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MPN

CML

PV
PMF
ET
Non-Hodgkin
& Hodgkin
Lymphoma
B cell NonHodgkin
Lymphomas
(B-cell NHL)
All CD20+; treat
w/ rituximab
Follicular
lymphoma
Chronic
lympholytic
leukemia/small
lymphocytic
lymphoma
(CLL/SLL)
Marginal Zone
Lymphoma
(MZL)
Hairy cell
leukemia (HCL)
Aggressive Bcell non-

Presentation
splenomega
ly

Diagnosis
t(9,22)
Philadelphi
a
chromosom
e

Labs/Cytogenetics
High WBC,
high/preserved
platelets, low
emoglobin

Images

Treatment
Tyrosine kinase inhibitor

hodgkin
lymphomas
Diffuse large B
cel lymphoma
(DLBCL)
Mantle cell
lymphoma (MCL)
Burkitts
lymphoma/leuke
mia

Starry sky
Lymphaden
opathy

Starry sky

Like AL, monitor/prophylaxis for tumor


lysis
Like ALL, CNS prophylaxis

t(8,14) =>
incr c-myc
expression
EBV in
almost all
African
cases, but
only 50%
US
Primary CNS
lymphoma
(PCNSL)
Post-transplant
lymphoproliferati
ve disorder
(PTLD)

T-cell NonHodgkin
Lymphomas (Tcell NHL)
Hodgkin

CD15 &

Reed-Sternberg cells (bi-

Lyphoma (HL)
Myelodysplasti
c Syndromes
(MDS)

BM
Cellularity
(+/-10%) =
100-age
Median age
dx: 65 yo
Incidental
finding on
CBC
Lineagespecific
symptoms:
Leukopenia:
recurrent
infxn, poor
wound
healing
Anemia:
fatigue,
chest pain,
shortness
of breath w/
rest/exertio
n, dizziness
Thrombocyt
openia:
easy
bleeding,
bruising
Constitutio
nal
symptoms:

CD30
(CD20 neg)
Use history
& labs to
rule out
other
causes; if
CBC
abnormaliti
es persist,
order flow
cytometry
&
cytogenetic
s/FISH

nucleat cell)
Dysmyelopoiesis/dysgranulo
poiesis: abnormalities in
nuclear segmentation
hyposegmented (1-2 lobeS)
or hypersegmented (>5-6
lobes)

Hypercellul
ar BM
Blasts
<20%
(otherwise,
AML)
Peripheral
blood
cytopenia(s
)
Dysplastic,
dysfunction
al cells
abnormal granulocytes
(eosinophil, basophil); may
show vacuolated cytoplasm

Goals: restore marrow fn; delay


progression to AML
Treatment varies based on prognosis:
Low-risk transfusion support,
growth factors, antibiotics
Higher-risk, but frail lowintensity chemotherapy
High-risk, good performance
status aggressive leukemia-like
chemotherapy, stem cell
transplant

fevers,
night
sweats,
weight loss

Dyserythropoiesis
Abnormality in nuclear
contour; multi-nuclearity

Dyssynchrony: discrepancy
btwn nuclear & cytoplasmic
maturation
Megablastic changes:
abnormally large precursors

Cytoplasmic/basophilic
stippling (also seen with
lead poisoning)

Prussian blue stain for iron:


iron abnormally localized in
mitochondria & forms ring
around nucleus => ringed
sideroblasts

Dysmegakaryopoiesis
Hypolobation, separation of
nuclear lobes, abnormally
small megakaryocytes

Abnormal bone marrow (fat


replaces marrow)

Acute Myeloid
Leukemia
(AML)

Blast
fraction
greater
than or
equal to
20%
BMB: >20%
marrow
cells
comprising
myeloblasts
;
hypercellul
ar (often
100%)
Cytogenetic
abnormaliti
es:

Platelet count low


(thrombocytopeni
a), mild to severe
anemia
High
(leukocytosis) or
low (leukopenia)
WBC (WBC n:
4,000-10,000
cells/uL), up to
600,000
Neutropenia
WBC differential:
blasts

Auer rods (myeloperoxidase


granules in cytoplasm) in
myeloblasts

1) Induction (goal = remission =


peripheral blood counts restored;
neutrophils >1000 cells/uL, Hb>10g/dL,
platelets>100,000/uL, BM <5% blasts);
7+3 (&d cytarabine + first 3d
idarubicin)
Day 14: repeat BMB to assess response
to therapy

myeloblast

Day 28-30: BMB when peripheral blood


counts recovered
2) Consolidation: chemo; if curate rate
with chemo alone poor, then allogeneic
bone marrow transplant
Leukopheresis to reduce WBCs/blasts
(goal: get blast count <50,000) &
prevent leukostasis
End goal: restore normal hematopoiesis

Watch for tumor lysis syndrome

Acute
promyelocytic
leukemia with
t(15;17)
(q22;q12)

Acute
Lymphoblastic

Translocatio
n btwn PML
& RARA
gene =>
Cells
cannot
mature
beyond the
promyelocy
te stage
given the
abnormal
fusion
protein and
subsequent
block of
transcriptio
n

Promyelocytes
instead of blasts,
with slightly
clefted/bilobed
nucleus &
prominent
cytoplasmic
granules, multiple
Auer rods

Flow
cytometry

25% of adult ALL:


t(9;22) BCR-ABL

All-trans retinoic acid (ATRA) & arsenic


to dissolve fusion protein

High risk of DIC


low fibrinogen,
elevated Ddimer/PT/aPTT,
thrombocytopenia
, schistocytes on
smear

CNS prophylaxis with intrathecal and/or


systemic chemotherapy to penetrate

Leukemia
(ALL)
Subtypes:
precursor B
(~80%),
precursor T

for TdT to
test for
lymphoblas
ts, and
categorize
subtypes

Philadelphia
chromosome

CNS and/or craniospinal irradiation


Maintenance therapy: prolonged
courses of low-dose chemotherapy
Philadelphia chromosome+ ALL:
tyrosine kinase inhibitors (imatinib or
dasatinib)
Induction
Consolidation-Intensification (just
chemo)
Allogeneic stem cell transplant for pts
predicted to have low cure rate with
just chemo

Prognostic factors for each;


Normal lab values & morphology

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