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Flashcards in Robbins CH 13 WBCs Deck (107):
1

Syndrome caused by overwhelming acute infection. Petechial hemorrhages occur and there is presence of granulocytopenia?

Systemic inflammatory response syndrome

2

What are the causes of neutrophilic leukocytosis?

Chronic infections and ongoing inflammatory conditions--lung abscesses

3

What can prolonged glucocorticoid use cause?

Increased release of marrow storage pool cells and diminish extravasation of neutrophils into tissues

4

What type of leukemia is associated with basophilia?

Chronic myelogenous leukemia

5

Eosinophilia is a feature of what conditions?

Allergic conditions
Tissue parasitic infections
CML

6

Cytopenia seen with acute infections and inflammatory conditions?

Neutrophilia

7

What are toxic granulations?

Coarse and dark primary granules seen with neutrophils that indicate an overwhelming inflammatory condition

8

What are Dohle bodies?

Patches of dilated ER of neutrophils indicating an overwhelming inflammatory condition

9

Patient presents with leukocytosis and immature myeloid cells in the peripheral blood. A high leukocyte alkaline phosphatase (LAP) score is present. What is the reaction occurring in this patient?

Leukemoid reaction

If abnormal maturation of myeloid cells in marrow and low LAP--> CML

10

What is seen on a blood smear of a patient with infectious mononucleosis?

Large atypical lymphocytes with abundant cytoplasm

Large nucleus with fine chromatin

11

What is the cause of infectious mononucleosis, be specific?

EBV genes--cause proliferation and activation of multiple cones of B cells--Polyclonal B-cell expansion

12

What cells are not increased with infectious mononucleosis?

Basophils
Eosinophils
Monocytes

13

What is present in lymph nodes draining cancer?

Reactive pattern, with dilated sinusoid that have endothelial hypertrophy and filled with histiocytes

14

What are seen on biopsy of lymph nodes caused by a reactive condition (painful)?

Large, variably sized, germinal centers containing numerous mitotic figures

Numerous parafollicular and sinusoidal neutrophils

15

What is CD3?

T-cell marker

16

What is CD19 and 20?

B-cell marker

17

What is CD68?

Macrophage (histiocyte) marker

18

A 5 year old presents with enlarged axillary lymph nodes and a raised erythematous nodule over abrasions. Histology is performed and stellate, necrotizing granulomas are seen. The mother states that she thinks the child was bitten by something. What is the most likely cause of this patients symptoms?

Cat-scratch disease--Bartonella henselae

19

A 7 year old patient present with a mediastinal mass that expresses CD2 and CD7. The patient has a point mutation in NOTCH1 gene. What do the markers represent and what is the dx?

Pre-T cell types

Acute lymphoblastic leukemia/lymphoma (ALL)

20

What does a + TdT marker indicate?

Pre-T and Pre-B cells

21

56 year old pt presents with non-tender axillary and cervical lymphadenopathy. blood smear shows monotonous population of small, round, mature-looking lymphocytes. Flow cytometry is + for CD19 and 5. What is the Dx?

Chronic lymphocytic leukemia (CLL)

22

What translocation is present with Burkitt lymphoma?

8;14

23

What translocation is present with chronic myeloid leukemia (CML)?

9;22

24

What translocation is present with follicular lymphomas?

14;18

25

What are features that support an acute leukemia?

Anemia
Thrombocytopenia
Presence of blasts in the peripheral blood and bone marrow

26

What subtype of acute lymphoblastic leukemia has a good response to chemo, what age group are these patients in and what translocation is present?

Early pre-B cell type--hyperdiploidy

Patients btwn ages 2 and 10

Chromosomal trisomy and t(12;21)

27

What subtype of acute lymphoblastic leukemia has a poor response to chemo/poor prognosis, what age groups, what translocation and what prognostic factors?

T-cell phenotype

Patients 100k

28

What leukemia appears in young to middle-aged adults with peroxidase-postive myeloblasts?

Acute myelogenous leukemia

29

What cells increase in circulation in chronic lymphocytic leukemia?

Many small circulating mature B lymphocytes with scant cytoplasm

30

What do the cells express in chronic lymphocytic leukemia?

CD5/19/20

31

Is chronic lymphocytic leukemia seen in younger or older ind?

Older adults

32

A condition in which platelet destruction is caused by an antibody-mediated process. Lab values show all normal cells except platelet count is reduced?

Idiopathic thrombocytopenic purpura

33

What type of pattern is seen with follicular lymphoma?

nodular or follicular pattern

34

What translocation is seen with follicular lymphoma?

14;18

35

What does the 14;18 translocation cause in follicular lymphoma?

Overexertion of the BCL2 gene by juxtaposing it with the IgH locus--cells resistant to apoptosis

36

Is there bone marrow involvement with acute lymphadenitis?

No

37

What type of cell are involved in a mantle cell lymphoma?

B-cell tumor

38

What translocation is seen with mantle cell lymphoma?

11;14

39

What is caused by the 11;14 translocation seen with mantle cell lymphoma?

Overexertion of the cyclin D1 gene-causing G1-S phase transition

40

What B cell lymphoma is commonly seen in HIV positive patients? How does this occur?

Diffuse large-cell lymphoma

Immunosuppression allows unregulated proliferation and neoplastic transformation of EBV-infected B-cells

41

What are the 2 different Burkitt lymphomas and which one is positive for EBV?

Sporadic-- seen in young children EBV-negative

Endemic-- seen in african children EBV positive

42

B-cell lymphoma that typically appears in the maxilla or mandible of the jaw?

Endemic Burkitt lymphoma seen in african children which is EBV +

43

What translocation is present in Burkitt lymphoma?

8;14--tranlocation of the the MYC gene on ch 8

44

57 year old patient present with a waldeyer's tonsillar ring. Biopsy is performed and the cells are CD19 and 10 positive. A BCL6 mutation is present. The disease responds well to chemo, but is rapidly fatal if it goes untreated. What is the dx?

Diffuse large B-cell lymphoma

1/3 of the cases have BCL6 mutation

T-cell CD3 ABSENT
Monocytic CD15 ABSENT

45

T-cell neoplasm that occur typically in the mediastinum of children?

Lymphoblastic lymphoma

46

Characterized by Reed-Sternberg cells?

Hodgkin lymphoma

47

A B-cell neoplasm that manifest with widespread lymphadenopathy, liver and spleen enlargement, and lymphocytosis?

Small lymphocytic lymphoma

48

Patient presents with bone lysis and pain. A skull X-ray shows punched-out lytic lesions--caused by plasma cells. Urinalysis shows Bence Jones proteinuria. Hypercalcemia and high serum IL-6 is found. What is the dx?

Multiple myeloma

49

What causes the hypercalcemia seen with multiple myeloma?

cell production of MIP1-a which up-regulates RANKL production and increases osteoclast activity--punched out lytic lesions

50

Why are patient with multiple myeloma more susceptible to infection with encapsulated bacteria?

Decreased IgG production

51

Hyperviscosity syndrome involving visual disturbances, HA, Raynaud phenomena, hepatosplenomegaly, lymphadenopathy and very high protein (IgM)?

Lymphoplasmactic lymphoma--waldenstrom macroglobulinemia

52

Russell bodies...

Lymphoplasmactic lymphoma--waldenstrom macroglobulinemia

Bone marrow infiltrated with plasmacytoid lymphocytes that have stored immunoglobulins in their cytoplasm

53

What spike is seen with Lymphoplasmactic lymphoma--waldenstrom macroglobulinemia?

IgM spike caused by neoplastic B-cell differentiation to IgM-producing cells

54

Disease characterized by the presence of an M protein spike in the absence of any associated disease of B cells. the dx is made when M spike is small and the patient has NO Bence jones proteinuria.

Monoclonal gammopathy of uncertain significance (MGUS)

55

disease caused by 11;14 translocation causing the activation of cyclin D1 gene and also does not respond well to chemo.

Mantle cell lymphoma

56

A lymphoma that arises in middle-aged adults at sites of autoimmune or infectious stimulation. Most common sites are thyroid, salivary glands, stomach. Disease can regress with antibiotic therapy, but may also transform into diffuse large B-cell lymphoma...

Marginal zone lymphoma--MALT lymphoma or MALToma

57

What CDs are + with hairy cell leukemia?

CD19+
CD20+
CD11c+

58

What are the 2 defining characteristics of Hairy cell leukemia?

Presence of hairy projections from neoplastic leukocytes in the peripheral blood smear

Coexpression of B-cell (CD19 and 20) and monocyte (CD11c) markers

59

What test is used to ID the cells of hairy cell leukemia?

TRAP

60

What causes the pancytopenia seen in hairy cell leukemia?

poor production of hematopoietic cells in the marrow and sequestration of the mature cells in the spleen

61

Where are auer rods seen?

in myeloblasts in acute myeloblastic leukemia

62

What characteristic gene rearrangement is seen with anaplastic large-cell lymphoma and what does this cause?

2p23 gene rearrangement--causes production of anaplastic lymphoma kinase (ALK) with tyrosine kinase activity

63

What is CD10 a marker for?

B-cell

64

Malignancies of CD4+ and CD3+ Tcells that involves the skin and there is presence of lymphocytes with complex cerebriform nuclei?

Cutaneous T-cell lymphomas

65

What infection causes cloverleaf cells on blood smear?

HTLV-1 infection

66

Bartonella henselae infection that presents with lymphadenopathy with microscopic stellate necrosis?

Cat-scratch disease

67

What is helicobacter pylori associated with?

Associated with MALToma

68

What are Reed-Sternberg cells?

Elaborate cytokines that promote an accompanying reactive cellular proliferation--forming blue of neoplastic mass

CD15+ large cells with multiple nuclei or a single nucleus with multiple nuclear lobes

69

A biopsy of a Hodgkin lymphoma mass with have what cells?

Macrophages
Lymphocytes
Neutrophils
Eosinophils
Plasma cells

70

A type of hodgkins lymphoma with bands of fibrosis, is common in adult women, and commonly involves the mediastina?

Nodular sclerosis HL

71

Type of hodgkin lymphoma that tends to affect older men, express CD15 AND CD30 (which is marker on Tcell/Bcells/monocytes?

Mixed cellularity HL

72

Type of hodgkin lymphoma that has an abundance of reed-sternberg cells and a paucity of lymphocytes. Most cases present with advanced disease and EBV is present in over 90% of the cases?

Lymphocyte depletion variant HL

73

BCL6 gene rearrangements?

typical of diffuse large B-cell lymphomas

74

Deletions of 5q...

Typical of myelodysplastic syndrome

75

JAK2 mutations...

Found in polycythemia vera and other myeloproliferative diseases

76

Lacunar cells...

characteristic of HL

77

Histiocytes with birbeck granules...

Characteristic of the langerhans cell histiocytoses

78

Myeloblasts are characteristic of...

acute myelogenous leukemia

79

A disease involving infiltration of the marrow and the reduction of normal hematopoiesis--anemia and thrombocytopenia. Auer rods are present on smear. Nose bleeds and bleeding gums are seen and blasts are CD33+.

Acute myelogenous leukemia (AML)

80

What CD marker is seen with acute myelogenous leukemia (AML)?

CD33+

81

What do Dohle bodies indicate?

Indicative of marked inflammation--bacterial sepsis caused by reactive changes in mature neutrophils

82

What leukemia can cause DIC by release of granules from promyelocytes?

Acute promyelocytic leukemia (APL)

83

What translocation is seen with acute promyelocytic leukemia (APL)?

15;17

84

What does the 15;17 translocation, seen with acute promyelocytic leukemia, cause?

Fusion of retinoic acid receptor gene on 17 with the promyelocytic leukemia gene on 15--blocking myeloid differentiation

85

What is the treatment for acute promyelocytic leukemia (APL)?

Retinoic acid (Vit A)

86

T(8;14)

Burkitt lymphoma

87

T(8;21)

M2 variant of acute myelogenous leukemia

88

t(14;18)

Follicular lymphoma

89

Peripheral blood count of leukocytes is not high, but leukemic blasts fill the marrow. Blasts are peroxidase negative and nonspecific esterase positive.

Acute monocytic leukemia

90

M5 leukemia is characterized by what?

high incidence of tissue infiltration and organomegly

91

Auer rods
very high WBC count
Presence of peroxidase-postive blasts filling the marrow. most often seen in ind. 15-39.

Acute myelogenous leukemia (AML)

92

Characterized by a cellular marrow in which there are maturation defects in multiple lineages. Dx confirmed by ringed sideroblasts, megaloblasts, abnormal megakaryocytes, and myeloblasts in the marrow.

Myelodysplasia

93

Myelodyplasia develops either...

De novo or after chemo with alkylating agents

94

What is a marker of post therapy myelodyplasia?

chromosomal deletions--such as 5q

95

Ph chromosome 9;22
BCR-ABL rearrangements
Tyrosine kinase activation
disease of pluripotent stem cells

Chronic myelogenous leukemia (CML)

96

t(15;17)

Acute promyelocytic leukemias

97

Myeloproliferative disorder with thrombocytosis, increased megakaryocytes with no evidence of leukemia and presents with throbbing, burning pain in the extremities?

Essential thrombocytosis

98

What causes the burning, throbbing pain seen with essential thrombocytosis?

platelet aggregates that occlude small arterioles

99

Results from an increased RBC mass with increased HCT and blood volume. Very low EPO levels and abnormal platelet function that predisposes pt to bleeding.

Polycythemia vera

100

Polycythemia vera can blast out into...

Acute myelogenous leukemia and other patient develop chronic myelogenous leukemia

101

Teardrop RBCs are seen with what?

When marrow undergoes fibrosis in myelofibrosis with myeloid metaplasia

102

What disorder has Birbeck granules--tennis rackets?

Langerhans cell proliferations--langerhans cell histiocytosis

103

Hand-Schuller-Christain disease--diabetes insidious is seen in what disease?

Langerhans cell histiocytosis

104

A form of langerhans cell histiocytosis with birbeck granules..

Letterer-Siwe disease

105

Seen in anterior medistinal (thymic) masses in children with acute lymphoblastic leukemia/lymphoma?

Lymphoblasts that mark as T cells (CD3+)

106

Ringed sideroblasts...

Myelodysplastic syndromes

107

Sezary cells...

Peripheral T-cell lymphoma/leukemias

Often involves the skin