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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

follicular lymphoma

follicular lymphoma cells show strong BCL2 staining

11

describe the image

follicular lymphoma

nodular aggregates of lymphoma cells are present throughout lymph node

12

describe the etiology of the condition seen in the image

EBV (mostly) and CMV

"kissing disease" that is common in HS kids

13

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

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

14

describe the presentation of the condition in the image

a rash forms AFTER administration of ____

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

a rash forms AFTER administration of ampicllin

15

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

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

16

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

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

17

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

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

18

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

therefore, patients with this condition should avoid ____

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

19

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

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)

20

describe the image

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 

21

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

what translocation is associated with this condition?

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

22

describe the 3 types of the condition seen in the image

  • 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

23

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

whereas

the sporadic (non-endemic) variant occurs _____

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) 

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 ____

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

25

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

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

26

list poor prognostic factors for the condition seen in the image

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

27

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

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

28

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

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

29

describe the presentation of the condition seen in the image

  • 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 

30

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

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

31

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

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

32

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

most common COD = recurrent infections due to neutropenia & hypogammaglobulinemia 

33

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

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

34

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

marginal zone lymphoma is an indolent lymphoma of small B cells

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

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)

37

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

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

38

describe the presentation of the condition in the image

what is NOT seen on presentation?

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

39

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

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)

40

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

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

41

describe bone marrow biopsy of the condition seen in the image

  • 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

42

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

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)

43

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

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

44

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

CD20, CD11c, CD25, CD103

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

monoclonal proliferation of plasma cells in the bone marrow

47

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

  • 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

48

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

M spike (monoclonal Igs) = IgG and IgA

49

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

explain this

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

 

50

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

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

51

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

  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 

52

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

why?

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

53

describe the classical vignette for the condition seen in the image

55+ year old man presents with back pain

54

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

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

55

describe the image

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____

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

57

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

  • 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

58

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

along with the presence of ____ cells

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

59

describe what is seen in the image

Hodgkin lymphoma, nodular sclerosis type

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

 

60

describe what is seen in the image

Hodgkin lymphoma

Lacunar RS cells

61

describe the presentation of the condition seen in the image

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

62

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

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

63

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

what factors cause poor prognosis?

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

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

64

describe how to differentiate between ALL and AML

65

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

 

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

 

66

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

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

67

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

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

68

describe the presentation of the condition seen in the image

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

69

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

classic B cell CD markers = CD10, 19, 20

also TdT positive

70

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

71

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

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

and is also TdT positive

72

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

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

73

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

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

74

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

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

75

describe what is seen in the image

76

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

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

77

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

while 

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

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)

78

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

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

79

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

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

80

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

  • 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)

81

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

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

82

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

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

83

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

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

84

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

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

85

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

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

86

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

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

87

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

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

88

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

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"

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

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

basophils are markedly increased

91

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

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

92

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

 

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

93

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

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

increase in eosinophils and basophils

94

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

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

95

describe the presentation of the condition seen in the image

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

96

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

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

97

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

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

98

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

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

99

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

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

100

list other potential treatments for the condition seen in the image

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

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 ____

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 

104

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

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

105

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

DRY TAP due to massive fibrosis

106

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

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

107

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

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

108

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

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

109

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

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

110

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

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

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)