Hematologic Malignancies Flashcards
(172 cards)
1
Q
myeloid sarcoma
A
malignancy of lymph nodes, spleen, sub epithelium of the GI tract
2
Q
hematologic malignancy
A
abnormal proliferation of cells derived from those normally found in the blood, bone marrow, or lymphatic tissues
3
Q
leukemia
A
malignancy of bone marrow or blood
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
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
6
Q
chronic leukemia
A
slowly proliferating and not an immediate threat to patient’s life
7
Q
lymphoma
A
malignancy of lymph nodes, spleen, sub epithelium of GI tract
8
Q
lymphoproliferative disease
A
peripheral blood and lymphatic tissue
a.k.a. leukemia/lymphoma
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
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)
11
Q
CD34
A
hematopoietic stem cells marker
ALL marker
12
Q
CD33
A
granulocyte marker
ALL marker
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
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
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
16
Q
FLT3
A
tyrosine kinase
mutation: proliferation inducing activation AML
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
18
Q
Tet1/2
A
de-methylate cytosine residues
mutation (inactive): reduced differentiation
TFs: RARA, CBF
AML
19
Q
WT-1
A
localizes Tet to target genes
mutation (inactive): reduced differentiation
TFs: RARA, CBF
AML
20
Q
alpha-kg
A
cofactor for Tet: oxidize methyl group to hydroxymethyl group
21
Q
DNMT3A
A
methyl cytosine modification
mutation: proliferation inducing inactivation, decreases methylation
22
Q
Auer rod
A
myeloid blasts
crystallization of granule contents: mostly made up of myeloperoxidase
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
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+
25
MLL rearranged
```
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+
```
26
TEL-AML1 (ETV6-RUNX1)
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-
27
hyperdiploid
>50 chromosomes
| ALL: good prognosis
28
T-ALL
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
29
blast immunophenotype
CD34+
30
myeloid blast immunophenotype
CD34+, CD33+
31
lymphoid blast immunophenotype
TdT+, CD10+
32
mature B lymphocyte/lymphoma immunophenotype
CD19+, CD20+
centrocytes and centroblasts: CD10
blasts: CD10, TdT
33
mature T lymphocyte/lymphoma immunophenotype
CD3+, CD5+
34
chronic myelogenous leukemia (CML)
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)
35
chronic myelomonocytic leukemia (CMML)
myelodysplastic/myeloproliferative
v, PDGFRB
high WBC: monocytes, promonocytes, monocyte/granulocyte hybrids
36
myeloid bulge
more myelocytes than metamyelocytes: evidence of myeloproliferative neoplasm
37
polycythemia vera
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
38
essential thrombocythemia
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
39
primary myelofibrosis
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
40
refractory cytopenia with unilineage dysplasia
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
41
refractory anemia with ring sideroblasts
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
42
myelodysplastic syndrome with isolated del(5q)
```
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
```
43
refractory cytopenia with multilineage dysplasia
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
44
refractory anemia with excess blasts
```
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
```
45
bone marrow
VDJ rearrangement
46
paracortex
expands in response to viral infection
47
multiple myeloma
light chain restricted
| Poor risk: B2M (>/=4), LDH (>/= LDH), CRP (>/=4), Creatinine (>/=2), focal lesions (>/=5)del 17p (p53 region)
48
Chronic lymphocytic leukemia/lymphoma (CLL)
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
49
mantle cell lymphoma (MCL)
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
50
follicular lymphoma
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
51
diffuse large B-cell lymphoma
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
52
Classical hodgkin lymphoma
```
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
```
53
Reed/sternberg
constitutive NFkB OR EBV OR mutations in any anti-apoptotic pathways
54
nodular lymphocyte predominant hodgkin lymphoma (NLPHD)
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
55
Burkitt lymphoma
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
56
bone marrow aspirate for special studies
additional 5-20 cc: has blood and bone marrow
57
core biopsy
no blood forming elements if obtained from same site as aspirate
58
normal bone marrow aspirate
blasts <5%
myeloid: erythroid ratio 2:1 to 5:1
see all 3 lineages: meg, erythroid, myeloid
59
core biopsy
look for adequacy, cellularity, myeloid: erythoid ratio, iron stores, abnormal cells
60
core biopsy cellularity
cellularity: 100 - age
| rest is fat
61
normal B-cell immunophenotype
CD45, CD79a, CD20, IgG kappa or lambda
62
normal T-cell immunophenotype
CD45, CD3 (TCR), CD7, CD4 or CD8
| precursor T cells: TdT, CD3 (cytoplasmic), CD4 AND 8, CD7
63
side scatter (SSC)
flow cytometry
90 degrees off beam
high for cells with a lot of internal granules or segmented nuclei
64
forward scatter
flow cytometry
slightly off beam
proportional to size
65
CD45
on almost all marrow cells at different levels
| can plot against SSC
66
immunohistochemistry
immunophenotyping by immune stain with enzyme conjugated Ab to proteins
unusually large cells, to see morphology of cells with markers
67
cytogenetic
must be dividing and arrest in mitosis to see chromosomes
68
RT-PCR
DNA/RNA sequencing
| essential in Dx of AML
69
NPM1
oncogene
| mutation: AML
70
CEBPA
oncogene
| mutation: AML
71
class 1 mutations in AML
promote proliferation
72
class 2 mutations in AML
inhibit differentiation
73
complete genome or exome sequencing (epigenetics)
compare leukemia cells to skin biopsy
| how malignancies are generated and how to diagnose, categorize and treat them
74
acute myeloid leukemia (AML)
rapidly proliferating
myeloid, erythroid, or megs lineage
all ages
75
acute undifferentiated leukemia
no committed lineage
76
mutations that block differentiation
alter large patterns of gene expression
| ex: transcription factor fusions, DNA methylation, chromatin modification mutations
77
mutations that enhance proliferation
activate signaling initiated by extracellular ligands, DNA mehtylation
78
Kit
tyrosine kinase
79
genetic instability
TP53: tumor supressor
promote DNA repair (halt cell cycle) and induce apoptosis (damage or senescent)
both alleles disabled
80
RUNX1-RUNX1T1
```
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
CBFB-MYH11
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
PML-RARA
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
RARA
retinoid acid receptor alpha
84
Runx1
part of heterodimeric TF called core binding factor (CBF)
85
CBFB
part of heterodimeric TF called core binding factor (CBF)
86
CD117
blasts
87
CD11b
monocytes
88
AML with normal cytogenetics
any age, most cases
blast markers: CD34+ and CD117+
prognosis: depends on sequencing
89
AML with complex karyotype
```
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
chronic eosinophilic leukemia (CEL)
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
PDGFR neoplasm
myeloproliferative disease
increased eosinophils
some respond to imatinib
92
chronic neutrophilic leukemia
myeloproliferative disease
increased neutrophils
rare
93
mastocytosis
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
SCF
role in growth and migration of mast cells
95
plasma cell neoplasm
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
mycosis fungoides
?
97
plasma cell immunophenotype
CD38+, CD138+
| CD20-,
98
CLL: ZAP-70 expression
bad prognosis
99
CLL: CD38 expression
bad prognosis
100
CLL: 17p deletion (p53)
bad prognosis
101
CLL: 13q deletion only
good prognosis
102
CLL: presence of >30% smudge cells
good prognosis
103
CLL: increasing fraction of immature forms (pro lymphocytes)
bad prognosis
104
MCL: Ki-67
bad prognosis?
| mitotic rate
105
serum protein electrophoresis (SPEP)
use voltage to determine proteins
106
immunofixation electrophoresis (IFE)
identifies abnormal single protein species
visualize with ELP
normal: immunoglobulin lanes show up as smears
abnormal: a single band
107
Negative clinical predictors for plasma cell neoplasms
t(4;14) FGFR3
t(14;16) C-MAF
t(14;20) MAFB
del 17p (p53)
108
Hodgkin lymphoma morphology
normal lymph node architecture wholly or partially effaced
| can have: nodular sclerosis, mixed cellularity, mostly lymphocyte background, large number of RS cells
109
NK cell immunophenotype
CD3+ cytoplasmic
110
gamma delta T cell immunophenotype
CD3+
111
mycosis fungoides
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
sezary syndrome
mycosis fungicides that involves blood stream
113
peripheral T cell lymphoma NOS
```
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
Common symptoms of pancytopenia?
mouth sores, fever, shortness of breath
115
Most common etiology of aplastic anemia
idiopathic
116
mechanisms that cause pancytopenia
bone marrow failure and destruction of blood cells in peripheral blood
117
pancytopenia
low red cells, WBC, and platelets
118
symptoms of anemia
difficulty breathing, chest pain, fatigue
119
symptoms of leukopenia/neutropenia
fever, infection, mouth sores
120
symptoms of thrombocytopenia
bleeding
121
causes of pancytopenia
increased destruction: immune destruction, sepsis, hypersplenism
decreased production: myelodysplasia, marrow infiltrate, B12 deficiency, aplastic anemia, drugs, viruses, radiation
122
causes of hyper cellular bone marrow failure
hematological malignancies: leukemia, myeloma, lymphoma
| carcinoma, storage disorders, myelodyplastic syndromes, B12/folate deficiency
123
causes of hypo cellular bone marrow failure
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
aplastic anemia
pancytopenia
| hypo cellular bone marrow
125
mechanism of idiopathic aplastic anemia
immune mediated destruction of hematopoietic stem cells
depletion of hematopoietic stem cells
damage, defective microenvironment, immunosuppression of hematopoeisis
126
Faconi's anemia
congenital
symptomatic: 5 yrs old
progressive bone marrow hypoplasia: aplastic
skin hyperpigmetnation, small stature
127
familial aplastic anemia
subset of Faconi's anemia without congenital defects
128
paroxysomal nocturnal hemoglobinuria
membrane issue of RBC, WBC, platelets
| complement mediated lysis
129
aplastic anemia lab findings
severe pancytopenia with relative lymphocytosis
normochromic, normocytic RBCs
milde to moderate anisocytosis and poikilocytosis
decreased reticulocytes
hypo cellular bone marrow
130
pure red cell aplasia
selective decrease in erythroid precursor
acquired: infection, hemolytic anemia, thymoma: T cell mediated responses against erythroblasts or EPO
Tx: supportive care, immunosuppression
131
MDS Tx
hematopoetic GFs
hypomethylating agents: azacytidine and decitabine
lenalidomide for 5q syndrome
Stem cell transplant (HSCT/BMT/PBSCT)
132
symptoms of acute leukemia
ferver (due to leukemia or infection), nose bleeds, fatigue, rash
133
best way to differentiate between AML and ALL
flow cytometry- immunophenotyping
134
primary AML
de novo
| easier to treat than secondary
135
secondary AML
develops from MDS or other hematological malignancy
136
AML treatment
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
ALL: poor prognosis
MLL-AF4 and BCR-ABL
138
ALL: good prognosis
hyperdiploidy, E2A-PBX, TEL-AML
139
Physical findings in polycythemia vera
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
P. vera Tx
```
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
causes of reduced platelets
inflammation, trauma, malignancy, iron deficiency, splenectomy, myeloproliferative neoplasm
142
Tx of bleeding with ET
correct thrombocytosis
withdraw ASA and antithrombotics
maybe: DDAVP, antifibrinolytics, plasma products with vWF
143
ET Tx
platelet reduction
hydroxyurea
anagrelide: decrease platelets, thrombosis, myelofibrotic transformation
interferon
144
physical findings of chronic idiopathic myelofibrosis (CIM)
v. common: splenomegaly, hepatomegaly, fatigue, anemia, leukocytosis, thrombocytosis
common: asymptomatic, weight loss, night sweats, bleeding, splenic pain, leukopenia, thrombocytosis, thrombopenia
145
MF Tx
high risk: allogenic SCT, Jak2 inhibitors
intermediate: Jak2 inhibitors, IMIDS, HDAC inhibitors, hypomethylators
low risk: observation
146
stage I lymphoma
single node or lymphoid structure
147
stage II lymphoma
two or more lymph regions onesie of diaphragm
148
stage III lymphoma
both sides of diaphragm
149
stage IV lymphoma
extra nodal beyond E (sole site of disease)
150
Indolent lymphoma Tx
```
local radiation
alkylators and prednisone
anthracyclines
fludarabine
Ab therapy
radiation labeled Ab
CHOP
```
151
aggressive lymphoma Tx
```
CHOP, HyperCVAD
Ab plus CHOP
radio-immuno therapy
short course chemo + radiation
bone marrow transplant for relapse
```
152
R-CHOP
CD20 mAb : activates complement for macrophages to kill cells
plus CHOP
lymphoma
153
alemtuzumab
CD 52 mAb; T cells
| secondary infections common
154
brentuximab vedotin
CD 30 mAb
| Tx: hodgkins
155
ibrutinib
bruton tyrosine kinase inhibitor
| important for relapses
156
GS-1101
PI3Kdelta inhibitor
157
Fostamatinib
SYK inhibitor
158
lymphomas caused by HIV
CNS lymphoma, aggressive B cell lymphoma
159
lymphomas caused by EBV
after transplants
| Burkitt's
160
lymphomas caused by HHV8
primary pleural effusion, castleman, kaposi sarcoma
161
lymphomas caused by HTLV-1
T cell lymphoma
162
lymphomas caused by H. pylori
MALT
163
lymphomas caused by chlamydia
MALT lymphoma of eye
164
CLL treatment
alkylators: chlorambucil, cyclophosphamide, bendamustin
fludarabine
chemo combo: FCR
immunotherapy: rituximab, alemtuzumab, ofatumumab and obinutuzumab (CD20)
radiation
corticosteroids
165
Hairy cell leukemia
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
immunofixation electrophoresis (IFE)
identifies the type of monoclonal protein
167
serum protein electrophoresis (SPEP)
best screening test for multiple myeloma
quantitative
M spike: monoclonal
168
tests for multiple myeloma
```
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
myeloma presentation
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
smoldering myeloma
No CRAB
| monoclonal protein > 3gm/dl or bone marrow plasma cells >/= 10%
171
myeloma Tx
alkylating agents, corticosteroids, anthracyclines, radiation, IMiDs, proteasome inhibitors, transplants, bisphosphonates
172
IMiDs
immunomodulatory drugs
| lenalidomide
