WT - WBC disorders Flashcards
ALL
- 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)
B cell ALL
- 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
T cell ALL
- Teenage males
- Thymic mass (mediastinal)
- Tracheal obstruction, SVC syndrome
- tDt+
- CD 7, CD2, CD3, CD5, CD4, CD8 (blasts)
AML
- myeloblast malignancy
- adult males (median=65)
- sx of BM suppression, fatigue, thrombocytopenia, neutropenia
- AUER RODS (MPO –> DIC)
- CD33+ (blasts)
- blasts >20% of cells
APL
(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
acute monocytic leukemia
monoblasts
LACK MPO
infiltrate gums
acute megakaryoblastic leukemia
lack MPO
assoc w Downs <5 y/o
myelodysplasia
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
CML
- 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)
leukomoid rxn
nml response to infec
-inc bands but no inc basophils
dx: leukocyte alkaline phosphatase (LAP) - absent in neutrophils of CML
CLL
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)
small lymphocytic lymphoma
- same malignant cells as CLL, but lymphocyte counts <5000
- a subtype of indolent NHL
- nml/mild lymphocytosis
- CD20+CD5
hairy cell leukemia
- 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
HL
- 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
HL subtypes
- Nodular lymphocyte predominant
- CD20, popcorn cells - Classical
Classical HL subtypes
- nodular sclerosing HL (clear space around RS cells), mediastinal mass, most comm, young women
- mixed cellularity (older males, IL5, eos)
- lymphocyte rich (excellent prognosis
- lymphocyte depleted (poor prob, elderly, HIV)
**more lymphocytes=better prognosis
NHL
- painless nodes
- multiple peripheral sites
- noncontiguous spread
- rapid spread
- EXTRANODAL involv (GI, skin)
- 60 y/o or children
- CD20+
ENTIRE MASS=malig cells
indolent NHL
- follicular
- marginal zone lymphoma (MALT)
- small lymphocytic lymphoma
aggressive NHL
- diffuse lg B cell
- mantle cell
- Burkitt’s
diffuse lg B cell NHL
via HIV
BCL6, Zn finger
tx: rituximab
follicular NHL
t(14;18)
- BCL2 over expression
- abundant follicles of SIMILAR SIZE
mantle cell NHL
CD5 + CD20
follicle or germinal center
t(11;14)
-overexpressed CYCLIN D –> G1–>S unregulated
marginal zone NHL
via inflammation (marginal zone forms/expands due to inflamm)
- Sjrogrens
- Hashimoto
- H pylori (MALToma)
small lymphocytic NHL
-same ass CLL but <5000 lymphocytes
CD5 and CD20
Burkitts NHL
via HIV or EBV
- t(8;14)
- c-myc –> translocated to Ig heavy chain locus on 14
histo: starry sky morph
Burkitts NHL endemic
-jaw
-africa/new guinea
-children
males
-mandible mass
Burkitts NHL sporadic
- abd
- children, industrialized countries
primary CNS lymphoma
SINGLE enhancing lesion
via EBV, AIDS
cutaneous T-cell lymphoma
seazary (diffuse) mycosis fungicides (localized)
-malignant T cells
mult myeloma
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)
Waldenstrom macroglbulinemia
B cell lymphoma
- -> IgM (PENTAMER)
- -> hyper viscosity
NO bone lesions
medical EMERGENCY
polycythemia vera
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
essential thrombocytosis
- 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
myelofibrosis
- 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
Langerhans cell histiocytosis
- clonal prolif of dendritic cells (myeloid origin)
- CD1a, S100, CD207
- lyric bone lesions, otitis media w/ mastoid mass
histo: birbeck granules (tennis rackets)
