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SS- Heme > Hematologic Malignancies > Flashcards

Hematologic Malignancies Flashcards

1
Q

myeloid sarcoma

A

malignancy of lymph nodes, spleen, sub epithelium of the GI tract

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2
Q

hematologic malignancy

A

abnormal proliferation of cells derived from those normally found in the blood, bone marrow, or lymphatic tissues

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3
Q

leukemia

A

malignancy of bone marrow or blood

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4
Q

acute leukemia

A

hematologic malignancy with > 20% blasts, rapidly proliferating and immediate threat to patient’s life
enumerate blasts on the basis of morphology, best identified by immunophenotype

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5
Q

blasts in hematologic malignancy

A

abnormally proliferating cells in leukemia
large cells, high nuclear/cytoplasmic ratio, prominent single or multiple nucleoli, immature chromatin (faint, smudgy), appearance shared by many on slide

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6
Q

chronic leukemia

A

slowly proliferating and not an immediate threat to patient’s life

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7
Q

lymphoma

A

malignancy of lymph nodes, spleen, sub epithelium of GI tract

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8
Q

lymphoproliferative disease

A

peripheral blood and lymphatic tissue

a.k.a. leukemia/lymphoma

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9
Q

bone marrow aspirate

A

0.5 cc: thick bloody fluid containing bony spicules
most accurate count of blasts in bone marrow
smear and extra clots in a tube

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10
Q

flow cytometry

A

tells immunophenotype
aspirate used is hemodilute: have to lyse RBC
uses a laser to scatter light: fluorescence (fluorescent Ab to desired cell surface proteins)

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11
Q

CD34

A

hematopoietic stem cells marker

ALL marker

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12
Q

CD33

A

granulocyte marker

ALL marker

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13
Q

FISH

A

fluorescent oligonucleotides specific for chosen target: suspect a particular translocation (cytogenic studies are normal)
can do in cells with intact nuclei (interphase): don’t have to be growing

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14
Q

myeloproliferative disease

A

hematologic malignancy with chronically proliferating clones which differentiate to circulating blood cells
types: myeloid, erythroid, megs
cytogenetics and/or FISH

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15
Q

myelodysplastic syndrome (MDS)

A

hematologic malignancy with poorly functioning clones (proliferate and differentiate)
cytogenetics: increase in apoptosis in bone marrow
present: unexplained cytosine, bicytopenia, pancytopenia
Dx may include: abnormal dyspoietic bone marrow morphology, abnormal dyspeptic immunophenotypes of maturing precursors, abnormal cytogenetics, increased morphologic blasts (>5%, <20%)
elderly
can progress to AML
Key: increased cell mass, clonal origin, committed stem cell mutations, full maturation

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16
Q

FLT3

A

tyrosine kinase

mutation: proliferation inducing activation AML

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17
Q

IDH1/2

A

generates alpha-kg
mutation (gain of function): reduced differentiation, results in overproduction of molecule similar to alpha-kg that inhibits Tet
TFs: RARA, CBF

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18
Q

Tet1/2

A

de-methylate cytosine residues
mutation (inactive): reduced differentiation
TFs: RARA, CBF
AML

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19
Q

WT-1

A

localizes Tet to target genes
mutation (inactive): reduced differentiation
TFs: RARA, CBF
AML

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20
Q

alpha-kg

A

cofactor for Tet: oxidize methyl group to hydroxymethyl group

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21
Q

DNMT3A

A

methyl cytosine modification

mutation: proliferation inducing inactivation, decreases methylation

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22
Q

Auer rod

A

myeloid blasts

crystallization of granule contents: mostly made up of myeloperoxidase

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23
Q

Acute lymphoblastic Leukemia (ALL)

A 
rapidly proliferating
lymphoid lineage
most under 6 years; secondary rise by age 40
most acute leukemias in children are ALL
prognosis: 80% kids; 50% adults
really high WBC count: 25% blasts in all
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24
Q

BCR-ABL1

A

t(9;22)(q34;q11.2)
proliferation: fusion of ser/thr kinase (BCR) to a tyrosine kinase (ABL1): p190
differentiation inhibition: IKZF1 TF mutation (80% of cases)
older adults and kids <1
ALL: bad prognosis
CD10+, CD19+, TdT+

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25
Q

MLL rearranged

A 
t(v;11q23)
inhibition of differentiation: fusion of transcription regulator (histone methyl transferase) to any of several partners
proliferation: FLT3 (20% of cases)
ALL: bad prognosis
CD10-, CD19+, TdT+
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26
Q

TEL-AML1 (ETV6-RUNX1)

A

t(12;21)(p13;q22): fusion protein that acts as a dominant negative TF with multiple effects on gene expression (blocks maturation)
kids: 25% of pediatric B-ALL: good prognosis, 90% cure
TdT+, CD34+, CD10+, CD20-

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27
Q

hyperdiploid

A

> 50 chromosomes

ALL: good prognosis

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28
Q

T-ALL

A

t(14qq11;10q24)(TCR-alpha;HOX11): translocation of oncogene to TCR promoter (multiple partners)
kids: 25% of pediatric B-ALL
thymic mass or lymph node, spleen involvement
prognosis: High risk; genetics not yet useful for Tx
TdT+, CD3+, CD5+, can express B-cell or myeloid antigens

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29
Q

blast immunophenotype

A

CD34+

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30
Q

myeloid blast immunophenotype

A

CD34+, CD33+

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31
Q

lymphoid blast immunophenotype

A

TdT+, CD10+

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32
Q

mature B lymphocyte/lymphoma immunophenotype

A

CD19+, CD20+
centrocytes and centroblasts: CD10
blasts: CD10, TdT

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33
Q

mature T lymphocyte/lymphoma immunophenotype

A

CD3+, CD5+

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34
Q

chronic myelogenous leukemia (CML)

A

myeloproliferative disease
high WBC: all stages of granulocyte maturation in blood
Dx: FISH or RT-PCR for BCR-Abl1 fusion protein with peripheral blood (p210) with TK activity
cytogenetics: Philadelphia chromosome t(9;22)
Tx: imatinib (TKI) or allogenic transplant
gage efficacy: complete molecular response
splenomegaly; early neutrophils in PB
concern: progression to AML (occasionally ALL)

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35
Q

chronic myelomonocytic leukemia (CMML)

A

myelodysplastic/myeloproliferative
v, PDGFRB
high WBC: monocytes, promonocytes, monocyte/granulocyte hybrids

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36
Q

myeloid bulge

A

more myelocytes than metamyelocytes: evidence of myeloproliferative neoplasm

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37
Q

polycythemia vera

A

myeloproliferative: increased RBC mass (megs increase too)
activating Jak2-V617F mutations (most), some mutations in EPO receptor (MPL)
thrombosis, HTN, stroke or MI
prognosis: >10 yrs
splenomegaly, low EPO
can progress to: myelofibrosis, MDS, acute leukemia
Tx: phlebotomy

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38
Q

essential thrombocythemia

A

myeloproliferative: increased platelets
Jak2-V617F mutations (50%) or calreticulin (CALR) mutations (25%)
thrombosis
>1.5 million platelets: von Willebrand’s disease: responds to platelet reduction
prognosis: >10 yrs
can progress to: myelofibrosis, MDS, acute leukemia

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39
Q

primary myelofibrosis

A

myeloproliferative: increased platelets
Jak2 mutations (50%)- detectible in peripheral blood leukocytes
thrmobocytosis and/or leukoerythroblastic picture
prognosis: shorter than ET
can progress to marrow failure, acute leukemia

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40
Q

refractory cytopenia with unilineage dysplasia

A

unexplained cytopenia, >65 yrs
nonspecific cytogenetic abnormalities MAY be present (trisomies, monosomies)
survival not less than normal for age, rarely progresses to AML
Dx: depends on morphology

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41
Q

refractory anemia with ring sideroblasts

A

unexplained cytopenia, >65 yrs
nonspecific cytogenetic abnormalities MAY be present (trisomies, monosomies)
survival not less than normal for age, rarely progresses to AML
Dx: depends on morphologic findings + iron stain results

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42
Q

myelodysplastic syndrome with isolated del(5q)

A 
severe anemia, >65 yrs, female
all megs mononuclear
cytogenetics: only loss of the large arm of chromosome 5
good median survival
Tx: lenalidomide
10% progress to AML
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43
Q

refractory cytopenia with multilineage dysplasia

A

severe anemia, >65 yrs, female
two or more lineages show dysplastic changes
about half show nonspecific cytogenetic abnormalities
median survival 30 months, 10% progress to AML in 2 years

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44
Q

refractory anemia with excess blasts

A 
cytopenia, >65 yrs
about half how nonspecific cytogenetic abnormalities
CD34+ and/or CD117+
RAEB-1: 25% progress to AML
RAEB-2: 33% progress to AML
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45
Q

bone marrow

A

VDJ rearrangement

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46
Q

paracortex

A

expands in response to viral infection

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47
Q

multiple myeloma

A

light chain restricted

Poor risk: B2M (>/=4), LDH (>/= LDH), CRP (>/=4), Creatinine (>/=2), focal lesions (>/=5)del 17p (p53 region)

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48
Q

Chronic lymphocytic leukemia/lymphoma (CLL)

A

kappa OR gamma light chain, memory B cells
del13q14.3 > trisomy 12 > del11q22-23, del17p13 (p53)
CD20 weak, CD5+, CD23+
peripheral blood > bone marrow, lymph nodes
chronic, high familial incidence, lymphocytosis in older males

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49
Q

mantle cell lymphoma (MCL)

A

t(11;14)(q13;q32)(IgH;cyclin D1)
over expression of cyclin D1 under the control of IgH promoter (FISH): G1-> S phase
kappa OR gamma light chain, mantle cell
CD20 strong, CD5+, CD23-
lymph nodes > bone marrow,
spleen, peripheral blood, GI tract
lymphadenopathy and/or lymphocytosis in older males

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50
Q

follicular lymphoma

A

t(14;18)(q32;q21) in 85%, others possible: translocated an anti-apoptotic oncogene (BCL-2) to an IgH promoter
CD19+, CD20+, CD10+ (60%), BCL-2+ (90%), BCL-6+ (85%)
failure of germinal center B cells to apoptose
lymphadenopathy in older adults, often asymptomatic, variable, depends on stage, grade, cytogenetics
40-70% cases: bone marrow, can be involved in peripheral blood
30% progress: diffuse large B-cell lymphoma

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51
Q

diffuse large B-cell lymphoma

A

t(v, 3q27)(v, BCL-6): 30%
t(14;18): 20-30%
other translocations/deletions possible
CD19+, CD20+, CD10+ (30-60%)
rapidly growing adenopathy, elderly, 40% extra nodal disease (GI, bone marrow, other)
bone marrow involvement and appearance are clinical predictors

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52
Q

Classical hodgkin lymphoma

A 
most: CD30+, CD15+, Pax5+, CD20-/weak
males 30-50, localized or diffuse adenopathy (cervial, mediastinal or abdominal lymph nodes, spleen)
Reed/sternberg: NO IgG
cannot use flow cytometry or genetics
curable with chemo/RT
predictors: stage, histologic type
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53
Q

Reed/sternberg

A

constitutive NFkB OR EBV OR mutations in any anti-apoptotic pathways

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54
Q

nodular lymphocyte predominant hodgkin lymphoma (NLPHD)

A

popcorn cells instead of RS cells
CD30-, CD15-, CD20+, Pax5+, T-cells surround the R/s cells
R/S: IgG expression
80% 10 yr survival, may not need to treat stage 1 and 3-5% profession to diffuse B-cell lymphoma

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55
Q

Burkitt lymphoma

A

memory B cells
ileocecal area/ovaries/kidneys
sporadic: abdominal mass in children or young adults, higher incidence in HIV+
endemic: Jaw/facial bone mass (4-7 yrs) in a malaria area; EBV+
(8;14) or (8;2) or (8;22): translocatation of MYC (8q24) to an IgH or IgL (kappa or gamma) promoter

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56
Q

bone marrow aspirate for special studies

A

additional 5-20 cc: has blood and bone marrow

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57
Q

core biopsy

A

no blood forming elements if obtained from same site as aspirate

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58
Q

normal bone marrow aspirate

A

blasts <5%
myeloid: erythroid ratio 2:1 to 5:1
see all 3 lineages: meg, erythroid, myeloid

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59
Q

core biopsy

A

look for adequacy, cellularity, myeloid: erythoid ratio, iron stores, abnormal cells

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60
Q

core biopsy cellularity

A

cellularity: 100 - age

rest is fat

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61
Q

normal B-cell immunophenotype

A

CD45, CD79a, CD20, IgG kappa or lambda

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62
Q

normal T-cell immunophenotype

A

CD45, CD3 (TCR), CD7, CD4 or CD8

precursor T cells: TdT, CD3 (cytoplasmic), CD4 AND 8, CD7

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63
Q

side scatter (SSC)

A

flow cytometry
90 degrees off beam
high for cells with a lot of internal granules or segmented nuclei

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64
Q

forward scatter

A

flow cytometry
slightly off beam
proportional to size

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65
Q

CD45

A

on almost all marrow cells at different levels

can plot against SSC

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66
Q

immunohistochemistry

A

immunophenotyping by immune stain with enzyme conjugated Ab to proteins
unusually large cells, to see morphology of cells with markers

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67
Q

cytogenetic

A

must be dividing and arrest in mitosis to see chromosomes

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68
Q

RT-PCR

A

DNA/RNA sequencing

essential in Dx of AML

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69
Q

NPM1

A

oncogene

mutation: AML

70
Q

CEBPA

A

oncogene

mutation: AML

71
Q

class 1 mutations in AML

A

promote proliferation

72
Q

class 2 mutations in AML

A

inhibit differentiation

73
Q

complete genome or exome sequencing (epigenetics)

A

compare leukemia cells to skin biopsy

how malignancies are generated and how to diagnose, categorize and treat them

74
Q

acute myeloid leukemia (AML)

A

rapidly proliferating
myeloid, erythroid, or megs lineage
all ages

75
Q

acute undifferentiated leukemia

A

no committed lineage

76
Q

mutations that block differentiation

A

alter large patterns of gene expression

ex: transcription factor fusions, DNA methylation, chromatin modification mutations

77
Q

mutations that enhance proliferation

A

activate signaling initiated by extracellular ligands, DNA mehtylation

78
Q

Kit

A

tyrosine kinase

79
Q

genetic instability

A

TP53: tumor supressor
promote DNA repair (halt cell cycle) and induce apoptosis (damage or senescent)
both alleles disabled

80
Q

RUNX1-RUNX1T1

A 
t(8;21)(q22;q22): fusion of two TFs
AML: regardless of blast count
dominant neg. repressor of myeloid maturation
young patients
CD34+, HLA-DR+, CD13+, weak CD33
prognosis: good with chemo
81
Q

CBFB-MYH11

A

inv(16)(p13.1;q22): fusion of TFs with MYH11
AML: regardless of blast count
dominant neg. repressor of myeloid maturation
younger patients
CD34+, CD117+, CD13+, CD33+, CD14+, CD11b+
prognosis: better than most if risk adapted therapy is used

82
Q

PML-RARA

A

t(15;17)(q22;q12): fusion of PML (TF) with RARA (TF)
AML: regardless of blast count
APL: acute promyelocytic leukemia or AML-M3
dominant neg. blockade of normal RARA: inhibits granulocyte differentiation
severe thrombocytopenia
CD13+, CD33+
weak/absent CD34, HLA-DR
Tx: all trans retinoic acid (ATRA) induces differentiation of blasts to granulocytes
prognosis: good with Dx

83
Q

RARA

A

retinoid acid receptor alpha

84
Q

Runx1

A

part of heterodimeric TF called core binding factor (CBF)

85
Q

CBFB

A

part of heterodimeric TF called core binding factor (CBF)

86
Q

CD117

A

blasts

87
Q

CD11b

A

monocytes

88
Q

AML with normal cytogenetics

A

any age, most cases
blast markers: CD34+ and CD117+
prognosis: depends on sequencing

89
Q

AML with complex karyotype

A 
three or more cytogenic findings (translocations, trisomies, monosomies)
often effects TP53
5-10% of cases 
any age
blast markers: CD34+, CD117+
typically CD33+
prognosis: poor
90
Q

chronic eosinophilic leukemia (CEL)

A

myeloproliferative disease
increased eosinophils (monoclonal): molecular or cytogenetics, increased blasts
some respond to imatinib (FIPL1-PDGFRA)
Complications: myocarditis, lung infiltration, enteritis, encephalopathy, neuropathy, thromboses, eczema, angiodema
Tx for organ damage: glucocorticoids, hydroxyurea, IFN

91
Q

PDGFR neoplasm

A

myeloproliferative disease
increased eosinophils
some respond to imatinib

92
Q

chronic neutrophilic leukemia

A

myeloproliferative disease
increased neutrophils
rare

93
Q

mastocytosis

A

myeloproliferative disease
increased mast cells
cKIT mutation or PDGFRA activation (FIP1 translocation)
kids, benign cutaneous lesions (urticaria pigmentosa most common)
if spread from skin (confusing presentation): flushing, abdominal pain, tachycardia, hypotension
tryptase, CD117 (c-kit, the SCF receptor), CD25 usually
some respond to imatinib

94
Q

SCF

A

role in growth and migration of mast cells

95
Q

plasma cell neoplasm

A

elderly
CD38+++, CD138+++, light chain restricted
CD19-, CD20-
2/3 cases: translocation of IgH to various oncogenes (FISH)
hyperdiploidy: trisomies of ODD numbered chromosomes
bone marrow > peripheral blood
mild: asymptomatic lab findings (monoclonal gammopathy of uncertain significance, MGUS)
severe: multiple lytic bone lesions (plasma cell myeloma); pain, fractures, renal failure
increased total protein: SPEP and IFE, rouleaux

96
Q

mycosis fungoides

A

?

97
Q

plasma cell immunophenotype

A

CD38+, CD138+

CD20-,

98
Q

CLL: ZAP-70 expression

A

bad prognosis

99
Q

CLL: CD38 expression

A

bad prognosis

100
Q

CLL: 17p deletion (p53)

A

bad prognosis

101
Q

CLL: 13q deletion only

A

good prognosis

102
Q

CLL: presence of >30% smudge cells

A

good prognosis

103
Q

CLL: increasing fraction of immature forms (pro lymphocytes)

A

bad prognosis

104
Q

MCL: Ki-67

A

bad prognosis?

mitotic rate

105
Q

serum protein electrophoresis (SPEP)

A

use voltage to determine proteins

106
Q

immunofixation electrophoresis (IFE)

A

identifies abnormal single protein species
visualize with ELP
normal: immunoglobulin lanes show up as smears
abnormal: a single band

107
Q

Negative clinical predictors for plasma cell neoplasms

A

t(4;14) FGFR3
t(14;16) C-MAF
t(14;20) MAFB
del 17p (p53)

108
Q

Hodgkin lymphoma morphology

A

normal lymph node architecture wholly or partially effaced

can have: nodular sclerosis, mixed cellularity, mostly lymphocyte background, large number of RS cells

109
Q

NK cell immunophenotype

A

CD3+ cytoplasmic

110
Q

gamma delta T cell immunophenotype

A

CD3+

111
Q

mycosis fungoides

A

CD4+ T cell
CD3+, CD5+, CD4+
genetic: one predominant clonal re-arrangement of TCR gene
patchy, flat red skin lesions that can progress to think, psoriasis-like or ulcerated lesions

112
Q

sezary syndrome

A

mycosis fungicides that involves blood stream

113
Q

peripheral T cell lymphoma NOS

A 
expect: CD3, 5, 7,  and 4 or 8
actually see: loss of one or more
variants: CD4+/CD8+; CD20, CD56, CD30
complex karyotype 
diffuse lymphadenopathy, B symptoms (fever, night sweats, weight loss), paraneoplastic features (eosinophilia, pruritis, hemolytic anemia)
NOT in bloodstream but anywhere else
aggressive
114
Q

Common symptoms of pancytopenia?

A

mouth sores, fever, shortness of breath

115
Q

Most common etiology of aplastic anemia

A

idiopathic

116
Q

mechanisms that cause pancytopenia

A

bone marrow failure and destruction of blood cells in peripheral blood

117
Q

pancytopenia

A

low red cells, WBC, and platelets

118
Q

symptoms of anemia

A

difficulty breathing, chest pain, fatigue

119
Q

symptoms of leukopenia/neutropenia

A

fever, infection, mouth sores

120
Q

symptoms of thrombocytopenia

A

bleeding

121
Q

causes of pancytopenia

A

increased destruction: immune destruction, sepsis, hypersplenism
decreased production: myelodysplasia, marrow infiltrate, B12 deficiency, aplastic anemia, drugs, viruses, radiation

122
Q

causes of hyper cellular bone marrow failure

A

hematological malignancies: leukemia, myeloma, lymphoma

carcinoma, storage disorders, myelodyplastic syndromes, B12/folate deficiency

123
Q

causes of hypo cellular bone marrow failure

A

aplastic anemia

congenital: Faconi’s anemia
acquired: idiopathic (most), myelodysplastic syndrome, drugs (chloramphenicol, quinacrine), chemicals (benzene ring), radiation (recovers eventually), infection (mono, CMV, hepatitis, parvo, TB), paroxysmal nocturnal hemoglobinuria

124
Q

aplastic anemia

A

pancytopenia

hypo cellular bone marrow

125
Q

mechanism of idiopathic aplastic anemia

A

immune mediated destruction of hematopoietic stem cells
depletion of hematopoietic stem cells
damage, defective microenvironment, immunosuppression of hematopoeisis

126
Q

Faconi’s anemia

A

congenital
symptomatic: 5 yrs old
progressive bone marrow hypoplasia: aplastic
skin hyperpigmetnation, small stature

127
Q

familial aplastic anemia

A

subset of Faconi’s anemia without congenital defects

128
Q

paroxysomal nocturnal hemoglobinuria

A

membrane issue of RBC, WBC, platelets

complement mediated lysis

129
Q

aplastic anemia lab findings

A

severe pancytopenia with relative lymphocytosis
normochromic, normocytic RBCs
milde to moderate anisocytosis and poikilocytosis
decreased reticulocytes
hypo cellular bone marrow

130
Q

pure red cell aplasia

A

selective decrease in erythroid precursor
acquired: infection, hemolytic anemia, thymoma: T cell mediated responses against erythroblasts or EPO
Tx: supportive care, immunosuppression

131
Q

MDS Tx

A

hematopoetic GFs
hypomethylating agents: azacytidine and decitabine
lenalidomide for 5q syndrome
Stem cell transplant (HSCT/BMT/PBSCT)

132
Q

symptoms of acute leukemia

A

ferver (due to leukemia or infection), nose bleeds, fatigue, rash

133
Q

best way to differentiate between AML and ALL

A

flow cytometry- immunophenotyping

134
Q

primary AML

A

de novo

easier to treat than secondary

135
Q

secondary AML

A

develops from MDS or other hematological malignancy

136
Q

AML treatment

A

remission induction therapy: 1-2 courses of intensive therapy
post remission: 3-4 courses consolidation therapy
follow up in some: less intense maintenance therapy and allogenic bone marrow transplant

137
Q

ALL: poor prognosis

A

MLL-AF4 and BCR-ABL

138
Q

ALL: good prognosis

A

hyperdiploidy, E2A-PBX, TEL-AML

139
Q

Physical findings in polycythemia vera

A

Common: splenomegaly, skin plethora, conjunctival plethora,, HTN
other: engorged retinal vessels, headache, weak, pruritis, dizzy, sweating, visual disturbances, weight loss, paresthesia, dyspnea, joint symptoms, epigastric discomfort

140
Q

P. vera Tx

A 
phlebotomy
aspirin: decrease thrombosis
hydroxyurea: reduce thrombosis
Interferon: control platelets, HCT; reduce spleen size and alleviate pruritus 
can use Busulifan in elderly
JAK2 inhibitors
141
Q

causes of reduced platelets

A

inflammation, trauma, malignancy, iron deficiency, splenectomy, myeloproliferative neoplasm

142
Q

Tx of bleeding with ET

A

correct thrombocytosis
withdraw ASA and antithrombotics
maybe: DDAVP, antifibrinolytics, plasma products with vWF

143
Q

ET Tx

A

platelet reduction
hydroxyurea
anagrelide: decrease platelets, thrombosis, myelofibrotic transformation
interferon

144
Q

physical findings of chronic idiopathic myelofibrosis (CIM)

A

v. common: splenomegaly, hepatomegaly, fatigue, anemia, leukocytosis, thrombocytosis
common: asymptomatic, weight loss, night sweats, bleeding, splenic pain, leukopenia, thrombocytosis, thrombopenia

145
Q

MF Tx

A

high risk: allogenic SCT, Jak2 inhibitors
intermediate: Jak2 inhibitors, IMIDS, HDAC inhibitors, hypomethylators
low risk: observation

146
Q

stage I lymphoma

A

single node or lymphoid structure

147
Q

stage II lymphoma

A

two or more lymph regions onesie of diaphragm

148
Q

stage III lymphoma

A

both sides of diaphragm

149
Q

stage IV lymphoma

A

extra nodal beyond E (sole site of disease)

150
Q

Indolent lymphoma Tx

A 
local radiation
alkylators and prednisone
anthracyclines
fludarabine
Ab therapy
radiation labeled Ab
CHOP
151
Q

aggressive lymphoma Tx

A 
CHOP, HyperCVAD
Ab plus CHOP
radio-immuno therapy
short course chemo + radiation
bone marrow transplant for relapse
152
Q

R-CHOP

A

CD20 mAb : activates complement for macrophages to kill cells
plus CHOP
lymphoma

153
Q

alemtuzumab

A

CD 52 mAb; T cells

secondary infections common

154
Q

brentuximab vedotin

A

CD 30 mAb

Tx: hodgkins

155
Q

ibrutinib

A

bruton tyrosine kinase inhibitor

important for relapses

156
Q

GS-1101

A

PI3Kdelta inhibitor

157
Q

Fostamatinib

A

SYK inhibitor

158
Q

lymphomas caused by HIV

A

CNS lymphoma, aggressive B cell lymphoma

159
Q

lymphomas caused by EBV

A

after transplants

Burkitt’s

160
Q

lymphomas caused by HHV8

A

primary pleural effusion, castleman, kaposi sarcoma

161
Q

lymphomas caused by HTLV-1

A

T cell lymphoma

162
Q

lymphomas caused by H. pylori

A

MALT

163
Q

lymphomas caused by chlamydia

A

MALT lymphoma of eye

164
Q

CLL treatment

A

alkylators: chlorambucil, cyclophosphamide, bendamustin
fludarabine
chemo combo: FCR
immunotherapy: rituximab, alemtuzumab, ofatumumab and obinutuzumab (CD20)
radiation
corticosteroids

165
Q

Hairy cell leukemia

A

more often males, splenomegaly, pancytopenia, decreased cell mediated immuity, tartrate resistant acid phosphatase
CD 19, 20, 22, 11c, 25, 103
treat if symptomatic or if cytosine is severe
Tx: 2-chlorodeoxyadenosine, deoxycoformycin

166
Q

immunofixation electrophoresis (IFE)

A

identifies the type of monoclonal protein

167
Q

serum protein electrophoresis (SPEP)

A

best screening test for multiple myeloma
quantitative
M spike: monoclonal

168
Q

tests for multiple myeloma

A 
CBC
chemistry profile: assess renal function 
calcium
B2 microglobulin
SPES, IFX, quantitative immunoglobulins
UPEP: bence jones
serum free light chain
skeletal survey
unilateral bone marrow biopsy, cytogenetics, FISH
MRI/PET
169
Q

myeloma presentation

A

usually IgG
CRAB
hypercalcemia: altered mental status
renal insufficiency: light chain neuropathy, amyloid, uric acid, hypercalcemia, infection
bone destruction: pain, fracture, spinal cord compression
anemia, amyloidosis
hyperviscosity, hypogammaglobulinemia

170
Q

smoldering myeloma

A

No CRAB

monoclonal protein > 3gm/dl or bone marrow plasma cells >/= 10%

171
Q

myeloma Tx

A

alkylating agents, corticosteroids, anthracyclines, radiation, IMiDs, proteasome inhibitors, transplants, bisphosphonates

172
Q

IMiDs

A

immunomodulatory drugs

lenalidomide

SS- Heme (13 decks)

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