration of BM via fibrosis due to inc fibroblast activ
  • BM failure -> extramedullary hematopoiesis
  • fatigue, weight loss, night sweats
  • normocytic, normochromic anemia
  • hyperuricemia GOUT
  • POOR PROGNOSIS

tx: stem cell transplant

  • massive splenomegaly
  • left upper abd pain
  • early satiety

histo: tear drop cells (dacrocytes, reticulin fibrosis

35
Q

Langerhans cell histiocytosis

A
  • clonal prolif of dendritic cells (myeloid origin)
  • CD1a, S100, CD207
  • lyric bone lesions, otitis media w/ mastoid mass
    histo: birbeck granules (tennis rackets)

Emma Step 1 (17 decks)

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