Diseases of White blood cells Flashcards
(161 cards)
1
Q
what percentage of the bone marrow must be blasts in order for the diagnosis of AML to be made
A
The diagnosis of AML is based on the presence of at least 20% myeloid blasts in the bone marrow (not required to be in the peripheral blood?)
2
Q
what is a leukmoid reaction
A
it is a response of healthy bone marrow to increased stress, infection but it is NOT a malignancy
3
Q
what is leukocytosis
A
increase in neutrophils incease in eosinophils increase in monocytes increased lymphocytes increased basophils
4
Q
tdt
A
terminal deoxytransferase
seen in acute lymphoblastic leukemia (ALL)
5
Q
CD56+
A
NK Cells
6
Q
AMML
A
MPO
NSE positive
degranulate and mess up your skin
7
Q
APL
A
acute promyelocytic leukemia
DIC
in middle age
Auer rods
t(15:17)
treat with all trans retinoic acid
8
Q
yellow eyes
fatigue
after stressful situation
A
Gilbert’s disease
indirect will be elevated–> enzyme that conjugates the bili is decreased functioning (UDPGT)
TB < or = to 3.0
teen adult
9
Q
Crigler najjar I
A
auto recessive
absence of UDPGT
worry about kernicterus
severely increased unconjugated bili
10
Q
Dubin-johnson
A
increased conjugated bili
black liver
AR inheritence
11
Q
Rotor syndrome
A
increased conjugated bili
AR inheritence
problems with transfer enzymes –> getting out is a problem
12
Q
haptoglobin is increased or decreased in hemolytic disease?
A
low
13
Q
clay colored stools
A
posthepatic
increased direct and indirect
14
Q
where does hematopoiesis take place at 12 weeks gestation
A
in the liver
15
Q
give hydroxy urea to what kinds of patients
A
sickle cell
b/c they have HbS –> kicks them back to having HbF (more likely to put O2 into tissues)
16
Q
name the cells of the myeloid lineage
A
basophils neutrophils Eo's monocytes platelets erythrocytes
17
Q
name the cells of the lymphoid lineage
A
NK cells (large granular lymphocyte)
B cells–> plasma cells
T cells
18
Q
Cd3 and CD4, CD8
A
T cells
19
Q
CD20, CD19
A
B cells
20
Q
tdt
A
premature lymphocyte
21
Q
CD56
A
NK Cell
22
Q
bleeding in pt?
A
go down myeloid lineage! b/c of platelets
23
Q
monocytes in the skin
A
itching
hives
pustules
necrotic tissue
24
Q
what causes 5th’s disease and how does it present in a sickle cell patient
A
aplastic crisis in sickle cell patient due to parvovirus B19
infeffective granulopoiesis related to suppression of hematopoietic stem cells
25
what are the granulocytes
basophils, neutrophils, eosinophils, macrophages
come from the myeloblasts
26
hematocrit
relative measure of red cell mass
27
anemia of chronic inflammation
decreased Fe
decreased TIBC
increased ferritin
hepcidin would be increased --> acute phase reactant
28
increase green leafy vegetable intake to increase
folate
29
megaloblastic anemia with hypersegmented neutrophils
Vitamin B12 deficiency/folate deficiency
you need this to help with DNA production
30
what causes beta thalassemia
excess in alpha due to point mutation
target cells in the periphery
31
Howell-Jolly bodies
NO SPLEEN
often seen in sickle cell anemia
32
```
54 y o male
abd pain, diarrhea
altered mental status
temp 101.6
WBC 35,000 high
Hb 13
HCT 36%
Plt 460,000
bands 32% (immature neutrophils)
segmented neturophils 24%
lymphocytes 15%
```
after IV antibiotics --> WBC's go up 56,000
Leukemoid rxn
-a reactive increase in leukocytes in the setting of severe infection/inflammation
LAP - elevated
LAP that is NOT elevated/low is CML
33
EBV
lymphocytosis- atypical
convoluted nuclei
highly vacuolated cytoplasm
non neoplasmic
34
decreased margination
myelodysplastic syndrome
white blood cells can de-marginate
- steroids
- exercise
35
effaced lymph node
DLBCL
36
t(14;18)
follicular lymphoma
associated with BCL2
37
t(8;14) EBV
endemic Burkitt's
38
HTLV-1
Adult T cell leukemia (ATLL)
japan
caribbean
CD4 T cells
this virus also have reverse transcriptase
39
HHV-8
Diffuse large B cell lymphoma
40
urea breath test is associated with what?
H. pylori
Marginal zone lymphoma
41
t(11;14)
mantle cell lymphoma
older males
high cyclin D1
CD5 +
lymphomatoid polyposis
42
```
CD11c,
CD19,
CD20
CD25
CD103
```
BRAF
dry tap
atypical mycobacterium infection
Hairy cell leukemia
43
t(15;17)
presents with DIC
M3
auer rods
acute promyelocytic leukemia (form of acute myeloid leukemia)
RAR-PML
treat with all trans retinoic acid -> binds to the mutation receptor inducing maturation of the promyelocytes to myelocytes
44
nonspecific esterase positive
having to do with AML - that has positive for monocytes
45
myeloperoxidase positive
having to do with AML - that has positivity for myelocytes
46
basophils are characteristically increased in what
CML
47
CD34+
hematopoietic stem cells
48
major cause of neutropenia
chemotherapy or other drug toxicity is the major cause - decreased production of neutrophils
chemo works by preventing cell division
49
high cortisol state
can cause apoptosis of lymphocytes
lymphopenia
neutrophilic leukocytosis
50
most sensitive cell to radiation
lymphocytes
51
monocytosis
chronic inflammatory state
-autoimmune or infectious
malignancy
52
IL-5
eosinophils
53
eosinophils are associated with what cancer
hodgkins lymphoma
b/c of RS cells ability to attract them (IL-5)
54
tdt
DNA polymerase- in nucleus of lymphoblasts
only seen in ALL
lymphoblasts only
55
myeloblasts have what positivity
myeloperoxidase
56
t(12;21)
better prognosis for B-All
57
NOTCH 1
T-ALL
58
better prognosis factor
t(12;21)
2-10 years old
low WBC count
trisomy 4, 7 , 10
hyperdiploidy
59
auer rods
AML
too many can lead to DIC
60
DIC is associated with what AML
APL
intermediate prognosis
t(15;17)
61
peak age for AML
60
62
t(15;17)
APL
M3 stage
forms abnormal retinoic acid receptor
63
M2
t(8;21)
auer rods present in most cases
AML with myelocytic maturation
64
M3
t(15;17)
high incidence of DIC
many auer rods
MPO positive
APL
65
BRAF mutation
```
CD19
CD20
Surface Ig
CD11c
CD25
CD103
```
Hairy cell leukemia
66
t(8;14) - C-MYC
Burkitt's lymphoma
endemic --> BCL2 negative, EBV, mandible
sporadic--> iliocecal mass
67
t(14;18) -BCL2 positive
follicular lymphoma
68
t(11;14) cyclin D1
Mantle cell
69
t(11;18)(14;18)(1;14)
Marginal zone lymphoma
(chronic inflammation)
all of these translocations upregulate the expression and function of BCL10 or MALT1 which promote the survival and growth of B cells
70
t (9;22) BCR-ABL
CML
bad prognosis in pt's with B-ALL
71
t(12;21)
B-ALL
72
t(8;21)
AML (M2)
73
BCL6
DLBCL
74
Trisomy 12
Deletions of 11q,13q, 17p
CD19
CD20
CD23
CD5
CLL/SLL
75
Monosomies 5 and 7
Deltetions of 5q, 7q, 20 q
Trisomy 8
myelodysplastic syndrome
76
MYD88
Lymphoplasmacytic lymphoma
77
3 y.o. hispanic male
bone marrow precursor B cell
peak age 3
mostly in children
t(12;21)
Tdt
CD19
CD10 (negative for very immature forms, positive for late forms)
abrupt onset
fatigue, anemia, fever, bleeding
t(9;22) is worse prognosis
CNS involvement --> headache, nerve palsies
pancytopenia
B-cell ALL
78
what are the criteria for a worse prognosis for T-ALL or B-ALL
• Age 100,000
79
adolescent male
thymic mass
splenomegaly
lymphadenopathy
tdt
NOTCH1
CD1, 2, 5, 7
late--> CD3, CD4, CD8
mediastinal mass
anemia, neutropenia, thrombocytopenia
bone pain
enlarged testes
CNS → headaches, vomiting, nerve palsies from meningeal spread
treat with chemo
T-ALL
80
Naive B cell or memory cell
median age 60
2:1 M:F
diffuse effacement nodal architecture
smudge cells
CD19,20,23,5
surface IgM or IgM and IgD
most common leukemia of adults in the western world
asymptomatic usually at diagnosis (fatigue, weight loss, anorexia)
lymphadenopathy
increased susceptibility to infections
hepatosplenomegaly
Median survival is 4-6 yrs.
Transform (Richter syndrome) with survival less than 1 year
Chronic lymphocytic leukemia/Small lymphocytic lymphoma
81
B cell
older adults 60-70s
headaches, dizziness, visual impairment, deafness
symptoms like Raynauds
LAD, splenomegaly, hepatomegaly
Anemia
Waldenstrom macroglobulinemia --> plasma cells secreting monclonal IgM
Russell bodies, dutcher bodies
lymph nodes, spleen and liver dissemination
bone destruction is NOT common, nor is secretion of Ig light chains
lymphoplasmacytic lymphoma
10%--> autoimmune hemolysis due to cold agglutinins
82
what is the prognosis of lymphoplasmacytic lymphoma
incurable
Progressive disease, doesn’t respond well to chemo- median survival of 4 years
Symptoms caused by high IgM can be alleviated by plasmapheresis
83
```
male
50-60's
naive B cell
t(11;14)
cyclin D1
```
CD19
CD20
Surface Ig (usually IgM and IgD)
CD5+
CD23 negative
generalized painless lymphadenopathy
lymphomatoid polyposis (polyp like lesion in GI)
poor prognosis (3-4 yrs)
Mantle cell lymphoma
84
what re the 3 indolent tumors
Marginal
Hairy cell
follicular lymphoma (most common)
85
mature B cell neoplasm - germinal centers devoid of apoptotic cells
older adults
M=F
most common form on indolent NHL
lymphocytosis
painless waxing and waning LAD
GI, testes can be involved
Incurable - survival 7=9 yrs
paratrabecular lympohid aggregates
often involves splenic white pulp and portal triads
follicular lymphoma
86
what histo transformation can take place in follicular lymphoma
large B cell lymphoma or similar to Burkitt
median survival of less than 1 year
87
what are the markers for follicular lymphoma
BCL2- apoptotic inhibitor
t(14;18)
CD19, 20, 10
BCL6
No CD5
88
memory B cell origin
adults
arise in lymph node, spleen, extranodal tissues
arises within tissues involved by chronic inflammatory disorders of autoimmune or infectious etiology;
- Salivary glands
- Stomach (H-pylori)
- Thyroid (hashimoto)
-t(11;18)
-t(14, 18)
-t(1,14)
all of these t’s upregulate the expression and function of BCL10 or MALT1
Marginal zone lymphoma
89
what is the prognosis of marginal zone lymphoma
transform to diffuse large B cell lymphoma can occur
indolent
regress possibly if the inciting agent is removed
90
middle aged white male (median age 55)
memory B cell
infiltrate bone marrow, liver, spleen
massive splenomegaly
hepatomegaly
dry tap
pancytopenia
atypical mycobacterial infections - kansaii
excellent prognosis and sensitive to chemo
hair cell leukemia
91
what are the markers for hairy cell leukemia
BRAF
```
CD19
CD20
Surface Ig (usually IgG)
CD11c
CD25
CD103
Annexin A1
```
92
most common form of NHL
mature B cell neoplasm
M>F, older adults
BM involvement is NOT common
Dysregulation of BCL6 (30%) or BCL2 (10%)
C-MYC- 5%
presents as a rapidly growing mass, commonly involving waldeyers ring
large destructive masses of liver and spleen
can involve brain, GI, skin, bone
rapidly fatal without treatment
Diffuse large B cell lymphoma
MYC gives it a worse prognosis
93
Immunodeficiency associated large B cell lymphoma
subtype of DLBCL
T cell deficiency
Neoplastic B cells are usually infected with EBV
94
Primary effusion lymphoma
subtype of DLBCL
malignant pleural or ascetic effusion.
Seen in the setting of HIV.
Anaplastic cells that fail to express B or T cell markers, have clonal IgH rearrangements
Infected with KSHV/HHV-8
95
children or young adults
starry sky pattern, royal blue cytoplasm with clear cytoplasmic vacuoles
manifest at extranodal sites (mandible, GI tract)
fastest growing human tumor due to Warburg effect
C-MYC translocation
t(8;14)
```
Immunophenotype:
CD19
CD20
CD10
BCL6
IgM
```
**Fail to express the antiapoptotic protein BCL2
very aggressive, treat with chemo and it responds well
Burkitt lymphoma
96
Endemic Burkitts lymphoma (African)
children young adult
all associated with EBV
mass involving the mandible
abdominal viscera can also be involved (kidneys, ovaries, adrenal glands)
97
Sporadic (nonendemic) Burkitts lymphoma
-mass involving the ileocecum and peritoneum
98
what does the overexpression of MYC in burkitt's cause
increases expression of genes required for aerobic glycolysis (Warburg effect) → allows cells to synthesize nucleotides, lipids, proteins needed for growth and cell division
99
Adults
neutropenia and anemia
lymphocytosis
splenomegaly
No LAD or hepatomegaly
Felty syndrome--> 1) RA 2) splenomegaly 3) Neutropenia
Point mutations in STAT3
T cell variants:
CD3+
NK-cell variant:
CD3-
CD56+
blue cytoplasm
Large granular lymphocytic leukemia
100
what is the prognosis of large granular lymphocytic leukemia
variable course
Depends on severity of cytopenias and response to low dose chemo or steroids
Indolent course- T cells
NK cells are more aggressive
101
CD4 T cells
3 phases:
inflammatory premycotic phase
-a plaque phase
-tumor phase
CLA
CCR4
CCR10
homes to skin
late can spread to lymph nodes, bone marrow
indolent
mean survival 8-9 yrs
Mycosis fungoides/Sezary syndrome
102
what is sezary syndrome
- generalized exfoliative erythroderma
| - characteristic cerebriform nuclei
103
helper or cytotoxic T cells
mainly older adults
CD2,3 ,5
LAD
eosinophilia, pruitis, fever, weight loss
aggressive
Peripheral T cell lymphoma unspecified
104
cytotoxic T cell
children or young adults
Hallmark cells- horse shoe shaped nuclei and voluminous cytoplasm
cluster about venules and infiltrate lymphoid tissues
ALK gene rearrangements
CD30 +
presents as soft tissue masses in children
good prognosis
Morphologically similar lymphomas (but without ALK rearrangements) occur in adults and have a poor prognosis
Anaplastic large cell lymphoma
105
CD4 T cells
adults infected with HTLV-1- and present in all tumor cells
japan
multilobated nuclei
"cloverleaf or flower cells"
Japan, west africa, caribbean
Generalized LAD, hepatosplenomegaly
hypercalcemia
HTLV-1 can also cause CNS disease
aggressive
Adult T cell leukemia/lymphoma
106
STAT3
Large granular lymphocytic leukemia
107
Destructive extranodal masses – most commonly sinonasal, less common in testes or skin
Surrounds and invades small vessels leading to extensive ischemic necrosis
aggressive
Adults
Asia
EBV assoicated
CD21 negative
CD3 negative
Extranodal NK/T cell lymphoma
108
Reed sternberg cells
Hodgkin lymphoma
these cells release factors that induce accumulation of reactive lymphocytes, macrophages, and granulocytes
109
what characterizes hodgkin lymphoma versus non hodgkin lymphoma
localized single group of nodes
depressed TH1 response
extranodal involvment is rare
contigous spread
prognosis is much better than NHL
RS cells
bimodal distribution
young adult to >55
Spread of HL is stereotyped:
1) nodal
2) spleen
3) hepatic
4) marrow and other tissues
Tumor stage (rather than histologic type) is the most important prognostic variable
110
most common form of HL
EBV negative
CD15, CD30
Mediastinal involvement frequent
m=f
young adults
lacunar variant
deposition of collagen bands that divide the involved lymph nodes into circumscribed nodules
Bacground infiltrate of T lymphocytes, eo’s, macrophages, plasma cells
Nodular sclerosis HL
111
what is the prognosis of Nodular sclerosis HL
excellent prognosis
usually stage I or II
112
M > F
Peaks in young adults and then again in > 55 range
Frequent mononuclear and diagnostic RS cells
Bacground infiltrate of T lymphocytes, eo’s, macrophages, plasma cells
CD15, CD30
night sweats, weight loss
infected with EBV about 70% of time
mixed cellularity HL
113
what is the prognosis of mixed cellularity HL
advanced tumor stage usually III or IV
114
M>F
tends to be seen in older adults
Reactive lymphocytes (T cells) make up the vast majority of the cellular infiltrate
Lymph nodes are usually effaced
CD15
CD30
EBV assoicated in about 40%
Very good to excellent prognosis
Lymphocyte rich HL
115
least common HL
M>F
older adults
Reticular variant
Frequent diagnostic RS cells and variants and a paucity of background reactive cells
90% are EBV associated
CD15, CD30
More common in HIV individuals
often presents with advanced disease
less favorable than other subtypes
lymphocyte depletion
116
```
CD20+
CD15-
CD30-
EBV-
BCL6+
```
popcorn cell (lymphocytic histiocytic cell)
young males
lymphocyte predominance HL
uncommon
non classical type
L&H variants express Bcell markers typical of germinal center B cells (CD20, BCL6) and show evidence of ongoing somatic hypermutation –
117
what can happen in a small percentage of Lymphocyte predominate HL
In small percent this transforms into a tumor resembling diffuse large B cell lymphoma
118
Adult black male
age 65-70
lytic bone lesions
rouleaux formation
M-spike
plasma cell neoplasm
CD138
CD56
hypercalcemia
renal failure due to bence jones proteins
recurrent bacterial infections
survival of 4-7 yrs
Multiple myeloma
119
what Ig and light chains are involved with Multiple myeloma
M spike on serum protein electrophoresis
Increased levels of Igs in the blood and/or light chains (Bence jones proteins- mostly kappa light chain) in the urine → mostly IgG** followed by IgA
NOTE→
-more than 3 gm/dL of serum Ig and/or more than 6 gm/dL of urine Bence jones proteins
Excessive production of M proteins (of IgA or IgG subtype) leads to hyperviscosity
120
solitary MM
single mass in bone or soft tissue
| -Extraosseous lesions can occur in lungs, oronasopharynx, nasal sinuses
121
smoldering MM
- lack of symptoms and a high plasma M component
- serum M protein level >3gm/dL
- 75% progress to MM over 15 year period
122
what is waldenstrom macroglobulinemia
High levels of IgM lead to symptoms related to hyperviscosity of the blood
Occurs in association with lymphoplasmacytic lymphoma
123
** most common plasma cell dyscrasia
older adults
>50 3%
>70 5%
No signs or symptoms
Small to moderately large M components in their blood – level
Monoclonal gammopathy of undetermined significance (MGUS)
124
incidence peaks after age 60
Complications of marrow failure:
- fatigue (anemia)
- fever/infection (neutropenia)
- spontaneous mucosal and cutaneous bleeding (thrombocytopenia)
infections frequent→ oral cavity, skin, lungs, kidney, bladder, colon (pseudomonas, fungi, commensals)
often involves soft tissue around the mouth
Leukemic cutis – frequently moncytic features
Acute myleoid leukemias
-accumulation of immature myeloid forms (Blasts) in the bone marrow suppresses normal hematopoiesis
based on the presence of at least 20% myeloid blasts in the bone marrow
125
what is the prognosis of AML
difficult to treat
60% achieve complete remission with chemo but only 15-30% remain free of disease for 5 years
126
t(8;21)
prognosis?
subytpe?
AML with myelocytic maturation
favorable prognosis
MPO +
M2
127
inv (16)
prognosis?
AMML- acute myelomonocytic leukemia
M4
favorable prognosis
MPO positive
esterase positive
128
t(15;17)
prognosis?
intermediate prognosis
treat with all trans retinoic acid and arsenic salts
M3
DIC
APL** diagnosis
129
prognosis of therapy related AML
very poor
along with prior myelodysplastic syndrome
130
mean age of 70
clonal stem cell disorder
Monosomies 5 and 7
Deltetions of 5q, 7q, 20 q
Trisomy 8
Most characteristic finding is disordered differentiation affecting the erythroid, granulocytic, monocytic, and megakaryocytic lineage
presents with cyopenias--> weakness, infections, hemorrhages
Myelodysplastic syndromes
defective maturation of myeloid progenitors gives rise to ineffective hematopoiesis leading to cytopenias
131
what are myeloproliferative disorders
usually increased production of one or more types of blood cells (usually one or more mature blood elements- particularly granulocytes and platelets)
Presence of mutated, constitutively activated tyrosine kinases or other acquired aberrations in signaling pathways that lead to growth factor independence
often transform to AML
132
peak incidence b/w 50-60s
BCR-ABL
t(9;22)
splenomegaly (LUQ pain, dragging)
hepatosplenomegaly
anemia, fatigue, weaknesss
labs--> leukocytosis exceeding 100,000
insidious onset
LAD due to extramedullary hematopoiesis
low LAP
hypercellular bone marrow
CML
Morphology:
Hypercellular bone marrow b/c of massive amounts of maturing granulocytic precursors – eo’s and basophils, megakaryo’s, erythroid precursors
***basophils are signature
133
what is the prognosis of CML
Median survival is about 3 years → can go into accelerated phase after this time → ends in presentation similar to acute leukemia (blast crisis)
134
how do you treat CML
BCR ABL inhibitors
younger pt's - hematopoietic stem cell transplant
135
middle age adult
JAK2 mutation
incrased platelets, baso's in peripheral blood, increased eo's
increased erythroid precursors causing increased HCT, blood viscosity and decreased EPO
pt's can be plethoric and cyanotic due to stagnation of blood in peripheral vessels
headache, dizziness, GI symptoms, intense pruitis
peptic ulceration
hyperuricemia
organomegaly
prone to thrombosis and bleeding
can present looking like MI, DVT , or stroke
polycythemia vera
136
what is the spent phase of polycythemia vera
progresses to spent phase, with extensive marrow fibrosis that displaces hematopoietic cells → leads to extramedullarhematopoiesis in the liver and spleen
137
what is the prognosis of polycythemia vera
2% transform to AML
without treatment, fatal with bleeding and thrombosis
Treatment→ maintain the red cell mass at normal levels by phlebotomy- extends survival to about 10 years
138
past age of 60
abnormally large platelets on smear
```
JAK2 (50%)
or MPL (5-10%)
```
organomegaly
thrombosis and hemorrhage - presents with DVT, MI, stroke
clustered megakaryocytes in BM
Erythromelalgia (throbbing and burning of hands and feet caused by occlusion of small arterioles by platelet aggregates)
Essential thrombocytosis
139
what is the prognosis of essential thrombocytosis
Indolent
Long asymptomatic periods with some thrombotic or hemorrhagic crises
Median survival is 12-15 years
Worse prognosis:
- very high platelet count
- homozygous JAK2 mutations
Treatment→ gentle chemo that suppresses thrombopoiesis
140
Early on→ marrow is hypercellular
Hallmark is the development of obliterative marrow fibrosis → deposition of collagen in the marrow by non-neoplastic fibroblasts
Megakaryocyt→ release TGF-b and PDGF
Extensive extramedullary hematopoiesis
Teardrop shaped red cells
individuals older than 60
with splenomegaly and spleen infarcts subcapsular
fatigue, weight loss, night sweats
normochromic, normocytic anemia
white cell count elevated early on and thrombocytopenia later on
extensive extramedullary hematopoiesis (spleen, liver, lymph nodes)
primary myelofibrosis
141
what is the prognosis and treatment of primary myelofibrosis
Difficult disease to treat
Median survival of 3-5 years
Threats to life
- infections
- thrombotic events
- bleeding
- transformation to AML
Treat:
JAK2 inhibitors
-hematopoietic stem cell transplant (young pt’s)
142
what are the tumor cell markers for langerhans cell histiocytosis
HLA-DR
S-100
CD1a
143
what cell type is characteristic of langerhans cell histiocytosis
birbeck granules - tennis racket
144
pt younger than 2
Cutaneous lesions resembling seborrheic eruption on the front and back of trunk and on scalp
Hepatosplenomegaly
Lymphadenopathy
Pulmonary lesions
Bone lesions
Fever/Infections (anemia and thrombocytopenia) → Chronic otitis media, mastoiditis
rapidly fatal
Multifocal/multisystem LCH: (Letterer-Siwe)
with intensive chemo - 50% 5 year survival
145
Eo's, lymphocytes, plasma cells, and neuts
arises within medullary cavities of bones (calvarium, ribs, femur)
older children, rarely adults
-skeletal system in older children or rarely adults
- ocassionally skin, lung or stomach
- most commonly the calvarium, ribs and femur
Unifocal LCH (eosinophilic granuloma)
unifocal - older children, rarely adults
multifocal - young children
146
Eo's, lymphocytes, plasma cells, and neuts
arises within medullary cavities of bones (calvarium, ribs, femur)
multiple erosive bony masses YOUNG children
-50% have diabetes insipidus (b/c of involvement of the posterior pituitary stalk of hypothalamus)
**Hand-Schuller-Christian triad
Multifocal unisystem LCH
147
what is the Hand Schuller christian triad
1) Calvarial bone defects
2) Diabetes insipidus
3) Exophthalmos
seen in Multifocal unisystem LCH
148
seen in adult smoker
Could be inflammatory/reactive process but many cases are now considered to be neoplastic if a pt case exhibits BRAF mutation
BRAF mutation is seen in 40%
bilateral interstitial disease of lungs
Multiple fine nodules and cysts in the middle and upper lung zones
Strongly associated with cigarette smoking
pulmonary LCH
May regress spontaneously on cessation of smoking
149
throbbing, burning pain in the extremities
essential thrombocythemia
caused by platelet aggregates that occlude small arterioles
150
ringed sideroblasts
megaloblasts
abnormal megakaryocytes
myeloblasts
myelodysplasia
151
t (8;14)
EBV associated african endemic Burkitts
152
CD5+
t (11;14)
cyclin D1
lymphomatoid polyposis
mantle cell
153
what is serum
blood that has been allowed to clot
so it is plasma with diminshed fibrinogen and other clotting factos
154
what is plasma
To obtain plasma from withdrawn blood, add anticoagulant to prevent clotting
9 % molecules [proteins, organic
and inorganic]
91 % Water
155
normal range for WBC's
4800 - 10800
156
granulocytes make up what percentage of peripheral blood WBC's
40-70%
157
normal range of platelets
150-450 (x10^3)
158
CD5+
t(11;14)
mantle cell lymphoma
159
monocytosis can be seen in the setting of what
chronic infections (TB), bacterial endocarditis, rickettsiosis, malaria. Autoimmune disorders (SLE), inflammatory bowel disease (ulcerative colitis)
160
skin lesions
clover leaf cells
demyelinating disease of the CNS and spinal cord
Adult T cell leukemia/lymphoma
HTLV-1 associated
161
tear drop shaped red blood cells
myelofibrosis
