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This is probably more important for the wards

than for the test


Tumors that P.A.C. in EPO:
Secondary erythrocytosis
pheochromocytomas (from adrenal medullary
Due to tumors tissuesecrets cats), adrenal adenoma,
cerebellar hemangiomas, uterine fibroids

!
High altiduteget travel hx, once down, EPO
disappears, but crit still high
Cardiopulmonary dsfailure to load O2 into
Secondary erythrocytosis lungs (shunts). Hyperviscosity.
Due to tissue hypoxia, drugs, and other Renal disorderdistortion of anatomy leads to
increased EPO. 90% EPO normally made in
kidney.
Hepatictymors, hemangiomas, cysts
Drugstestosterone, EPO
Neoplastic from pluripotent hematopoietic
stem cell
Many, if not all WBC, RBCs, platelets from
single neoplastic stem cell
Polycythemia vera Grow and divide in absence of erythropoietin
General Proliferative phase
Spent phaseBM wears itself outanemia,
laukopenia, thrombocytopenia, secondary
myleofibrosis, secondary AML
Some pts develop secondary AML
Dx: No definitive test--Use low or undetectable erythropoietin
level
Sx: Polychromasia
hyperviscosity: headache, weakness, dizziness,
decreased mental acuity
Polycythemia vera purities after bathing
erythomelagiabright red hands, soles
Dx, symptoms hypermetabolic symptomswt loss, excessive
sweating, fever
thrombosis in artery or veinBudd-Chiari
syndrome
GI bleed,

P. vera ET
Incr: crit/hb, WBC, plts, Incr: plts; normal
basophilia, uric acid, coagulation, iron. Cyto:
P. vera vs. ET: B12, LAP; decr EPO. giant platlest with odd
Labs? Cyto: abnormalno shapes, clusters of
single one. abnormal
megakaryocytes
Facial plethorafull red face
HSM due to extramedullary
Polycythemia vera hematopoeisis
Lab:
Physical exam findings Increased: crit/hb, B12, uric acid, WBC,
Lab findings plts, basophilia, LAP
Low EPO
Cytogeneticno single abnormality

P. vera ET
Neopl. Hematopoietic Monoclonalh. Or polcl.
stem cell Megakaryocyte
Hydryurea, alpha I, Hydryurea, alpha I,
P. vera vs. ET: buslfan, phlb, 32P Anagrelide
Type of abnormality? Tx? Given all sx, which Hypervisc. Sx, GI bleed but also CNS,
are different? hypermetab., GI bleed, ecchymoses, vWFD,
facial plethora, more asympt.
HSM

Phlebotomybest initial treatmentno


progress to AML, BM suppression, incr risk of
thrombosis, do NOT do if iron defic, does not
delay spent phase
Polycythemia vera Hydrosyurea, busulfanmyelosuppresive
Tx
agents
P32
These last 3 increase risk for AML
Interferon alphaflu like side effects, daily
injectionuse for elderly, non-compliance

Sx: initially asympt., microvascular


thrombi, erythromyalgia, purities,
hemorrhagic, acquired vWd desease
ET Lab: Fe norm, coag norm, platelet high,
Sx and lab clusters of abnormal megakaryocytes.
Watch out for pseudohyperkalcemia,
psudohypoglycmia.

Mostly a platelet problemgiant platelets, odd shapes


Need to exclude secondary cause of thrombocytosis (cancer,
iron defic, inflamm, infection)

Essential thrombocythemia Platelet count should be >600 on two separate occasions a
month apart
General: what exclude? How dx? ET rarely progresses to AML; complication=thrombosis
arterial or venous
Complications? May progress to myelofibrosis
**send tube right away in heparinized tube
2therwise you will get pseudohyperkalcemia,
psydohypoglycemia (metabol. Active plts use gluc
Reducing platelet count:
Tx anyone who has thrombotic event, risk
factors for thrombosis, over 65, platelet
count over 1-1.5M
Essential thrombocythemia Treat: anagredlidedecreases platelet count
Tx Side effects: hypotension, palpations,
headaches, fluid retention, congestive heart
failure, bloating and diarrhea due to lactose in
pill
Hydroxyurea, interferon alpha

Phase
Clonal stem cell predom affects
megakaryocytes
Idopathic Myelofibrosis aka agnogenic All other myeloprolif can culminate with this
myeloid metaplasia with myelofibrosis Myloid metaplasia: early proliferative stage
where extramedullary hematopoises
predominates
Can transform to AML

adhesion:
Idopathic Myelofibrosis aka agnogenic thrombocytosis, HSM, (the following are
myeloid metaplasia with myelofibrosis not found in ET according to our sylabus)
Sx anemia, hypermetabolic, SMabdominal
pain, early satiety

IM ET P. vera
Incr: RBC< Incr: plts; Incr: crit/hb,
WBC (leuk.), normal WBC, plts,
plts. Cyto: coagulation, basophilia, uric
Labs: Idopathic myelofibrosis vs. ET leukoerythrobl iron. Cyto: acid, B12, LAP;
vs. P. vera astic with giant platlest decr EPO.
teardrop RBC, with odd shapes, Cyto: abnormal
nucleated RBC, clusters of no single one.
early abnormal
granulocytosis megakaryocytes


Idopathic myelofibrosis
No definitive treatment
May do allogenic BM transplant in very
Tx youngsee some reversal of fibrosis
Disordered maturation of one or more myeloid lineages,
abnormal clone of cells
May remain stable or progress to AML
Myelodysplatic syndrome

Elderly
AsymmptCBC, anemia, thrombocytopenia
What is it? Who gets it? Sx? Labs? Macrocytosis, plts larger, hypogranular neutrophils
This ds is a SPECTRUMworse is close
may have Pelger-Huet anomaly (bilobed spectacle).
Marrowringed sideroblasts, megaloblastic
to AML erythropoiesis, nucleus of RBC precursor is
abnormal, small megak with abnormal nuclei,
blasts are fewer than 30%

Induction Induction=high dose initial chemo used to


Consolidation induce remission
Consolidationchemo given to those in
Multi-modal therapy
remission to extend disease-free survival.
Sarcoma Multi-modal therapy=chem.+surgery+XRT
Carcinoma Sarcoma=mesnchymal tissue, never benign
Carcinoma=epitheial, never benign

PS0=no symptoms
Performance Status PS1=restricted in physically strenusous ds
PS2=ambulatory and capable of self-care. Cant
do work activities
PS3=capable of only limited self-care, confined to
bed or chair more than 50% of waking hours
PS4=completely disabled
PS5=mort

Extrapolate from size of lesion via radiologic,


measure a marker protein made by tumor
Tumor burden Cytogisolate metaphase spreads, low
sensitivity, strong prognostic
Cytogenetics Detected via PCR, high sensitivity. Measure for
Translocations minimal residual ds
Most used for gene expression, lots of inter-lab
IHC variability
Malignant Invasion and metastasis

Lack of reliance on growth factors


Characteristics of malignancy Resistance to apop
Ability to promote angiogenesis
Ability to invade tissues and metastatize
Genomic instability
What is responsible for majority Malignancy as they interrupt the function of a normal organ
leading to failure of that organ
of cancer deaths? Staging? Micrometastasestargeted with adjuvant, neoadjuvant tx
Staging:Impt. For prognosis, tx plan, outcome,
Via history, physical exam, lab, radiographic,
pathologic, LN, metastatic
T=0-4, extent of primary tumor
NLN, 0-3; MMets 0-1

Lethal=10^12
Drug resistance limits curability and reistant
clone will expand in reponse to selective
Role of surgical oncology in pressure.
cancer Tx Surgery
Resectr bulk, metastases, oncologic
emergencies (impinge SCord), pallation,
reconstructoin

Phase I=toxicity, use of surrogate markers,


pharmokinetics
IIefficacy asertainment, does it work?
Eval new drugs: name phases IIIestablish role in lg. pop., comparison with
standard therapy, does it work double bind?
IVpost marketing surveillance

FAB M0-M7
M3(t15:17)
AML Based on blast morphology, surface antigens
via flow
Classification, epidemiology, Sx EPI: incidence increased with age, secondary
presentation leukemias due to prior chemo
Sx: anemia, infection, bleeding, fatigue,
weakness, fevers, bruising, SM, pallor,
petechiae

Those with BM failure show:


Neutropenia (fever, infection)
Anemia (fatigue, pallor, malasise, weakness)
ALL Thrombocytopenia (bruising, hemorrhage)
Lab dx At least a few blasts in peripheral blood
Use peripheral blood, BM morph, flow, cytogenetic,
molec. Assays

stain for myeloperoxidaseauer rods
>20%
Neutropenia (fever, infection)
AML Anemia (fatigue, pallor, malasise, weakness)
Lab dx Thrombocytopenia (bruising, hemorrhage)
DICgranules fuel toxic nasties
At least a few blasts in peripheral blood

+ in myeloblasts: MPO, CAE


+ in monoblasts, monocytes: ANAE

Good prognosis: (t15:17), t(8:21), t(16,16) or 16


AML inversion
Staining, cytogenetics, Tx Bad: -5, -7

Tx: supportive, all get ablative intensive chemo +/-


BMTransplant except M3ATRA, less intense chemo.
(85% cured)
epi: mostly a dz of childhood, peak 2-10. most
common malignancy of childhood.
Similar to AML presenting, but higher incidence
of CNS, LN, and testicular dz. >30% blasts in
ALL BM
Epi, presenting sx, classification DIC is uncommon
Classification: L1little cyto, L2look like
myeloblasts, L3vacuoles
Flow impt. For classifying preB, matureB, preT

preBbest prognosis, also most common, Ig


negative, mediastinal mass
matureBpoor prognosis, L3 typically, light
chain restricted, associ. with c-myc,
AML Burkitts lymphoma
Classification types of cells, ddx ddxITP, aplastic anemia, pancytopenia,,
reactive lymphocytoses, atypical
lymphocytesuse flow

Favorablehyperdiploidy (more sen to chemo)


unfavorable: t(9;22) (philidelphia)
t(8;14)
t(4;11)
AML hypodiploidy
Prognosis, Tx
x: supportive, less intense chemo for longer. For
CNSuse intrathecal chmo, rarely radiation.
remission: <5% blasts, not a cure
AML=chemo only 35% cure, BMT 50% cure;
AML and ALL prognosis: younger does better, translocations
Remission, prognosis ALL=cure75%; prognostic: AGE, quick
remission, nonB, nonT lineage, hyperdiploidy,
no translocations,