WT - WBC disorders

Question 1

ALL

Answer 1

• 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)

Question 2

B cell ALL

Answer 2

• 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

Question 3

T cell ALL

Answer 3

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

Question 4

AML

Answer 4

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

Question 5

APL

Answer 5

(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

Question 6

acute monocytic leukemia

Answer 6

monoblasts
LACK MPO
infiltrate gums

Question 7

acute megakaryoblastic leukemia

Answer 7

lack MPO

assoc w Downs <5 y/o

Question 8

myelodysplasia

Answer 8

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

Question 9

CML

Answer 9

• 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)

Question 10

leukomoid rxn

Answer 10

nml response to infec
-inc bands but no inc basophils

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

Question 11

CLL

Answer 11

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)

Question 12

small lymphocytic lymphoma

Answer 12

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

Question 13

hairy cell leukemia

Answer 13

• 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

Question 14

HL

Answer 14

• 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

Question 15

HL subtypes

Answer 15

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

Question 16

Classical HL subtypes

Answer 16

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

Question 17

NHL

Answer 17

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

ENTIRE MASS=malig cells

Question 18

indolent NHL

Answer 18

• follicular
• marginal zone lymphoma (MALT)
• small lymphocytic lymphoma

Question 19

aggressive NHL

Answer 19

• diffuse lg B cell
• mantle cell
• Burkitt’s

Question 20

diffuse lg B cell NHL

Answer 20

via HIV
BCL6, Zn finger

tx: rituximab

Question 21

follicular NHL

Answer 21

t(14;18)

• BCL2 over expression
• abundant follicles of SIMILAR SIZE

Question 22

mantle cell NHL

Answer 22

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

Question 23

marginal zone NHL

Answer 23

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

• Sjrogrens
• Hashimoto
• H pylori (MALToma)

Question 24

small lymphocytic NHL

Answer 24

-same ass CLL but <5000 lymphocytes

CD5 and CD20

Question 25

Burkitts NHL

Answer 25

via HIV or EBV - t(8;14) - c-myc --> translocated to Ig heavy chain locus on 14 histo: starry sky morph

Question 26

Burkitts NHL endemic

Answer 26

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

Question 27

Burkitts NHL sporadic

Answer 27

- abd | - children, industrialized countries

Question 28

primary CNS lymphoma

Answer 28

SINGLE enhancing lesion via EBV, AIDS

Question 29

cutaneous T-cell lymphoma

Answer 29

``` seazary (diffuse) mycosis fungicides (localized) ``` -malignant T cells

Question 30

mult myeloma

Answer 30

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)

Question 31

Waldenstrom macroglbulinemia

Answer 31

B cell lymphoma - -> IgM (PENTAMER) - -> hyper viscosity NO bone lesions medical EMERGENCY

Question 32

polycythemia vera

Answer 32

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

Question 33

essential thrombocytosis

Answer 33

- 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

Question 34

myelofibrosis

Answer 34

- 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

Question 35

Langerhans cell histiocytosis

Answer 35

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