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Flashcards in WBC Disorders Deck (111):
1

list causes of neutrophilic leukocytosis (neutrophilia)

  • infections 
    • pyogenic bacteria
  • sterile inflammation
  • acute inflammation
    • gout, RA
  • acute hemorrhage
  • malignancy

2

list causes of eosinophilic leukocytosis

  • allergic disorders
  • skin diseases 
    • bullous pemphigus & pemphigoid, dermatitis herpetiformis
  • parasitic infestations
  • drug reactions
  • malignancies
    • Hodgkin's, T-cell
  • collagen vascular diseases, vasculitis

3

list causes of reactive monocytosis

  • chronic infxns
    • TB, rickettsiosis, bacterial endocarditis, malaria
  • collagen vascular diseases
    • SLE
  • IBD
    • UC

4

list causes of reactive lymphocytosis

  • viral infxns
    • EBV, Hep A, CMV
  • B. pertussis infxn

5

severe neutropenia is most commonly caused by ___

severe neutropenia is most commonly caused by drugs

  • predictable, dose-related (e.g. chemotherapy)
  • idiosyncratic; many drugs, usually immune-mediated by suppression of marrow precursors

6

for predictable neutropenia, such as following myelosuppressive chemotherapy, treatment includes ____

for predictable neutropenia, such as following myelosuppressive chemotherapy, treatment includes G-CSF (drug = filgrastim)

7

in follicular lymphoma, the tumor arises from ____

list the 2 patterns of growth, 2 types of cells, and their behavior

in follicular lymphoma, the tumor arises from germinal center B cells

2 types = nodular and diffuse

2 cells = small and large

diffuse and large = most aggressive

8

in follicular lymphoma, the translocation occurs between ____

in follicular lymphoma, the translocation occurs between t(14,18), between the heavy chain of Ig and BCL2

9

about 40% of follicular lymphomas transform to a more aggressive lymphoma, usually ____

about 40% of follicular lymphomas transform to a more aggressive lymphoma, usually diffuse large B-cell lymphoma (DLBL)

10

describe the image

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follicular lymphoma

follicular lymphoma cells show strong BCL2 staining

11

describe the image

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follicular lymphoma

nodular aggregates of lymphoma cells are present throughout lymph node

12

describe the etiology of the condition seen in the image

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EBV (mostly) and CMV

"kissing disease" that is common in HS kids

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13

the pathogenesis of the condition in the image is that the virus infects ____ cells but ____ cells are the reactive ones

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the pathogenesis of the condition in the image is that the virus infects B cells but T cells are the reactive ones

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14

describe the presentation of the condition in the image

a rash forms AFTER administration of ____

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  • fever
  • sore throat
  • lymphadenopathy (cervical, axillary, inguinal)
  • hepatosplenomegaly

a rash forms AFTER administration of ampicllin

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15

a ____ test/____ antibody test is positive for ____ but negative for ____ in the condition seen in the image

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a monospot test/heterophile antibody test is positive for EBV but negative for CMV in the condition seen in the image

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16

on bone marrow biopsy of the condition seen in the image, there are atypical, reactive, ____ aka ____

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on bone marrow biopsy of the condition seen in the image, there are atypical, reactive, CD8 T cells aka Downey cells

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17

on lymph node biopsy of the condition seen in the image, there is _____

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on lymph node biopsy of the condition seen in the image, there is paracortical hyperplasia (T cells)

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18

a complication of the condition seen in the image is ___ rupture which can lead to ____

therefore, patients with this condition should avoid ____

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a complication of the condition seen in the image is splenic rupture which can lead to peritonitis → hypovolemic shock

therefore, patients with this condition should avoid contact sports

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19

since the virus causing the condition in the image remains dormant in B-cells, a complication is _____

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since the virus causing the condition in the image remains dormant in B-cells, a complication is non-Hodgkin B cell lymphoma (Burkitt's lymphoma)

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20

describe the image

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Burkitt's lymphoma - lymph node

The tumor cells and their nuclei are fairly uniform, giving a monotonous appearance

Note the high level of mitotic activity (arrowheads) and prominent nucleoli

The "starry sky" pattern is produced by interspersed, lightly staining, normal macrophages 

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21

the condition seen in the image is ____ lymphoma of ___ cells caused by ____

what translocation is associated with this condition?

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the condition seen in the image is non-Hodgkin lymphoma of medium B cells caused by EBV

t(8, 14): c-myc & heavy chain of Ig

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22

describe the 3 types of the condition seen in the image

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  • African/endemic: 100% EBV; doubling time = 24 hours
    • painless mass in mandible → mandibular lymphadenopathy
  • American/sporadic
    • mesenteric lymph nodes → intestinal obstruction (ileocecal)
  • HIV associated BL

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23

the African (aka endemic) variant of the condition seen in the image occurs _____ (location)

whereas

the sporadic (non-endemic) variant occurs _____

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the African (aka endemic) variant of the condition seen in the image occurs on the mandible

whereas

the sporadic (non-endemic) variant occurs in the abdomen (ileocecal) 

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24

on lymph node biopsy of the condition seen in the image, a ____ appearance is seen with microscopy

since the cell are rapidly dividing, ____ figures are also seen and stains positive with ____

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on lymph node biopsy, a starry sky appearance is seen with microscopy (high mitotic rate + apoptosis + macrophages)

since the cell are rapidly dividing, mitotic figures are also seen and stains positive with Ki67

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25

on flow cytometry of the condition seen in the image, the cells are CD____ positive (3 CD markers)

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on flow cytometry of the condition seen in the image, the cells are CD10, CD19, CD20 (B cells!) positive 

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26

list poor prognostic factors for the condition seen in the image

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poor prognosis = B symptoms (fever, weight loss, night sweats), extranodal spread

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27

the condition seen in the image has a good response to ____ and most ____ can be cured, but ____ do worse

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the condition seen in the image has a good response to high dose chemotherapy and most kids/young adults can be cured, but older adults do worse

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28

in the condition seen in the image, the pathogenesis is mature, non-functional malignant ____ that coexpress CD___ and CD___

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in the condition seen in the image, the pathogenesis is naive, non-functional malignant B-cells that coexpress CD20 and CD5

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29

describe the presentation of the condition seen in the image

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  • pancytopenia = malignant B cells activate normal B lymphocytes to form IgG → immune-mediated damage to RBCs (warm autoimmune hemolytic anemia) → (+) Coombs and spherocytes
  • recurrent infections caused by neutropenia and hypogammaglobulinemia 

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30

describe a characteristic finding that is seen on PB smear of the condition seen in the image

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PB smear: smudge cells (mature B cells that get crushed due to fragile membranes)

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31

describe what is seen on lymph node biopsy of the condition seen in the image

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  • LN: diffuse replacement by predominantly small round lymphocytes with few larger cells often concentrated in pale "proliferation centers"

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32

what is the most common cause of death of the condition seen in the image?

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most common COD = recurrent infections due to neutropenia & hypogammaglobulinemia 

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33

the condition seen in the image can also transform into ____, which is also called ____ transformation

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the condition seen in the image can also transform into DLBL, which is also called Richter's transformation

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34

marginal zone lymphoma is an (aggressive or indolent?) lymphoma of small ____ cells

marginal zone lymphoma is an indolent lymphoma of small B cells

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35

the most common site of a marginal zone lymphoma is the ____ (superimposed on  ____)

the most common site of a marginal zone lymphoma is the stomach (superimposed on  Helicobacter gastritis)

antibiotic treatment of Helicobacter gastritis has caused regression of some early stomach marginal zone lymphomas

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36

what are other causes of marginal zone lymphoma?

 

associated with chronic inflammatory states, like Hashimoto thyroiditis (thyroid gland), Sjogren syndrome (salivary glands) and H. pylori gastritis (stomach)

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37

the condition seen in the image is an ____ (aggressive vs. indolent?) ___ cell leukemia

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the condition seen in the image is an indolent B cell leukemia

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38

describe the presentation of the condition in the image

what is NOT seen on presentation?

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  • pancytopenia (normochromic normocytic anemia → fatigue)
  • recurrent infections (neutropenia)
  • bleeding (thrombocytopenia)
  • NO lymphadenopathy 

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39

in the condition seen in the image, there is extramedullary hematopoiesis, which leads to _____ that has a _____ appearance

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in the condition seen in the image, there is extramedullary hematopoiesis, which leads to splenomegaly that has a beefy red appearance (accumulation of hairy cells in red pulp)

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40

describe what is seen on PB smear of the condition in the image, and what it is stained with

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  • PB smear: malignant B cells with cytoplasmic fine hair-like projections
    • TRAP stain positive (TRAP = tartrate-resistant acid phosphatase)

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41

describe bone marrow biopsy of the condition seen in the image

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  • BM: dry tap due to fibrosis (TGF-B and PDGF)
    • infiltrate of small lymphocytes with abundant pale cytoplasm ("fried egg" appearance) enmeshed in reticulin 
    • usually fibrosis means marrow cannot be aspirated

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42

explain how the word "TRAP" helps determine clinical features of the condition seen in the image

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TRAP = tartrate-resistant acid phosphatase staining of hairy cell leukemia

cells get trapped in red pulp = splenomegaly

cells get trapped in bone marrow = dry aspiration

cells trapped in other locations = NO lymphadenopathy (where it should normally be)

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43

the most common cause of death in the condition seen in the image is ____

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the most common cause of death in the condition seen in the image is recurrent infections

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44

what are the characteristic CD markers in the condition seen in the image?

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CD20, CD11c, CD25, CD103

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45

acute leukemias normally have no ____

while 

chronic leukemias do have ____ (with ____ being the exception)

acute leukemias normally have no lymphadenopathy

while 

chronic leukemias do have lymphadenopathy (with hairy cell leukemia being the exception)

46

describe the pathogenesis of the condition seen in the image

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monoclonal proliferation of plasma cells in the bone marrow

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47

describe the pathogenesis of bone symptoms seen in the condition in the image

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  • myeloma-derived factors upregulate the expression of RANKL, which in turn activate osteoclasts → produces punched-out lytic lesions in bone
    • most commonly affected = lumbar spine = back pain
    • breaking bone = hypercalcemia

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48

describe what is seen on serum protein electrophoresis of the condition seen in the image

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M spike (monoclonal Igs) = IgG and IgA

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49

in the condition seen in the image, upon PB smear, ____ formation is seen

explain this

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in the condition seen in the image, upon PB smear, rouleaux formation is seen b/c increased serum protein decreases charge between RBCs

 

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50

describe what is seen on urine analysis of the condition seen in the image

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urine = BJ proteins and kappa & lambda lights chains (AL amyloid)

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51

list the 4 causes of renal failure in the condition seen in the image

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  1. AL amyloidosis
  2. increased uric acid = toxic to renal tubules & uric acid stones
  3. increased Ca2+ = toxic and makes stones
  4. recurrent kidney infxns = acute pyelonephritis 

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52

the most common cause of death in the condition seen in the image is ____

why?

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the most common cause of death in the condition seen in the image is recurrent infections

although there is an increase in Igs, there is monoclonal proliferation and therefore no antigenic diversity

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53

describe the classical vignette for the condition seen in the image

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55+ year old man presents with back pain

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54

the condition seen in the image is composed of ____ cells which are altered _____

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the condition seen in the image is composed of Reed-Sternberg cells which are altered germinal center B-lymphocytes

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55

describe the image

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neoplastic Reed-Sternberg cells (altered germinal center B-lymphocytes) in the minority

non-neoplastic inflammatory cells (lymphocytes, plasma cells, eosinophils) in the background in the majority

56

the classical form of the cells seen in the condition express CD___ and CD___

while

the variant form of the cells seen in the condition express CD___ and CD____

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the classical form of the cells seen in the condition express CD15 and CD30

while 

the variant form of the cells seen in the condition express CD20 and CD45

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57

describe the nodular sclerosis type of the condition seen in the image

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  • commonest subtype of HL
  • mediastinal involvement very common
  • large nodules, at least partly surrounded by thick fibrous collagen bands
  • presence of a type of RS cell called lacunar RS cells

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58

in the nodular sclerosis form of the condition seen in the image, there are large ___, surrounded by ____ 

along with the presence of ____ cells

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in the nodular sclerosis form of the condition seen in the image, there are large nodules, surrounded by thick fibrous collagen bands

along with the presence of lacunar RS cells cells

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59

describe what is seen in the image

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Hodgkin lymphoma, nodular sclerosis type

well-defined bands of pink, acellular collagen that subdivides the tumor into nodules 

 

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60

describe what is seen in the image

Hodgkin lymphoma

Lacunar RS cells

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61

describe the presentation of the condition seen in the image

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  • painless rubbery enlargement of one or more lymph nodes (cervical & axillary)
    • pain only after drinking alcohol
  • B symptoms:
    • fever
    • night sweats
    • weight loss

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62

on LN biopsy of the condition seen in the image, the causative cells have a ____ nucleus

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on LN biopsy, RS cells have a mirror-imaged Owl's eye nucleus

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63

in the condition seen in the image, ___ is the most important prognostic indicator

what factors cause poor prognosis?

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in the condition seen in the image, stage is the most important prognostic indicator

  • poor prognosis = increased RS cells, B symptoms, extranodal spread

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64

describe how to differentiate between ALL and AML

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65

15% of the condition seen in the image are precursor T-cell neoplasms that usually present as a _____ mass in an adolescent male

 

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15% of the condition seen in the image are precursor T-cell neoplasms that usually present as a mediastinal (thymic) mass in an adolescent male

 

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66

the mutation in the T-cell form of the condition seen in the image is usually in the ____ (gene)

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the mutation in this condition is usually in the NOTCH-1 (point mutation) gene

up to 70% of T-ALLs have gain-of-function mutations in NOTCH1, a gene that is essential for T cell differentiation

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67

the condition in the image commonly occurs in ____ and is associated with ____ (before or after?) the age of 5

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the condition in the image commonly occurs in childhood and is associated with Down syndrome after the age of 5

contrast this with AML, where it is associated with Down syndrome before the age of 5

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68

describe the presentation of the condition seen in the image

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  • presentation:
    • pancytopenia, recurrent infections and thrombocytopenia
    • extramedullary hematopoiesis = hepatosplenomegaly
    • bone pain (due to expansion by the blasts)

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69

in the B-cell form of the condition seen in the image, the associated CD markers are ____ (3), and is also ____ positive

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classic B cell CD markers = CD10, 19, 20

also TdT positive

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70

describe the T-cell form of the condition seen in the image

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71

the T cell form of the condition seen in the image is associated with CD markers ___ and is also ____ positive

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the T cell form of the condition seen in the image is associated with CD markers CD2 - CD8

and is also TdT positive

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72

the translocation associated with good prognosis of the condition seen in the image is ____

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the translocation associated with good prognosis of the condition seen in the image is t(12,21)

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73

the translocation associated with a poor prognosis of the condition seen in the image is ____

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the translocation associated with a poor prognosis of the condition seen in the image is t(9,22)

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74

the condition seen in the image most commonly occurs in ____ (age of patients)

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the condition seen in the image most commonly occurs in older adults >50 

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75

describe what is seen in the image

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76

the FAB classification for the condition seen in the image is ____

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the FAB classification for the condition seen in the image is M3

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77

chromosomal translocations of the condition seen in the image usually occur in ____

while 

deletions/monosomy of chr. 5 & 7 usually occur in ____

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chromosomal translocations of the condition seen in the image usually occur in younger adults with de novo AML

e.g. t(15,17), t(8,21), t(16,16)

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78

most translocation in the condition seen in the image confer a better prognosis, except those involving ___

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most translocation in the condition seen in the image confer a better prognosis, except those involving chr. 11

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79

in the condition seen in the image, deletions/monosomy of chr. ___ usually occur in ____

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in the condition seen in the image, deletions/monosomy of chr . 5/7 usually occur in older adults with AML, myelodysplasia (MDS), post-chemo/radiotherapy

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80

based on the condition seen in the image, describe the t(15;17) translocation

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  • translocation of the retinoic acid receptor (RAR) on chr. 17 with the PML gene on chr. 15
    • the PML/RAR fusion protein blocks myeloid differentiation at the promyelocytic stage (probably by inhibiting the function of normal retinoic acid receptors)

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81

describe the presentation of the t(15;17) translocation in the condition seen in the image

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  • presentation: pancytopenia, recurrent infxns, thrombocytopenia
    • extramedullary hematopoeisis: hepatosplenomegaly
    • Auer rods are thrombogenic → DIC
      • Waterhouse Friderichsen syndrome
        • destruction of adrenal cortex → Addison's disease

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82

in the condition seen in the image, there are ____ present in the cell, which are thrombogenic and can ultimately lead to ____

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in the condition seen in the image, there are Auer rods present in the cell, which are thrombogenic and can ultimately lead to DIC

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83

the treatment of the t(15;17) translocation of the condition seen in the image is with ____

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the treatment of the t(15;17) translocation of the condition seen in the image is with ATRA which induces differentiation to neutrophils

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84

the FAB classification for the monocytic variant of the condition seen in the image is ____

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the FAB classification for the monocytic variant of the condition seen in the image is M5

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85

the M4/M5 variant of the condition seen in the image characteristically involves ____ infiltrating the ____

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the M4/M5 (aka acute monocytic leukemia) variant of the condition seen in the image characteristically involves blasts infiltrating the gums

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86

the M4/M5 variant of the condition in the image involves proliferation of ___ and the subtype lacks ___

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the M4/M5 variant of the condition in the image involves proliferation of monoblasts and the subtype lacks MPO

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87

the condition seen in the image is positive for ____ (which helps differentiate it from ___ which is positive for ____)

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the condition seen in the image is positive for MPO (which helps differentiate it from ALL which is positive for TdT)

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88

the M7 variant of the condition seen in the image (aka ____) lacks ____ and is associated with ____

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the M7 variant of the condition seen in the image (aka acute megakaryoblastic leukemia) lacks MPO and is associated with Down sydrome before the age of 5

"need to karyo child under 5"

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89

describe what is seen on BM biopsy in myelodysplastic syndrome (MDS)

usually hypercellular, but ineffective disorganized hematopoiesis

90

in the condition seen in the image, there is proliferation of ____ cells, especially ____ 

____ are markedly increased

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in the condition seen in the image, there is proliferation of mature myeloid cells, especially granulocytes

basophils are markedly increased

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91

the translocation associated with the condition in the image is t(___), which results in ____

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the translocation associated with the condition in the image is t(9;22), which results in overactivity of TK receptor

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92

in the condition seen in the image, there is an increase in neutrophils, so why are there recurrent infections in these patients?

 

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PMNs are monoclonal with decreased LAP (leukocyte alkaline phosphatase) score, and are therefore unable to fight off infections

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93

describe what is seen on PB smear in the condition in the image

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striking left-shifted leukocytosis, with mostly neutrophils & precursors (metamyelocytes, myelocytes)

increase in eosinophils and basophils

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94

what is usually the first symptom seen in the condition in the image?

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sometimes the first symptom is a dragging sensation in the abdomen caused by splenomegaly

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95

describe the presentation of the condition seen in the image

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  • pruritus (itching) after hot showers b/c basophils make histamine
  • extramedullary hematopoiesis → hepatosplenomegaly
  • fatigue, weakness, weight loss

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96

the most common cause of death in the condition seen in the image is ___ due to a decrease in ____ score

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the most common cause of death in the condition seen in the image is recurrent infxns due to a decrease in LAP score

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97

in the condition seen in the image, a complication is ___ and ___ failure due to an increase in ____

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in the condition seen in the image, a complication is gout and renal failure due to an increase in uric acid (hyperuricemia)

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98

in the condition seen in the image, a complication is transformation to ___

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in the condition seen in the image, a complication is transformation to AML which can ultimately cause DIC

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99

the best treatment for the condition seen in the image is with ____

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treatment for the condition seen in the image is with imatinib/Gleevec/STI571 = TK inhibitor

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100

list other potential treatments for the condition seen in the image

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  • allogenic BM transplantation
  • interferon-a slows down disease
  • hydroxyurea = "gentle" chemotherapy

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101

the mutation associated with polycythemia vera is ____

the mutation associated with polycythemia vera is JAK2 mutation

102

in polycythemia vera, due to the JAK2 mutation, the cells are hypersensitive to ____, and there is ____in the serum

in polycythemia vera, due to the JAK2 mutation, the cells are hypersensitive to EPO, and there is low EPO in the serum

103

in the condition seen in the image, there is a mutation in ____ which leads to ____ secreting ____

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in the condition seen in the image, there is a mutation in JAK2 which leads to atypical megakaryocytes secreting TGF-B & PDGF → abnormal deposition of collagen →  BM fibrosis → pancytopenia 

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104

in the condition seen in the image, there is massive ____ hematopoiesis which leads to ____

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in the condition seen in the image, there is massive extramedullary hematopoiesis which leads to hepatosplenomegaly

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105

on BM aspiration in the condition in the image, what is seen?

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DRY TAP due to massive fibrosis

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106

describe what is seen on BM biopsy in the condition in the image

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  • BM biopsy: collagen stains black with reticulin b/c BM is hypocellular

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107

describe what is seen in the PB smear in the condition in the image

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teardrop cells (dacrocytes) & leukoerythroblastosis (immature RBCs and WBCs)

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108

____ cells are commonly seen in a PB smear of the condition seen in the image

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tear-drop cells are commonly seen in a PB smear of the condition seen in the image

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109

the most common cause of death in the condition seen in the image is ____

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the most common cause of death in the condition seen in the image is  recurrent infxns

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110

a complication of the condition in the image is transformation to ____

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a complication of the condition in the image is transformation to AML 

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111

list the diseases that can transform to AML

  • myeloproliferative disorders (blast crisis)
    • CML = most common
    • MDS
    • essential thrombocytosis
    • polycythemia vera
    • myelofibrosis
  • CLL
  • PNH (can also transform to MDS)