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Heme/Lymph Exam I > Diseases of White blood cells > Flashcards

Diseases of White blood cells Flashcards

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?)

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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

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3
Q

what is leukocytosis

A 
increase in neutrophils
incease in eosinophils
increase in monocytes
increased lymphocytes 
increased basophils
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4
Q

tdt

A

terminal deoxytransferase

seen in acute lymphoblastic leukemia (ALL)

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5
Q

CD56+

A

NK Cells

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6
Q

AMML

A

MPO
NSE positive

degranulate and mess up your skin

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7
Q

APL

A

acute promyelocytic leukemia

DIC
in middle age
Auer rods

t(15:17)

treat with all trans retinoic acid

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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

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9
Q

Crigler najjar I

A

auto recessive
absence of UDPGT

worry about kernicterus

severely increased unconjugated bili

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10
Q

Dubin-johnson

A

increased conjugated bili

black liver

AR inheritence

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11
Q

Rotor syndrome

A

increased conjugated bili

AR inheritence

problems with transfer enzymes –> getting out is a problem

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12
Q

haptoglobin is increased or decreased in hemolytic disease?

A

low

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13
Q

clay colored stools

A

posthepatic

increased direct and indirect

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14
Q

where does hematopoiesis take place at 12 weeks gestation

A

in the liver

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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)

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16
Q

name the cells of the myeloid lineage

A 
basophils
neutrophils
Eo's
monocytes
platelets
erythrocytes
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17
Q

name the cells of the lymphoid lineage

A

NK cells (large granular lymphocyte)

B cells–> plasma cells
T cells

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18
Q

Cd3 and CD4, CD8

A

T cells

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19
Q

CD20, CD19

A

B cells

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20
Q

tdt

A

premature lymphocyte

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21
Q

CD56

A

NK Cell

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22
Q

bleeding in pt?

A

go down myeloid lineage! b/c of platelets

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23
Q

monocytes in the skin

A

itching
hives
pustules
necrotic tissue

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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

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25
Q

what are the granulocytes

A

basophils, neutrophils, eosinophils, macrophages

come from the myeloblasts

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26
Q

hematocrit

A

relative measure of red cell mass

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27
Q

anemia of chronic inflammation

A

decreased Fe
decreased TIBC
increased ferritin

hepcidin would be increased –> acute phase reactant

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28
Q

increase green leafy vegetable intake to increase

A

folate

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29
Q

megaloblastic anemia with hypersegmented neutrophils

A

Vitamin B12 deficiency/folate deficiency

you need this to help with DNA production

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30
Q

what causes beta thalassemia

A

excess in alpha due to point mutation

target cells in the periphery

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31
Q

Howell-Jolly bodies

A

NO SPLEEN

often seen in sickle cell anemia

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32
Q 
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

A

Leukemoid rxn
-a reactive increase in leukocytes in the setting of severe infection/inflammation

LAP - elevated

LAP that is NOT elevated/low is CML

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33
Q

EBV

A

lymphocytosis- atypical

convoluted nuclei
highly vacuolated cytoplasm

non neoplasmic

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34
Q

decreased margination

A

myelodysplastic syndrome

white blood cells can de-marginate

  • steroids
  • exercise
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35
Q

effaced lymph node

A

DLBCL

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36
Q

t(14;18)

A

follicular lymphoma

associated with BCL2

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37
Q

t(8;14) EBV

A

endemic Burkitt’s

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38
Q

HTLV-1

A

Adult T cell leukemia (ATLL)

japan
caribbean

CD4 T cells

this virus also have reverse transcriptase

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39
Q

HHV-8

A

Diffuse large B cell lymphoma

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40
Q

urea breath test is associated with what?

A

H. pylori

Marginal zone lymphoma

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41
Q

t(11;14)

A

mantle cell lymphoma

older males
high cyclin D1
CD5 +

lymphomatoid polyposis

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42
Q 
CD11c, 
CD19, 
CD20
CD25
CD103

BRAF

dry tap

atypical mycobacterium infection

A

Hairy cell leukemia

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43
Q

t(15;17)

presents with DIC

M3

auer rods

A

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

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44
Q

nonspecific esterase positive

A

having to do with AML - that has positive for monocytes

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45
Q

myeloperoxidase positive

A

having to do with AML - that has positivity for myelocytes

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46
Q

basophils are characteristically increased in what

A

CML

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47
Q

CD34+

A

hematopoietic stem cells

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48
Q

major cause of neutropenia

A

chemotherapy or other drug toxicity is the major cause - decreased production of neutrophils

chemo works by preventing cell division

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49
Q

high cortisol state

A

can cause apoptosis of lymphocytes

lymphopenia

neutrophilic leukocytosis

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50
Q

most sensitive cell to radiation

A

lymphocytes

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51
Q

monocytosis

A

chronic inflammatory state
-autoimmune or infectious

malignancy

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52
Q

IL-5

A

eosinophils

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53
Q

eosinophils are associated with what cancer

A

hodgkins lymphoma

b/c of RS cells ability to attract them (IL-5)

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54
Q

tdt

A

DNA polymerase- in nucleus of lymphoblasts

only seen in ALL

lymphoblasts only

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55
Q

myeloblasts have what positivity

A

myeloperoxidase

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56
Q

t(12;21)

A

better prognosis for B-All

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57
Q

NOTCH 1

A

T-ALL

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58
Q

better prognosis factor

A

t(12;21)

2-10 years old

low WBC count

trisomy 4, 7 , 10

hyperdiploidy

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59
Q

auer rods

A

AML

too many can lead to DIC

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60
Q

DIC is associated with what AML

A

APL
intermediate prognosis

t(15;17)

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61
Q

peak age for AML

A

60

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62
Q

t(15;17)

A

APL

M3 stage

forms abnormal retinoic acid receptor

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63
Q

M2
t(8;21)
auer rods present in most cases

A

AML with myelocytic maturation

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64
Q

M3
t(15;17)
high incidence of DIC
many auer rods

MPO positive

A

APL

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65
Q

BRAF mutation

CD19
CD20
Surface Ig
CD11c
CD25
CD103
A

Hairy cell leukemia

66
Q

t(8;14) - C-MYC

A

Burkitt’s lymphoma

endemic –> BCL2 negative, EBV, mandible

sporadic–> iliocecal mass

67
Q

t(14;18) -BCL2 positive

A

follicular lymphoma

68
Q

t(11;14) cyclin D1

A

Mantle cell

69
Q

t(11;18)(14;18)(1;14)

A

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
Q

t (9;22) BCR-ABL

A

CML

bad prognosis in pt’s with B-ALL

71
Q

t(12;21)

A

B-ALL

72
Q

t(8;21)

A

AML (M2)

73
Q

BCL6

A

DLBCL

74
Q

Trisomy 12

Deletions of 11q,13q, 17p

CD19
CD20
CD23
CD5

A

CLL/SLL

75
Q

Monosomies 5 and 7

Deltetions of 5q, 7q, 20 q

Trisomy 8

A

myelodysplastic syndrome

76
Q

MYD88

A

Lymphoplasmacytic lymphoma

77
Q

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

A

B-cell ALL

78
Q

what are the criteria for a worse prognosis for T-ALL or B-ALL

A

• Age 100,000

79
Q

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

A

T-ALL

80
Q

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

A

Chronic lymphocytic leukemia/Small lymphocytic lymphoma

81
Q

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

A

lymphoplasmacytic lymphoma

10%–> autoimmune hemolysis due to cold agglutinins

82
Q

what is the prognosis of lymphoplasmacytic lymphoma

A

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
Q 
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)

A

Mantle cell lymphoma

84
Q

what re the 3 indolent tumors

A

Marginal
Hairy cell
follicular lymphoma (most common)

85
Q

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

A

follicular lymphoma

86
Q

what histo transformation can take place in follicular lymphoma

A

large B cell lymphoma or similar to Burkitt

median survival of less than 1 year

87
Q

what are the markers for follicular lymphoma

A

BCL2- apoptotic inhibitor

t(14;18)

CD19, 20, 10

BCL6

No CD5

88
Q

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

A

Marginal zone lymphoma

89
Q

what is the prognosis of marginal zone lymphoma

A

transform to diffuse large B cell lymphoma can occur

indolent

regress possibly if the inciting agent is removed

90
Q

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

A

hair cell leukemia

91
Q

what are the markers for hairy cell leukemia

A

BRAF

CD19
CD20
Surface Ig (usually IgG) 
CD11c
CD25
CD103
Annexin A1
92
Q

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

A

Diffuse large B cell lymphoma

MYC gives it a worse prognosis

93
Q

Immunodeficiency associated large B cell lymphoma

A

subtype of DLBCL

T cell deficiency

Neoplastic B cells are usually infected with EBV

94
Q

Primary effusion lymphoma

A

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
Q

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

A

Burkitt lymphoma

96
Q

Endemic Burkitts lymphoma (African)

A

children young adult

all associated with EBV

mass involving the mandible

abdominal viscera can also be involved (kidneys, ovaries, adrenal glands)

97
Q

Sporadic (nonendemic) Burkitts lymphoma

A

-mass involving the ileocecum and peritoneum

98
Q

what does the overexpression of MYC in burkitt’s cause

A

increases expression of genes required for aerobic glycolysis (Warburg effect) → allows cells to synthesize nucleotides, lipids, proteins needed for growth and cell division

99
Q

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

A

Large granular lymphocytic leukemia

100
Q

what is the prognosis of large granular lymphocytic leukemia

A

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
Q

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

A

Mycosis fungoides/Sezary syndrome

102
Q

what is sezary syndrome

A
  • generalized exfoliative erythroderma

- characteristic cerebriform nuclei

103
Q

helper or cytotoxic T cells

mainly older adults

CD2,3 ,5

LAD

eosinophilia, pruitis, fever, weight loss

aggressive

A

Peripheral T cell lymphoma unspecified

104
Q

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

A

Anaplastic large cell lymphoma

105
Q

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

A

Adult T cell leukemia/lymphoma

106
Q

STAT3

A

Large granular lymphocytic leukemia

107
Q

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

A

Extranodal NK/T cell lymphoma

108
Q

Reed sternberg cells

A

Hodgkin lymphoma

these cells release factors that induce accumulation of reactive lymphocytes, macrophages, and granulocytes

109
Q

what characterizes hodgkin lymphoma versus non hodgkin lymphoma

A

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
Q

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

A

Nodular sclerosis HL

111
Q

what is the prognosis of Nodular sclerosis HL

A

excellent prognosis

usually stage I or II

112
Q

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

A

mixed cellularity HL

113
Q

what is the prognosis of mixed cellularity HL

A

advanced tumor stage usually III or IV

114
Q

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

A

Lymphocyte rich HL

115
Q

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

A

lymphocyte depletion

116
Q 
CD20+
CD15-
CD30-
EBV-
BCL6+

popcorn cell (lymphocytic histiocytic cell)

young males

A

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
Q

what can happen in a small percentage of Lymphocyte predominate HL

A

In small percent this transforms into a tumor resembling diffuse large B cell lymphoma

118
Q

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

A

Multiple myeloma

119
Q

what Ig and light chains are involved with Multiple myeloma

A

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
Q

solitary MM

A

single mass in bone or soft tissue

-Extraosseous lesions can occur in lungs, oronasopharynx, nasal sinuses

121
Q

smoldering MM

A
  • lack of symptoms and a high plasma M component
  • serum M protein level >3gm/dL
  • 75% progress to MM over 15 year period
122
Q

what is waldenstrom macroglobulinemia

A

High levels of IgM lead to symptoms related to hyperviscosity of the blood

Occurs in association with lymphoplasmacytic lymphoma

123
Q

** 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

A

Monoclonal gammopathy of undetermined significance (MGUS)

124
Q

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

A

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
Q

what is the prognosis of AML

A

difficult to treat

60% achieve complete remission with chemo but only 15-30% remain free of disease for 5 years

126
Q

t(8;21)

prognosis?

subytpe?

A

AML with myelocytic maturation

favorable prognosis

MPO +

M2

127
Q

inv (16)

prognosis?

A

AMML- acute myelomonocytic leukemia

M4

favorable prognosis

MPO positive
esterase positive

128
Q

t(15;17)

prognosis?

A

intermediate prognosis

treat with all trans retinoic acid and arsenic salts

M3

DIC

APL** diagnosis

129
Q

prognosis of therapy related AML

A

very poor

along with prior myelodysplastic syndrome

130
Q

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

A

Myelodysplastic syndromes

defective maturation of myeloid progenitors gives rise to ineffective hematopoiesis leading to cytopenias

131
Q

what are myeloproliferative disorders

A

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
Q

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

A

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
Q

what is the prognosis of CML

A

Median survival is about 3 years → can go into accelerated phase after this time → ends in presentation similar to acute leukemia (blast crisis)

134
Q

how do you treat CML

A

BCR ABL inhibitors

younger pt’s - hematopoietic stem cell transplant

135
Q

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

A

polycythemia vera

136
Q

what is the spent phase of polycythemia vera

A

progresses to spent phase, with extensive marrow fibrosis that displaces hematopoietic cells → leads to extramedullarhematopoiesis in the liver and spleen

137
Q

what is the prognosis of polycythemia vera

A

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
Q

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)

A

Essential thrombocytosis

139
Q

what is the prognosis of essential thrombocytosis

A

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
Q

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)

A

primary myelofibrosis

141
Q

what is the prognosis and treatment of primary myelofibrosis

A

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
Q

what are the tumor cell markers for langerhans cell histiocytosis

A

HLA-DR

S-100

CD1a

143
Q

what cell type is characteristic of langerhans cell histiocytosis

A

birbeck granules - tennis racket

144
Q

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

A

Multifocal/multisystem LCH: (Letterer-Siwe)

with intensive chemo - 50% 5 year survival

145
Q

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
A

Unifocal LCH (eosinophilic granuloma)

unifocal - older children, rarely adults

multifocal - young children

146
Q

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

A

Multifocal unisystem LCH

147
Q

what is the Hand Schuller christian triad

A

1) Calvarial bone defects
2) Diabetes insipidus
3) Exophthalmos

seen in Multifocal unisystem LCH

148
Q

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

A

pulmonary LCH

May regress spontaneously on cessation of smoking

149
Q

throbbing, burning pain in the extremities

A

essential thrombocythemia

caused by platelet aggregates that occlude small arterioles

150
Q

ringed sideroblasts

megaloblasts

abnormal megakaryocytes

myeloblasts

A

myelodysplasia

151
Q

t (8;14)

A

EBV associated african endemic Burkitts

152
Q

CD5+
t (11;14)
cyclin D1
lymphomatoid polyposis

A

mantle cell

153
Q

what is serum

A

blood that has been allowed to clot

so it is plasma with diminshed fibrinogen and other clotting factos

154
Q

what is plasma

A

To obtain plasma from withdrawn blood, add anticoagulant to prevent clotting

9 % molecules [proteins, organic
and inorganic]

 91 %	       Water
155
Q

normal range for WBC’s

A

4800 - 10800

156
Q

granulocytes make up what percentage of peripheral blood WBC’s

A

40-70%

157
Q

normal range of platelets

A

150-450 (x10^3)

158
Q

CD5+

t(11;14)

A

mantle cell lymphoma

159
Q

monocytosis can be seen in the setting of what

A

chronic infections (TB), bacterial endocarditis, rickettsiosis, malaria. Autoimmune disorders (SLE), inflammatory bowel disease (ulcerative colitis)

160
Q

skin lesions
clover leaf cells
demyelinating disease of the CNS and spinal cord

A

Adult T cell leukemia/lymphoma

HTLV-1 associated

161
Q

tear drop shaped red blood cells

A

myelofibrosis

Heme/Lymph Exam I (4 decks)

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