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HemeOnc Block 2 > Path part 2 ho2 > Flashcards

Path part 2 ho2 Flashcards

1
Q

What is the gold standard in investigation of acute lymphadenitis?

A

culture

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2
Q

What is the most common type of reactive lymphoid hyperplasia, especially in children?

A

follicular lymphoid hyperplasia

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3
Q

The paracortical pattern of reactive lymphoid hyperplasia has…

A

interfollicular expansion, mainly T cells

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4
Q

What are the three patterns of reactive lymphoid hyperplasia?

A

follicular pattern

paracortical pattern

sinus pattern

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5
Q

When would you see a sinus pattern of reactive lymphoid hyperplasia?

A

lymph nodes draining tumors

whipple disease

rosai-dorfman

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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

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7
Q

Which one is the non-necrotizing granuloma of sarcoidosis?

A

the darker purple image

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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

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9
Q

Mets to the L supraclavicular node is probably from a primary tumor of…

A

abdominal, esp gastric

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10
Q

What is the MC congenital abnormality of the spleen? what is this also called?

A

accessory spleen

spleniculi

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11
Q

When is a congenital accessory spleen even significant?

A

in hematologic disorders when splenectomy is a tx

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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

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13
Q

thick white plaques covering the surface of the spleen, common incidental finding at autopsy

A

perisplenitis

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14
Q

most commonly, cirrhosis will cause splenomegaly due to its…

A

congestive state

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15
Q

congestive splenomegaly is a direct result of …

A

venous outflow obstruction

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16
Q

thrombosis of hepatic veins causing splenomegaly

A

Budd-Chiari syndrome

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17
Q

What are some causes of splenic infarcts?

A

endocarditis

severe atherosclerosis

massive splenomegaly

thrombosis of splenic vein

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18
Q

splenic infarct appearance

A

pale, tan, wedge shaped, esp if arterial

subcapsular

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19
Q

What are the MCC of splenic ruptures?

A

trauma and surgical intervention

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20
Q

What are splenic inplants?

A

splenosis - little mini spleens appearing after rupture

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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

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22
Q

What are some causes of hypersplenism?

A

autoimmune diseases

congestive splenomegaly

Gaucher disease

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23
Q

What is the MC primary tumor of the spleen?

A

hemangioma - usually cavernous type and less than 2 cm in size

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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

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25
Q

A rare, nodular lesion of splenic sinus lining cells, usually an incidental finding…

A

hamartoma

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26
Q

a malignant primary tumor of the spleen is rare, but the MC is…

A

angiosarcoma

well-defined hemorrhagic nodule or involve diffuse spleen

can lead to spontaneous rupture of organ

can be after FB placement (gauze sponge)

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27
Q

What are the MC type of malignancy in the spleen?

A

lymphomas

splenic involvement is usually secondary

DLBCL is MC and can be restricted to only the spleen

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28
Q

What are the most common primary sites if the spleen is getting mets?

A

melanoma

lung

breast

stomach

pancreas

liver

colon

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29
Q

premature infants or term infants exposed to stress in utero my have this issue with the thymus… what does this mean?

A

acute thymic involution

greatly increased lymphocyte death

and is probs mediated by high levels of steroids (adult cases)

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30
Q

an incidental finding during thyroid surgery in surgery in preteens, rare in adults

A

ectopic thymus tissue

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31
Q

What ectopic tissue is MC found in thymus?

A

parathyroid

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32
Q

Thymic hypoplasia syndrome

A

DiGeorge syndrome

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33
Q

Thymic hyperplasia

A

reactive B lymphoid follicles within the thymus seen in chronic inflammatory and immunologic states, esp myasthenia gravis

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34
Q

MC primary anterior mediastinal neoplasm….

made of what?

benign or malignant?

associated with anything?

A

thymoma

tumor of thymic epithelial cells

benign, mostly

parneoplastic syndromes, such as MG

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35
Q

What is this and what do the histo types look like?

A

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

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36
Q

What are the two types of malignant thymoma?

A

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

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37
Q

What are the main primaries of metastatic thymus disease?

A

esophagus

lung, pleura

breast

thyroid

melanoma

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38
Q

monoclonal proliferation is considered to be…

A

neoplastic

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39
Q

Who is affected by B cell NHL?

Where do they originate?

spread is…

staging NHL

A

mostly older adults

LN, but can be other tissues

unpredictable

is not super useful

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40
Q

What is the MC B cell NHL? follow by…

A

Diffuse large B cell lymphoma

follicular lymphoma

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41
Q

What is the MC leukemia in adults?

A

chronic lymphocytic leukemia/small lymphocytic lymphoma

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42
Q

What is the pathogenesis behind CLL/SLL?

A

Bcl-2 gets upregulated, an anti-apoptotic gene

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43
Q

If bone marrow is not involved in CLL, what is it called?

A

Small lymphocytic lymphoma

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44
Q

Clinical presentation of CLL

A

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

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45
Q

CLL lab findings

A

CBC - lymphocytosis >5k

may have monoclonal ig

periph blood cytopenias

incrased LDH

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46
Q

CLL peripheral smear has…

A

smudge cells

coarse, clumped chromatin making soccer ball appearances

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47
Q

Rai staging in CLL

A
  1. lymphocytosis in blood and marrow
  2. lymphocytosis and lymphadenopathy
  3. lymphocytosis and hepatosplenomegaly
  4. lymphocytosis and anemia
  5. lymphocytosis and thrombocytopenia

(higher stage = worse prognosis)

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48
Q

Binet staging CLL

A

A. no cytopenia, <3 lymphoid areas enlarged

B. no cytopenia, >=3 lymphoid areas enlarged

C. anemia or thrombocytopenia

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49
Q

What are three things that CLL could transform into?

A

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

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50
Q

DLBCL affects who?

How does it come about?

prognosis?

A

middle age and elderly

arises de novo usually

aggressive and requires therapy

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51
Q

What are sites of involvement of DLBCL?

A

LN most often, can affect GI tract and spleen

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52
Q

What is the clinical presentation of DLBCL?

A

rapidly enlarging nodal or extranodal masses

systemic B symptoms

increased LDH and beta-2 microglobulin

most present late

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53
Q

What is the MC variant of DLBCL?

A

centroblastic variant - cells look like centroblasts of germinal centers, large cells with moderate amount cytoplasm, vesicular nuclei with 2-3 small nucleoli

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54
Q

How would you tx DLBCL?

A

de novo arising - could cure with R-CHOP

if secondary arising, probs resistant to therapy

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55
Q

follicular lymphoma defined, path, and prognosis

A

malignant germinal center B cells

t(14:18) causing overexpression of anti-apoptotic BCL2

indolent but incurable, may transform to DLBCL (Richter transformation)

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56
Q

What is the gross appearance of follicular lymphoma?

A

spleen with lots of small nodules

LN has tan/white “fish flesh” appearance, +/- many small nodules

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57
Q

What will the microscopic veiw of follicular lymphoma look like?

A

close follicles without mantle or marginal zones (no super dark blue cells around circles)

“snake bite” cells/centroblasts

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58
Q

What differentiates reactive follicles from follicular lymphomas?

A

Bcl-2 stain, positive in lymphomas

(much darker area)

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59
Q

Rarely, peripheral blood is involved in follulicular lymphoma, but when it is, I see…

A

buttock cells - midline cleaved appearance

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60
Q

What is a plasma cell myeloma?

A

malignancy of monotypic plasma cells with clinical BM plasmacytosis, osteolytic bone lesions, and monoclonal gammopathy

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61
Q

What are some clinical features of plasma cell myeloma?

A

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

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62
Q

A person with plasma cell myeloma will usually die from…

A

bleeding or infection

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63
Q

What is the morphology of plasma cell myeloma like?

A

rouleax is common in peripheral blood (stacking of RBCs)

varying appearance of plasma cells with different growth patterns

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64
Q

What can occur in the absence of myeloma, though it is considered a plasma cell neoplasm?

A

primary amyloidosis

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65
Q

What is the structure associated with primary amyloidosis?

What kind of staining goes with amyloidosis?

A

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

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66
Q

What are solitary neoplasms of monoclonal plasma cells like?

A

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)

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67
Q

How do you tx solitary neoplasms of monoclonal plasma cells?

A

all tx by radiotherapy only

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68
Q

Where will you most often find extraosseous plasmacytoma?

A

upper respiratory tract and sinuses

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69
Q

Solitary plasmacytoma of bone, 2/3 of the time…

A

evolve into myeloma or additional plasmacytomas

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70
Q

What is lymphoplasmacytic lymphoma?

A

rare neoplasm, small B cells, plasmacytoid lymphocytes, and plasma cells

no specific immunophenotype or genetic abnls

includes Waldenstrom macroglobulinemia

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71
Q

What are clinical features of LPL?

A

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%

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72
Q

What is the morphology of LPL?

A

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)

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73
Q

What infection/conditions are a/w MALT lymphomas?

A

H. pylori infection

chronic inflammatory conditions

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74
Q

MALT lymphoma symptoms are…

A

usually related to organ involved, not usually involving BM

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75
Q

How would you tx MALT lymphoma?

A

tx the infection or underlying disease

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76
Q

old man, diffuse adenopathy, marrow positive, CD5 marker, Cyclin D1, t(11:14)

A

Mantle cell lymphoma

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77
Q

What does mantle cell lymphoma look like?

A

diffuse or nodular proliferation of monomorphic small lymphocytes, without prolif centers or centroblasts

no plasma cells

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78
Q

What can mantle cell lymphoma transform into?

A

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

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79
Q

AA boy with jaw swelling

A

Burkitt lymphoma, aggressive rapidly growing B cell neoplasm

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80
Q

Endemic Burkitt lymphoma characteristics

A

Africa

kids

jaw and facial bone involved, or gonadal, breast, abdominal sites

geographically corresponds to malaria

EBV positive in most cases

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81
Q

Sporadic Burkitt Lymphom appearance

A

WW, low incidence

children and YA

abdominal tumors MC, also gonads, kidneys, and breast (jaw tumors rare)

Only 30% EBV positive

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82
Q

immunodeficiency associated Burkitt lymphoma

A

adults

HIV/AIDS

post-transplant

iatrogenic (drugs for AI dz)

involves LN and BM in most

EBV varies

83
Q

Burkitt lymphoma immunophenotype

A

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
Q

What is the morphology a/w Burkitt lymphoma?

A

starry sky appearance

85
Q

What is the MC childhood malignancy (hematopoietic or solid tumor)

A

B- ALL

86
Q

What causes an increased risk for B-ALL and AML

A

Down syndrome, genetic alterations, radiation, toxin, previous chemo

87
Q

B-ALL/ LBL morphology

A

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
Q

bone marrow biopsy of B-ALL

A

hypercellular, increased lymphoblasts and lots of mitoses, packed with blasts

rarely will have regions of necrosis

89
Q

What is the MC hematopoietic neoplasm that causes marrow necrosis?

A

ALL

90
Q

What will the immunophenotype be of B-ALL?

A

CD19 and other pan-Bcell markers

plus markers of immaturity CD34 and TdT

91
Q

What scenarios account for the best prognosis of B-ALL

A

age 1-10 yo

female gender

WBC <50k

no CNS dz

common immunophenotype

hyperdiploidy (>50 chromosomes)

92
Q

What factors into a bad pronosis for B-ALL?

A

less than 1 year or greater than 10yo

male

WBC >50k

CNS disease

absences of CD10

hypodiploidy

93
Q

T-ALL and T-LBL is more common in…

A

adolescent males

94
Q

clinical presentation of T-ALL

A

bone marrow always involved

mediastinal involvement with rapidly growing mass

LAD and heptosplenomegaly

CNS involvement is MC than with T-LBL

95
Q

T- LBL clinical presentation

A

only in BM of 20% of cases at dx

rapidly growing mediastinal mass

pleural and pericardial effusions often present

96
Q

T-ALL blasts immunophenotypes

A

CD2, CD5, CD7, TdT, CD34, CD10+/-

97
Q

How are T cell and NK cell neoplasms more likely to be detected

A

more likely detected by T cell antigens lost than expressed

98
Q

As a group, which has generally worse prognosis between T-ALL and B-ALL

A

T-ALL

99
Q

What is the MC primary cutaneous T-cell lymphoma?

A

mycoses fungioidies

100
Q

What markers might cells in mycoses fungioides have?

A

CD4 T cells with loss of CD7 or another cell marker

101
Q

What is the clinical course of mycoses fungioides?

A

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
Q

cytologic features of Mycosis fungioides

A

small/med lymphocytes with cerebriform nuclei, like sezary cells

Pautrier microabscess develop in the plaque stage

103
Q

a form of leukemia,

has involvement of skin, blood, and LNs

usually arises de novo, sometimes with Mycosis fungioides

has a triad of…

A

sezary syndrome

erythroderma, generalized LAD, cerebriform nuclei in skin, peripheral blood and LNs

104
Q

What are these cells and what are they seen in?

A

cerebriform nuclei

sezary syndrome, mycosis fungioides

105
Q

What are markers of Adult T cell leukemia/lymphoma (ATLL)?

A

only in person with HTLV-1

from CD4 and CD25 positive T reg cells

old man from carribean

106
Q

What occurs in acute ATLL?

A

“flower cells”, LAD, skin lesions*, increased LDH, hypercalcemia, lytic bone lesions, renal disease, neuropsych disease

107
Q

What are three definitive parts of chronic ATLL?

A

lymphocytosis

exfoliative skin rash

NO hypercalcemia

108
Q

What are markers of lymphomatous ATLL?

A

worse LAD than acute

skin involved

less hypercalcemia than acute

no blood involvement

109
Q

What are features of smolder ATLL?

A

>5% tumor cells in periph blood, but normal WBC count

tumor cells less atypical

no hypercalcemia or LAD

110
Q

What has pautrier like microabscesses on histo and is HTLV-1 positive?

A

ATLL skin

111
Q

T cell large granular lymphocytic leukemia features

A

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
Q

What is this a histo pic of? describe it

A

T LGLL

large granular lymphocytes, intermediate size, inconspicuous nucleoli and abundant cytoplasm with azurophilic granules

113
Q

Anaplastic large cell lymphoma most often affect who? what do they often have, ssx?

A

adolescents and YA

often have “B symptoms” esp high fever

114
Q

What stage does a pt with anaplastic large cell lymphoma usually present in? Is this treatable?

A

stage III/IV

yes, somewhat tx’able, 80-90% survivial

115
Q

What features are extremely characteristic of anaplastic large cell lymphomas?

A

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
Q

What is circled?

A

hallmark cell of anaplastic large cell lymphoma

117
Q

Is staging more important for NHL or HL?

A

HL

118
Q

Where does HL originate?

A

nodal based

119
Q

What is the pathognomic cell of HL?

A

reed sternberg cells- abnormal B cells

120
Q

What does Nodular Lymphocyte Predominant Hodgkin lymphoma look like on histo?

A

nodal architecture is distored by multiple expansile nodules with compressed interfollicular zones

nodules have a moth-eaten appearance

121
Q

What are these slides of?

A

Nodular LP hodgkin lymphoma

mummified condensed cytoplasm and nuclei with irregular nuclear contours on L

clusters of histiocytes forming granulomas on the R

122
Q

Where will I find popcorn cells?

A

Neoplastic cells of NLPHL

they are lymphocyte predominate (LP) or lymphohistiocytic (LH) cells

large cells with multilobulated nuclear contours and resemble popcorn

123
Q

What is the clinical presentation of a pt with Nodular LP hodgkins lymphoma?

lab tests?

grossly, the LN is…

A

male, 35 yo, LAD, not often having B symptoms

normal CBC, not leukemic, rarely LDH or beta-2 elevated

large and multinodular

124
Q

Ancillary testing done for NLPHL would be…

A

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
Q

Classical hodgkin lymphomas are in what ages of people?

A

bimodal age curve

126
Q

What is this guy?

A

Reed sternberg cells

must have 2 nucleoli in two separate nuclear lobes to be called diagnostic

127
Q

pyknotic, reddish reed sternberg cells are called…

A

mummifed cells

128
Q

What are reed sternberg cells immunophenotype?

A

CD30 +

maybe CD15

negative for CD20 and CD45

129
Q

What is the MC type of classic hodgkin lymphoma in developed world? What does it look like?

A

nodular sclerosis CHL

nodules of neoplastic cells admixed with inflammatory cells, surrounded by collagenous bands

neoplastic cells are mainly lacunar cells (HRS variant)

130
Q

nodes tending to be matted, with visible nodulatiry and fibrous bands around the nodules are what type of CHL?

A

nodular sclerosis CHL

131
Q

What is this?

A

nodular sclerosis CHL

one has fibrous bands and the other has the lacunar cells

132
Q

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

A

mixed cellularity CHL

133
Q

MC CHL in underdeveloped world?

A

mixed cellularity CHL

134
Q

What is this?

A

mixed cellularity CHL with classic reed sternberg

135
Q

CHL with predominance of small reactive lymphocytes…

A

Lymphocyte rich CHL

136
Q

Involvement of ________ is common in lymphocyte rich CHL

A

Waldeyer ring

137
Q

What does the background of lymphocyte rich CHL look like?

A

follicular dendritic cells, similar to NLPHL

138
Q

What is this?

A

lymphocyte rich CHL

‘open’ areas are actually larger cells with increased cytoplasm

139
Q

likely a progression from other types of CHL… in older pts at onset… presents in what stage?

A

lymphocyte deplete CHL

stage III or IV presentation

140
Q

What is this?

A

lymphocyte depleted CHL

lots of reed sternberg

few small lymphocytes, inflammatory background

141
Q

What are common features of all types of myeloid neoplasms?

A

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
Q

Chronic myeloid leukemia is most frequent in this age and has this abnormality

A

middle aged

Philadelphia chromosome t(9:22) BCR/ABL1

143
Q

What are periph blood findings in CML?

A

leukocytosis and and granulocytic left shift

basophilia

eosinophilia common

blasts <2%

144
Q

Clinical presenation in CML usually has

A

40-60 yo

splenomegaly

anemia

NO LAD

anemia

platelet dysfunction

gout or renal disease from hyperuricemia

145
Q

Bone marrow findings in CML

A

increased cellularity

increased megakaryocytes with small monolobulated forms (dwarf megakaryocytes)

Pseudo-Gaucher cells (sea blue histiocytes) due to increased cell turnover

146
Q

When is CML considered to be in the “blast” stage? Once in this stage, what is the prognosis?

A

when blasts comprise >=20% of the peripheral blood WBC or the nucleated cells of marrow

usually fatal within weeks-months

147
Q

death in CML is usually due to…

A

infection or bleeding

148
Q

panmyelosis is usually seen in ….

what is responsible for most of the clinical ssx?

A

polycythemia vera

increase in RBCs

149
Q

What gain of function mutation is polycythemia vera a.w?

A

JAK 2

150
Q

RBCs responsible for ssx of polycythemia vera present as…

A

HTN, HA, dizziness, venous or arterial thrombosis, Splenomegaly

151
Q

What are the three phases of polycythemia vera?

A

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
Q

What is the major goal in polycythemia vera tx?

A

reduce risk of thrombosis

153
Q

What disease is this?

A

CML

154
Q

What disease is this?

A

polycythemia vera

155
Q

sustained thrombocytosis >450k and overproduction of megakaryocytes could indicate

are there any mutations a/w?

A

essential thrombocytosis

JAK2 in about half of the cases

NO genetic abnls associated

156
Q

What is the clinical presentation of essential thrombocytosis?

A

abnl CBC

vascular disturbances either thrombosis or hemorrhage, arterial or venous

157
Q

Periph blood in ET has no…

A

significant L shift

158
Q

BM in ET is…

A

normal to slightly hypercellular

striking megakaryocytic production

absent or minimal reticulin fibrosis

159
Q

This is peripheral blood in what?

A

essential thrombocytosis

160
Q

This is bone marrow in what?

A

essential thrombocytosis

161
Q

an indolent disease of adults >60 yo, it has nonspecific ssx, and proliferation of mainly megakaryocytes and granulocytes

A

primary myelofibrosis

162
Q

When does primary myelofibrosis present and what are some of its features?

A

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
Q

What is dyspoiesis?

A

dysplasia of blood cells

164
Q

What is this?

A

dyserythropoiesis

165
Q

What is this? What does it usually have in regards to nuclei

A

dysmegakaryopoiesis

odd number of nuclei

166
Q

Chronic myelomonocytic leukemia does not have…

A

Ph chromsome or BCR/ABL1 fusion gene

167
Q

CMML presents in who… what does it look like?

A

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
Q

Myelodysplastic neoplasms generally have..

A

bone marrow hyperceullarity

ineffective hematopoiesis

not often, organomegaly

169
Q

MDS/MPN has no known…

A

cytogenetic or molecular genetic abnormalities

170
Q

What do we see here?

A

CMML

2 segments of nuclei, coarse chromatin, hypogranular,

typically see increased monocytes on the right side

171
Q

What is this?

A

hypercellular bone marrow in CML

172
Q

general characteristics of myelodysplastic neoplasms

A

cytopenias

dyspoiesis

hypercellular marrow

normal or increased marrow blasts, <20%

organomegaly is not often

173
Q

symptoms of myelodysplastic neoplasms are….

A

related to cytopenias

most pts are anemic and transfusion dependent

174
Q

Blood: cytopenia in at least one cell type

unilineage dysplasia

no or rare blasts

BM: unilineage dysplasia

<5% blasts

<15% sideroblasts

A

Refractory cytopenia with unilineage dysplasia

175
Q

blood: anemia, dimorphic pattern, no blasts

BM: >15% of erythroid precursors are ringed sideroblasts, dysplasia in erythroid lineage only

<5% blast

A

refractory anemia with ring sideroblasts

176
Q

What am I looking at?

A

ringed sideroblasts with increased iron

177
Q

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

A

refractory cytopenia with multilineage dysplasia

178
Q

What am I looking at?

A

refractory cytopenia with multilineage dysplasia

179
Q

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

A

MDS with isolated del(5q)

180
Q

Differentiate Refractory anemia with excess blasts 1 and 2

A

RAEB 1 has <9% blasts in bm

RAEB 2 has 10-19% blasts in bm

181
Q

What are auer rods?

A

primary granules that have coalesced within myeloblasts, not in normal cells

182
Q

general characteristics of AML

A

malignant proliferation of immature myeloid cells

distinct peroxidase positive auer rods

183
Q

What is the MC type of leukemia in adults and neonates?

A

AML

184
Q

blasts in AML

A

comprise at least 20% blasts in blood or bone marrow

185
Q

AML specific ssx

A

gingival hyperplasia (with AML of monocytic lineage)

DIC with APL

organomegaly is uncommon

186
Q

When is the blast count of 20% not required for diagnosis of AML?

A

AML with recurrent genetic abnormalities

187
Q

AML t(8:21)

A

MC type of childhood AML

good response to chemo

ETO

188
Q

AML with inv (16)

A

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
Q

Acute promyelocytic leukemia t(15:17)

A

PML-RARalpha

pts commonly develop DIC, so rapid dx is essential

hematologic emergency

190
Q

What is this?

A

two variants of APL

191
Q

This is APL under which stain, super duper positive, obvi

A

myeloperoxidase stain

192
Q

acute monoblastic leukemia extramedullary lesions include

A

gingival hyperplasia

leukemia cutis

193
Q

What is the hallmark of SLE?

A

production of array of autoantibodies, esp ANAs.

194
Q

What are the 11 things that can characterize SLE? Need 4 /11

A

serositis

oral ulcers

arthritis

photosensitivity

blood disorders

renal involvement

ANA

immunologic phenomenon

neurologic disorder

malar rash

discoid rash

195
Q

What two abs are largely diagnostic of SLE?

A

anti smith

anti ds dna

196
Q

How is tissue injured in SLE?

A

deposition of immune complexes and binding of antibodies to various cell and tissues, esp kidneys

197
Q

What is the genetic predisposition for rheumatoid arthritis?

A

HLA-DR4

DR1

DR10

DR14

198
Q

What is an ab associated with RA?

A

anti-CCP ab

199
Q

synovial hyperplasia occurs in what autoimmune disease?

A

rheumatoid arthritis

200
Q

What abs are associated with Sjogrens syndrome? What does it appear on IF?

A

SS-A Ro

SS-B La

speckled appearance on IF studies

201
Q

What gives a 40x increase in developing MALT lymphoma?

A

Sjogrens syndrome

202
Q

What abs are associated with scleroderma?

A

DNA topoisomerase I (diffuse systemic)

anticentromere ab (limited systemic sclerosis)

203
Q

CREST syndrome is a/w what autoimmune disease?

A

scleroderma

204
Q

High antibody titers to U1 ribonucleoprotein

A

Mixed CT disease

HemeOnc Block 2 (3 decks)

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