Path part 2 ho2 Flashcards
1
Q
What is the gold standard in investigation of acute lymphadenitis?
A
culture
2
Q
What is the most common type of reactive lymphoid hyperplasia, especially in children?
A
follicular lymphoid hyperplasia
3
Q
The paracortical pattern of reactive lymphoid hyperplasia has…
A
interfollicular expansion, mainly T cells
4
Q
What are the three patterns of reactive lymphoid hyperplasia?
A
follicular pattern
paracortical pattern
sinus pattern
5
Q
When would you see a sinus pattern of reactive lymphoid hyperplasia?
A
lymph nodes draining tumors
whipple disease
rosai-dorfman
6
Q
What is strongly associated as a cause of paracortical lymphoid hyperplasia? What is the description of this?
A
infectious mononucleosis;
paracortical region expanded, residual follicles are present but obscured
7
Q
Which one is the non-necrotizing granuloma of sarcoidosis?
A
the darker purple image
8
Q
When metastatic tumor cells invade the lymph node, what is the order of locations in which they appear?
A
first in marginal sinus, then penetrate medually sinuses, then medulla, then cortex
9
Q
Mets to the L supraclavicular node is probably from a primary tumor of…
A
abdominal, esp gastric
10
Q
What is the MC congenital abnormality of the spleen? what is this also called?
A
accessory spleen
spleniculi
11
Q
When is a congenital accessory spleen even significant?
A
in hematologic disorders when splenectomy is a tx
12
Q
“septic splenitis” is a response to systemic infection by the spleen, seen in ….
A
follicular (lymphoid) hyperplasia
this can be acute (EBV inf) or chronic
13
Q
thick white plaques covering the surface of the spleen, common incidental finding at autopsy
A
perisplenitis
14
Q
most commonly, cirrhosis will cause splenomegaly due to its…
A
congestive state
15
Q
congestive splenomegaly is a direct result of …
A
venous outflow obstruction
16
Q
thrombosis of hepatic veins causing splenomegaly
A
Budd-Chiari syndrome
17
Q
What are some causes of splenic infarcts?
A
endocarditis
severe atherosclerosis
massive splenomegaly
thrombosis of splenic vein
18
Q
splenic infarct appearance
A
pale, tan, wedge shaped, esp if arterial
subcapsular
19
Q
What are the MCC of splenic ruptures?
A
trauma and surgical intervention
20
Q
What are splenic inplants?
A
splenosis - little mini spleens appearing after rupture
21
Q
What could hypersplenism cause? How could you resolve this issue?
A
any kind of blood cytopenia due to increased functioning (splenic sequestration and destruction)
typically resolve after splenectomy
22
Q
What are some causes of hypersplenism?
A
autoimmune diseases
congestive splenomegaly
Gaucher disease
23
Q
What is the MC primary tumor of the spleen?
A
hemangioma - usually cavernous type and less than 2 cm in size
24
Q
What is the morphology of lymphangiomas? Who do they occur in?
A
subcapsular region of the spleen; multicystic; lumina contain proteinaceous material, not RBCs; endothelium may form small papillary projections
most cases in children
25
A rare, nodular lesion of splenic sinus lining cells, usually an incidental finding...
hamartoma
26
a malignant primary tumor of the spleen is rare, but the MC is...
angiosarcoma
well-defined hemorrhagic nodule or involve diffuse spleen
can lead to spontaneous rupture of organ
can be after FB placement (gauze sponge)
27
What are the MC type of malignancy in the spleen?
lymphomas
splenic involvement is usually secondary
DLBCL is MC and can be restricted to only the spleen
28
What are the most common primary sites if the spleen is getting mets?
melanoma
lung
breast
stomach
pancreas
liver
colon
29
premature infants or term infants exposed to stress in utero my have this issue with the thymus... what does this mean?
acute thymic involution
greatly increased lymphocyte death
and is probs mediated by high levels of steroids (adult cases)
30
an incidental finding during thyroid surgery in surgery in preteens, rare in adults
ectopic thymus tissue
31
What ectopic tissue is MC found in thymus?
parathyroid
32
Thymic hypoplasia syndrome
DiGeorge syndrome
33
Thymic hyperplasia
reactive B lymphoid follicles within the thymus seen in chronic inflammatory and immunologic states, esp myasthenia gravis
34
MC primary anterior mediastinal neoplasm....
made of what?
benign or malignant?
associated with anything?
thymoma
tumor of thymic epithelial cells
benign, mostly
parneoplastic syndromes, such as MG
35
What is this and what do the histo types look like?
benign thymoma - grossly lobulated and usually encapsulated mass
spindly type (type A) is swirly and has oval cells
epithelioid type (type B) is pin point pretty dots throughout
can also have mixed
36
What are the two types of malignant thymoma?
invasive thymoma - benign cytologic features, but locally aggressive architectural features (invasion through capsule, pleural or pericardial implants, distant metastases)
thymic carcinoma - malignant cytologic features, most commonly like SCC; not associated with MG
37
What are the main primaries of metastatic thymus disease?
esophagus
lung, pleura
breast
thyroid
melanoma
38
monoclonal proliferation is considered to be...
neoplastic
39
Who is affected by B cell NHL?
Where do they originate?
spread is...
staging NHL
mostly older adults
LN, but can be other tissues
unpredictable
is not super useful
40
What is the MC B cell NHL? follow by...
Diffuse large B cell lymphoma
follicular lymphoma
41
What is the MC leukemia in adults?
chronic lymphocytic leukemia/small lymphocytic lymphoma
42
What is the pathogenesis behind CLL/SLL?
Bcl-2 gets upregulated, an anti-apoptotic gene
43
If bone marrow is not involved in CLL, what is it called?
Small lymphocytic lymphoma
44
Clinical presentation of CLL
most are asymptomatic at dx with lymphocytosis on CBC
ssx: fatigue, hemolytic anemia, infections, **lymphadenopathy,** hepatosplenomegaly
usually an indolent disease, but can transform to a higher grade lymphoma
45
CLL lab findings
CBC - lymphocytosis \>5k
may have monoclonal ig
periph blood cytopenias
incrased LDH
46
CLL peripheral smear has...
smudge cells
coarse, clumped chromatin making soccer ball appearances
47
Rai staging in CLL
0. lymphocytosis in blood and marrow
1. lymphocytosis and lymphadenopathy
2. lymphocytosis and hepatosplenomegaly
3. lymphocytosis and anemia
4. lymphocytosis and thrombocytopenia
(higher stage = worse prognosis)
48
Binet staging CLL
A. no cytopenia, \<3 lymphoid areas enlarged
B. no cytopenia, \>=3 lymphoid areas enlarged
C. anemia or thrombocytopenia
49
What are three things that CLL could transform into?
Prolymphocytic transformation - increase # lymphocytes with more distinct nucleoli; prolymphocytes incrase, 2x the size of nl - death in 2 years
Richter transformation - transforms to DLCL with retention of CLL immunophenotype, usually extrameduallary, death in less than a year
Transformation to hodgkin lymphoma - reed sternberg cells appearing
50
DLBCL affects who?
How does it come about?
prognosis?
middle age and elderly
arises de novo usually
aggressive and requires therapy
51
What are sites of involvement of DLBCL?
LN most often, can affect GI tract and spleen
52
What is the clinical presentation of DLBCL?
rapidly enlarging nodal or extranodal masses
systemic B symptoms
increased LDH and beta-2 microglobulin
most present late
53
What is the MC variant of DLBCL?
centroblastic variant - cells look like centroblasts of germinal centers, large cells with moderate amount cytoplasm, vesicular nuclei with 2-3 small nucleoli
54
How would you tx DLBCL?
de novo arising - could cure with R-CHOP
if secondary arising, probs resistant to therapy
55
follicular lymphoma defined, path, and prognosis
malignant germinal center B cells
t(14:18) causing overexpression of anti-apoptotic BCL2
indolent but incurable, may transform to DLBCL (Richter transformation)
56
What is the gross appearance of follicular lymphoma?
spleen with lots of small nodules
LN has tan/white "fish flesh" appearance, +/- many small nodules
57
What will the microscopic veiw of follicular lymphoma look like?
close follicles without mantle or marginal zones (no super dark blue cells around circles)
"snake bite" cells/centroblasts
58
What differentiates reactive follicles from follicular lymphomas?
Bcl-2 stain, positive in lymphomas
| (much darker area)
59
Rarely, peripheral blood is involved in follulicular lymphoma, but when it is, I see...
buttock cells - midline cleaved appearance
60
What is a plasma cell myeloma?
malignancy of monotypic plasma cells with clinical BM plasmacytosis, osteolytic bone lesions, and monoclonal gammopathy
61
What are some clinical features of plasma cell myeloma?
varying stages in presentation
multifocal bone lesions
monoclonal protein (M-protein) in urine, serum, or both
renal failure is common due to M protein damage
increased susceptibility in older persons
62
A person with plasma cell myeloma will usually die from...
bleeding or infection
63
What is the morphology of plasma cell myeloma like?
rouleax is common in peripheral blood (stacking of RBCs)
varying appearance of plasma cells with different growth patterns
64
What can occur in the absence of myeloma, though it is considered a plasma cell neoplasm?
primary amyloidosis
65
What is the structure associated with primary amyloidosis?
What kind of staining goes with amyloidosis?
due to extracellular deposition of fibrillar proteins having beta pleated sheet tertiary structure
a/w plasma cell neoplasm is **AL - amyloid light chain**
congo red stainging, with apple=green birefrengence on polarized light microscopy are seen with all forms of amyloid
66
What are solitary neoplasms of monoclonal plasma cells like?
pretty much how they sound; they have no clinical features of myeloma
Ig normal, no anemia, no hypercalcemia, no renal failure
typically in middle aged
can be extraosseous - outside of bone marrow (better prognosis), or intraossesous (worse prognosis)
67
How do you tx solitary neoplasms of monoclonal plasma cells?
all tx by radiotherapy only
68
Where will you most often find extraosseous plasmacytoma?
upper respiratory tract and sinuses
69
Solitary plasmacytoma of bone, 2/3 of the time...
evolve into myeloma or additional plasmacytomas
70
What is lymphoplasmacytic lymphoma?
rare neoplasm, small B cells, plasmacytoid lymphocytes, and plasma cells
no specific immunophenotype or genetic abnls
includes Waldenstrom macroglobulinemia
71
What are clinical features of LPL?
behavior is that of lymphoma, not myeloma, as there are no bony lesions
majority have monoclonal protein (IgM mostly, Waldenstroms)
hyperviscosity of blood in \<30% of cases (purpura rash)
cryoglobulinemia, raynaud like, a/w Hep C
cold agglutinin hemolytic anemia in 5%
72
What is the morphology of LPL?
bone marrow involvement is constant
mix of small lymphocytes, plasmacytoid lymphocytes, and plasma cells
mast cells often increased
Dutcher bodies (intranuclear pseudoinclusions in some plasma cells)
73
What infection/conditions are a/w MALT lymphomas?
H. pylori infection
chronic inflammatory conditions
74
MALT lymphoma symptoms are...
usually related to organ involved, not usually involving BM
75
How would you tx MALT lymphoma?
tx the infection or underlying disease
76
old man, diffuse adenopathy, marrow positive, CD5 marker, Cyclin D1, t(11:14)
Mantle cell lymphoma
77
What does mantle cell lymphoma look like?
diffuse or nodular proliferation of monomorphic small lymphocytes, without prolif centers or centroblasts
no plasma cells
78
What can mantle cell lymphoma transform into?
can go to blastoid variant, very aggressive
cells resemble blasts and the ddx includes Burkitt lymphoma and ALL but cells are mature, with typical MCL immunophenotype
79
AA boy with jaw swelling
Burkitt lymphoma, aggressive rapidly growing B cell neoplasm
80
Endemic Burkitt lymphoma characteristics
Africa
kids
jaw and facial bone involved, or gonadal, breast, abdominal sites
geographically corresponds to malaria
EBV positive in most cases
81
Sporadic Burkitt Lymphom appearance
WW, low incidence
children and YA
abdominal tumors MC, also gonads, kidneys, and breast (jaw tumors rare)
Only 30% EBV positive
82
immunodeficiency associated Burkitt lymphoma
adults
HIV/AIDS
post-transplant
iatrogenic (drugs for AI dz)
involves LN and BM in most
EBV varies
83
Burkitt lymphoma immunophenotype
Mature B cell markers - CD 19, CD20, CD10, BCL6, CD38
CD34 and TdT are uniformly negative (no blasts)
t(8:14) c-myc mutation, instead of making Ig, actively divides
84
What is the morphology a/w Burkitt lymphoma?
starry sky appearance
85
What is the MC childhood malignancy (hematopoietic or solid tumor)
B- ALL
86
What causes an increased risk for B-ALL and AML
Down syndrome, genetic alterations, radiation, toxin, previous chemo
87
B-ALL/ LBL morphology
leukoerythroblastosis common
blasts in blood and bone marrow varying in size
L1 blasts are smaller and look like small mature lymphocytes
L2 blasts are larger, with 'smudgey' handmirror appearance
88
bone marrow biopsy of B-ALL
hypercellular, increased lymphoblasts and lots of mitoses, packed with blasts
rarely will have regions of necrosis
89
What is the MC hematopoietic neoplasm that causes marrow necrosis?
ALL
90
What will the immunophenotype be of B-ALL?
CD19 and other pan-Bcell markers
plus markers of immaturity CD34 and TdT
91
What scenarios account for the best prognosis of B-ALL
age 1-10 yo
female gender
WBC \<50k
no CNS dz
common immunophenotype
hyperdiploidy (\>50 chromosomes)
92
What factors into a bad pronosis for B-ALL?
less than 1 year or greater than 10yo
male
WBC \>50k
CNS disease
absences of CD10
hypodiploidy
93
T-ALL and T-LBL is more common in...
adolescent males
94
clinical presentation of T-ALL
bone marrow always involved
mediastinal involvement with rapidly growing mass
LAD and heptosplenomegaly
CNS involvement is MC than with T-LBL
95
T- LBL clinical presentation
only in BM of 20% of cases at dx
rapidly growing mediastinal mass
pleural and pericardial effusions often present
96
T-ALL blasts immunophenotypes
CD2, CD5, CD7, TdT, CD34, CD10+/-
97
How are T cell and NK cell neoplasms more likely to be detected
more likely detected by T cell antigens lost than expressed
98
As a group, which has generally worse prognosis between T-ALL and B-ALL
T-ALL
99
What is the MC primary cutaneous T-cell lymphoma?
mycoses fungioidies
100
What markers might cells in mycoses fungioides have?
CD4 T cells with loss of CD7 or another cell marker
101
What is the clinical course of mycoses fungioides?
indolent for years to decades limited to the skin
extracutaneous dissemination may occur in late stages, BM involvement rare
pre-mycotic period - nonspecific skin lesions, often with slight scaling or pruritis
MF develops..
initial patch, inflitrated plaque, tumor stage
102
cytologic features of Mycosis fungioides
small/med lymphocytes with cerebriform nuclei, like sezary cells
Pautrier microabscess develop in the plaque stage
103
a form of leukemia,
has involvement of skin, blood, and LNs
usually arises de novo, sometimes with Mycosis fungioides
has a triad of...
sezary syndrome
erythroderma, generalized LAD, cerebriform nuclei in skin, peripheral blood and LNs
104
What are these cells and what are they seen in?
cerebriform nuclei
sezary syndrome, mycosis fungioides
105
What are markers of Adult T cell leukemia/lymphoma (ATLL)?
only in person with HTLV-1
from CD4 and CD25 positive T reg cells
old man from carribean
106
What occurs in acute ATLL?
"flower cells", LAD, skin lesions\*, increased LDH, hypercalcemia, lytic bone lesions, renal disease, neuropsych disease
107
What are three definitive parts of chronic ATLL?
lymphocytosis
exfoliative skin rash
NO hypercalcemia
108
What are markers of lymphomatous ATLL?
worse LAD than acute
skin involved
less hypercalcemia than acute
no blood involvement
109
What are features of smolder ATLL?
\>5% tumor cells in periph blood, but normal WBC count
tumor cells less atypical
no hypercalcemia or LAD
110
What has pautrier like microabscesses on histo and is HTLV-1 positive?
ATLL skin
111
T cell large granular lymphocytic leukemia features
persistent (\>6 mo) clonal proliferation of CD8 T cell large granular lymphocytes
T cell receptor is clonally rearranged
variable cytopenias
avg 60 yo
STAT3 mutation in 40%
112
What is this a histo pic of? describe it
T LGLL
large granular lymphocytes, intermediate size, inconspicuous nucleoli and abundant cytoplasm with azurophilic granules
113
Anaplastic large cell lymphoma most often affect who? what do they often have, ssx?
adolescents and YA
often have "B symptoms" esp high fever
114
What stage does a pt with anaplastic large cell lymphoma usually present in? Is this treatable?
stage III/IV
yes, somewhat tx'able, 80-90% survivial
115
What features are extremely characteristic of anaplastic large cell lymphomas?
occasional horseshoe shaped nuclei (hallmark cells), express CD30 and some T cell markers
t(2:5) NPM/ALK
ALK positive
consistently negative for EBV
116
What is circled?
hallmark cell of anaplastic large cell lymphoma
117
Is staging more important for NHL or HL?
HL
118
Where does HL originate?
nodal based
119
What is the pathognomic cell of HL?
reed sternberg cells- abnormal B cells
120
What does Nodular Lymphocyte Predominant Hodgkin lymphoma look like on histo?
nodal architecture is distored by multiple expansile nodules with compressed interfollicular zones
nodules have a moth-eaten appearance
121
What are these slides of?
Nodular LP hodgkin lymphoma
mummified condensed cytoplasm and nuclei with irregular nuclear contours on L
clusters of histiocytes forming granulomas on the R
122
Where will I find popcorn cells?
Neoplastic cells of NLPHL
they are lymphocyte predominate (LP) or lymphohistiocytic (LH) cells
large cells with multilobulated nuclear contours and resemble popcorn
123
What is the clinical presentation of a pt with Nodular LP hodgkins lymphoma?
lab tests?
grossly, the LN is...
male, 35 yo, LAD, not often having B symptoms
normal CBC, not leukemic, rarely LDH or beta-2 elevated
large and multinodular
124
Ancillary testing done for NLPHL would be...
IHC stain: LP cells + for CD45, CD20, and some other B cell markers
flow cyt: usually negative (true of all HL for the most part)
PCR: heavy chain rearrangements found when using singe cell PCR
60% have abnl BCL6 gene locus
125
Classical hodgkin lymphomas are in what ages of people?
bimodal age curve
126
What is this guy?
Reed sternberg cells
must have 2 nucleoli in two separate nuclear lobes to be called diagnostic
127
pyknotic, reddish reed sternberg cells are called...
mummifed cells
128
What are reed sternberg cells immunophenotype?
CD30 +
maybe CD15
negative for CD20 and CD45
129
What is the MC type of classic hodgkin lymphoma in developed world? What does it look like?
nodular sclerosis CHL
nodules of neoplastic cells admixed with inflammatory cells, surrounded by collagenous bands
neoplastic cells are mainly lacunar cells (HRS variant)
130
nodes tending to be matted, with visible nodulatiry and fibrous bands around the nodules are what type of CHL?
nodular sclerosis CHL
131
What is this?
nodular sclerosis CHL
one has fibrous bands and the other has the lacunar cells
132
Type of CHL with classic reed sternberg and mononuclear hodgkin cells, in highly mixed inflammatory background, with diffuse or interfollicular pattern without nodules or fibrosis
mixed cellularity CHL
133
MC CHL in underdeveloped world?
mixed cellularity CHL
134
What is this?
mixed cellularity CHL with classic reed sternberg
135
CHL with predominance of small reactive lymphocytes...
Lymphocyte rich CHL
136
Involvement of ________ is common in lymphocyte rich CHL
Waldeyer ring
137
What does the background of lymphocyte rich CHL look like?
follicular dendritic cells, similar to NLPHL
138
What is this?
lymphocyte rich CHL
'open' areas are actually larger cells with increased cytoplasm
139
likely a progression from other types of CHL... in older pts at onset... presents in what stage?
lymphocyte deplete CHL
stage III or IV presentation
140
What is this?
lymphocyte depleted CHL
lots of reed sternberg
few small lymphocytes, inflammatory background
141
What are common features of all types of myeloid neoplasms?
primarily involve blood and bone marrow
loss of normal hematopoeitc control in neoplastic cells
suppression of normal hematopoietic cells
can all progress to AML
142
Chronic myeloid leukemia is most frequent in this age and has this abnormality
middle aged
Philadelphia chromosome t(9:22) BCR/ABL1
143
What are periph blood findings in CML?
leukocytosis and and granulocytic left shift
basophilia
eosinophilia common
blasts \<2%
144
Clinical presenation in CML usually has
40-60 yo
splenomegaly
anemia
NO LAD
anemia
platelet dysfunction
gout or renal disease from hyperuricemia
145
Bone marrow findings in CML
increased cellularity
increased megakaryocytes with small monolobulated forms (dwarf megakaryocytes)
Pseudo-Gaucher cells (sea blue histiocytes) due to increased cell turnover
146
When is CML considered to be in the "blast" stage? Once in this stage, what is the prognosis?
when blasts comprise \>=20% of the peripheral blood WBC or the nucleated cells of marrow
usually fatal within weeks-months
147
death in CML is usually due to...
infection or bleeding
148
panmyelosis is usually seen in ....
what is responsible for most of the clinical ssx?
polycythemia vera
increase in RBCs
149
What gain of function mutation is polycythemia vera a.w?
JAK 2
150
RBCs responsible for ssx of polycythemia vera present as...
HTN, HA, dizziness, venous or arterial thrombosis, Splenomegaly
151
What are the three phases of polycythemia vera?
pre-polycythemic phase (missed usually)
polycythemic phase - RBC increase in periph blood but normal morph. BM is hypercellular with panmyelosis; megakaryocytes hyperlobulated
post-polycythemic phase - cytopenias and tear drop cells; BM has prominent reticulin and collagen fibrosis; decreased RBC and granulopoiesis
152
What is the major goal in polycythemia vera tx?
reduce risk of thrombosis
153
What disease is this?
CML
154
What disease is this?
polycythemia vera
155
sustained thrombocytosis \>450k and overproduction of megakaryocytes could indicate
are there any mutations a/w?
essential thrombocytosis
JAK2 in about half of the cases
NO genetic abnls associated
156
What is the clinical presentation of essential thrombocytosis?
abnl CBC
vascular disturbances either thrombosis or hemorrhage, arterial or venous
157
Periph blood in ET has no...
significant L shift
158
BM in ET is...
normal to slightly hypercellular
striking megakaryocytic production
absent or minimal reticulin fibrosis
159
This is peripheral blood in what?
essential thrombocytosis
160
This is bone marrow in what?
essential thrombocytosis
161
an indolent disease of adults \>60 yo, it has nonspecific ssx, and proliferation of mainly megakaryocytes and granulocytes
primary myelofibrosis
162
When does primary myelofibrosis present and what are some of its features?
presents at a late stage with anemia and massive splenomegaly
has leukoerythroblastosis and teardrop cells in periph blood
late stages also have BM fibrosis and extrameduallary hematopoiesis
\*starts out with many of everything and then goes to fibrotic stage and has hypoplasia
163
What is dyspoiesis?
dysplasia of blood cells
164
What is this?
dyserythropoiesis
165
What is this? What does it usually have in regards to nuclei
dysmegakaryopoiesis
odd number of nuclei
166
Chronic myelomonocytic leukemia does not have...
Ph chromsome or BCR/ABL1 fusion gene
167
CMML presents in who... what does it look like?
older males
risk factors include toxins, radiation, previous chemo
present with blood monocytosis for at least 6 months
tissue based leukemic infiltrates may occur
splenomegaly and hepatomegaly common
168
Myelodysplastic neoplasms generally have..
bone marrow hyperceullarity
ineffective hematopoiesis
not often, organomegaly
169
MDS/MPN has no known...
cytogenetic or molecular genetic abnormalities
170
What do we see here?
CMML
2 segments of nuclei, coarse chromatin, hypogranular,
typically see increased monocytes on the right side
171
What is this?
hypercellular bone marrow in CML
172
general characteristics of myelodysplastic neoplasms
cytopenias
dyspoiesis
hypercellular marrow
normal or increased marrow blasts, \<20%
organomegaly is not often
173
symptoms of myelodysplastic neoplasms are....
related to cytopenias
most pts are anemic and transfusion dependent
174
Blood: cytopenia in at least one cell type
unilineage dysplasia
no or rare blasts
BM: unilineage dysplasia
\<5% blasts
\<15% sideroblasts
Refractory cytopenia with unilineage dysplasia
175
blood: anemia, dimorphic pattern, no blasts
BM: \>15% of erythroid precursors are ringed sideroblasts, dysplasia in erythroid lineage only
\<5% blast
refractory anemia with ring sideroblasts
176
What am I looking at?
ringed sideroblasts with increased iron
177
blood: cytopenia, multilineage dysplasia, no blasts, no auer rods, no monocytosis
BM: dysplasia in 2+ myeloid lineages, \<5% blasts, no auer rods, w/o sideroblasts
refractory cytopenia with multilineage dysplasia
178
What am I looking at?
refractory cytopenia with multilineage dysplasia
179
elderly women, anemia, thrombocytosis (mild); in the blood we see macrocytic or normocytic anemia, nl or increased platelet count, no or rare blasts, no auer rods
BM shows nl or increased megakaryocytes with hypolobulated nuclei, few blasts, no auer rods
MDS with isolated del(5q)
180
Differentiate Refractory anemia with excess blasts 1 and 2
RAEB 1 has \<9% blasts in bm
RAEB 2 has 10-19% blasts in bm
181
What are auer rods?
primary granules that have coalesced within myeloblasts, not in normal cells
182
general characteristics of AML
malignant proliferation of immature myeloid cells
distinct peroxidase positive auer rods
183
What is the MC type of leukemia in adults and neonates?
AML
184
blasts in AML
comprise at least 20% blasts in blood or bone marrow
185
AML specific ssx
gingival hyperplasia (with AML of monocytic lineage)
DIC with APL
organomegaly is uncommon
186
When is the blast count of 20% not required for diagnosis of AML?
AML with recurrent genetic abnormalities
187
AML t(8:21)
MC type of childhood AML
good response to chemo
ETO
188
AML with inv (16)
CBFB-MYH11 fusion gene
abnormal eosinophil component
immature eosinophilic granules, abnormally large, purp, numerous
AML M4-eo
better response to chemo in younger pts
189
Acute promyelocytic leukemia t(15:17)
PML-RARalpha
pts commonly develop DIC, so rapid dx is essential
hematologic emergency
190
What is this?
two variants of APL
191
This is APL under which stain, super duper positive, obvi
myeloperoxidase stain
192
acute monoblastic leukemia extramedullary lesions include
gingival hyperplasia
leukemia cutis
193
What is the hallmark of SLE?
production of array of autoantibodies, esp ANAs.
194
What are the 11 things that can characterize SLE? Need 4 /11
serositis
oral ulcers
arthritis
photosensitivity
blood disorders
renal involvement
ANA
immunologic phenomenon
neurologic disorder
malar rash
discoid rash
195
What two abs are largely diagnostic of SLE?
anti smith
anti ds dna
196
How is tissue injured in SLE?
deposition of immune complexes and binding of antibodies to various cell and tissues, esp kidneys
197
What is the genetic predisposition for rheumatoid arthritis?
HLA-DR4
DR1
DR10
DR14
198
What is an ab associated with RA?
anti-CCP ab
199
synovial hyperplasia occurs in what autoimmune disease?
rheumatoid arthritis
200
What abs are associated with Sjogrens syndrome? What does it appear on IF?
SS-A Ro
SS-B La
speckled appearance on IF studies
201
What gives a 40x increase in developing MALT lymphoma?
Sjogrens syndrome
202
What abs are associated with scleroderma?
DNA topoisomerase I (diffuse systemic)
anticentromere ab (limited systemic sclerosis)
203
CREST syndrome is a/w what autoimmune disease?
scleroderma
204
High antibody titers to U1 ribonucleoprotein
Mixed CT disease
