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Blood and Lymph > MDS, MPNs, Lymphomas, and Plasma Cell Malignancies > Flashcards

MDS, MPNs, Lymphomas, and Plasma Cell Malignancies Flashcards

1
Q

Features of MDS (2)

A

Ineffective hematopoiesis, Increased risk of transformation to AML

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2
Q

Two scenarios of MDS

A

Idiopathic (primary), Therapy-related (secondary

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3
Q

Hypolobation of mature neutrophils (including bilobed nuclei: pseudo-Pelger-Huet cells), hypogranularity of cytoplasm

A

Dysgranulopoiesis

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4
Q

2 reasons for the frequent occurrence of splenomegaly and hepatomegaly in patients with MPNs

A

Sequestration of excess blood cells, extramedullary hematopoiesis

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5
Q

3 possible negative end points for MPNs

A

Transformation to acute leukemia, Transformation to MDS, Excessive marrow fibrosis (resultant marrow failure)

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6
Q

4 Causes of Myelodysplasia that may mimic MDS

A

drugs, deficiency of B12, folate, or other nutrients, viral infection, toxin exposure

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7
Q

BCL1 is characteristic of _____.

A

Mantle Cell Lymphoma

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8
Q

BCL2 is characteristic of ______.

A

Follicular Lymphoma

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9
Q

CHL: both sides of diaphragm, lack collagen bands, EBV+ (75%)

A

Mixed Cellularity CHL

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10
Q

CHL: found in children and older patients

A

Mixed Cellularity Hodgkin lymphoma

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11
Q

CHL: least common of 4 subtypes, usually EBV+, numerous RS cells that may appear anaplastic and bizarre

A

Lymphocyte Depleted CHL

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12
Q

CHL: most common subtype

A

Nodular Sclerosis HL

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13
Q

CHL: nodular growth pattern with residual germinal centers, RS cells present but rare

A

Lymphocyte Rich CHL

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14
Q

CHL: predominates in young adults, especially females

A

Nodular Sclerosis HL

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15
Q

CHL: thickened lymph node capsule with broad bands of collagen and nodular areas surrounded by broad collagen

A

Nodular Sclerosis HL

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16
Q

CLL/SLL is more common in ______ (sex).

A

males (2:1)

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17
Q

CLL/SLL is most common in ______ (demographic).

A

older adults (Median age: 65)

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18
Q

Cytogenetic findings in MDS (5)

A

Monosomy 7; Deletion 7q; Monosomy 5; Deletion 5q; Trisomy 8

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19
Q

Low Grade MDS: Anemia, increased platelets, and distinctive megakaryocytes with small, round, non-lobated nuclei

A

Isolated Deletion of 5q

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20
Q

Low Grade MDS: Good prognosis, median survival >5 years

A

Refractory Cytopenia with Unilineage Dysplasia

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21
Q

Low Grade MDS: Median survival 2.5 years, 10% transformation to AML in 2 years

A

Refractory Cytopenia with Multilineage Dysplasia

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22
Q

Low Grade MDS: myeloblasts account for less than __% of marrow cells and less than __% of peripheral blood cells

A

5; 2

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23
Q

MDS is most common in ______ (demographic)

A

older adults

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24
Q

Megakaryocytes with hypolobated or non-lobated nuclei, often hyperchromatic nuclei, often megakaryocytes with small size

A

Dysmegakaryopoiesis

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25
Q

Most common cause of death in polycythemia vera patients, including locations (3): _____

A

Thrombosis (Mesenteric vein, Splenic vein, Portal vein)

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26
Q

Most common leukemia in the western world

A

Chronic Lymphocytic Leukemia (CLL)

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27
Q

MPN Genetic Findings: BCR-ABL1 fusion gene on der22

A

Chronic Myelogenous Leukemia

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28
Q

MPN Genetic Findings: JAK2 mutations in 50% of cases

A

Polycythemia Vera, Primary Myelofibrosis, and Essential Thrombocytopenia

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29
Q

MPN Morphology: clusters of large bizarre megakaryocytes followed by reticulin fibrosis with loss of marrow space

A

Primary Myelofibrosis

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30
Q

MPN Morphology: Dacryocytes in peripheral blood

A

Primary Myelofibrosis

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31
Q

MPN Morphology: hypercellular marrow with small megakaryocytes containing round non-lobated nuclei

A

Chronic Myelogenous Leukemia

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32
Q

MPN Morphology: large bizarre megakaryocytes

A

Polycythemia Vera and Essential Thrombocytopenia

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33
Q

MPN Prognosis: 10-20 years or more

A

Polycythemia Vera and Essential Thrombocytopenia

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34
Q

MPN Prognosis: 2-3 years (untreated)

A

Chronic Myelogenous Leukemia

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35
Q

MPN Treatment: PTKIs

A

Chronic Myelogenous Leukemia

36
Q

MPN Treatment: serial phlebotomy, aspiring therapy, mild chemotherapeutic drugs

A

Polycythemia Vera

37
Q

MPN: characterized by trilineage hyperplasia with erythrocytosis

A

Polycythemia Vera

38
Q

MPN: Neutrophilia, Basophilia, and Thrombocytosis

A

Chronic Myelogenous Leukemia and Primary Myelofibrosis

39
Q

MPN: sustained marked thrombocytosis

A

Essential Thrombocytopenia

40
Q

MYC is characteristic of ______.

A

Burkitt Lymphoma

41
Q

NHL Cytogenetic Findigns t(11;14)(q13;q32)

A

Mantle Cell Lymphoma

42
Q

NHL Cytogenetic Findings: Burkitt Lymphoma

A

t(8;14)(q24;q32), MYC gene

43
Q

NHL Cytogenetic Findings: CLL/SLL

A

del13q14, Trisomy 12, del11q22-23, del17p13

44
Q

NHL Cytogenetic Findings: CLL/SLL: least common and worst prognosis

45
Q

NHL Cytogenetic Findings: CLL/SLL: most common and best prognosis

46
Q

NHL Cytogenetic Findings: del13q14

47
Q

NHL Cytogenetic Findings: Follicular Lymphoma

A

t(14;18)(q32;q21), BCL2 fusion gene

48
Q

NHL Cytogenetic findings: Mantle Cell Lymphoma

A

t11;14)(q13;q32)

49
Q

NHL Cytogenetic Findings: t(14;18)(q32;q21), BCL2 fusion gene

A

Follicular Lymphoma

50
Q

NHL Cytogenetic Findings: t(8;14)(q24;q32), MYC gene

A

Burkitt Lymphoma

51
Q

NHL Immunophenotype: CD19+, CD20+ only

A

Burkitt Lymphoma

52
Q

NHL Immunophenotype: CD19+, CD20+, CD10+, BCL6+, BCL1,+, CD5+

A

Mantle Cell Lymphoma

53
Q

NHL Immunophenotype: CD19+, CD20+, CD10+, BCL6+, BCL2+

A

Follicular Lymphoma

54
Q

NHL Immunophenotype: CD5+, CD19+, CD23+, CD20+ (weak), sIgG (weak)

55
Q

NHL Immunophenotype: Germinal Center markers

A

CD10+, BCL6+

56
Q

NHL symptoms: Burkitt presents in jaw/facial bones, distal ileum cecum, and omentum (______) or iliocecal (______)

A

Endemic; Sporadic

57
Q

NHL symptoms: highly aggressive with endemic and sporadic types

A

Burkitt Lymphoma

58
Q

NHL symptoms: Lymphadenopathy with massive splenomegaly (50%) and peripheral blood involvement (25%)

A

Mantle Cell Lymphoma

59
Q

NHL symptoms: most closely associated with EBV+, especially in malaria belt

A

Burkitt Lymphoma

60
Q

NHL symptoms: mostly asymptomatic except for lymphadenopathy

A

CLL/SLL and Follicular Lymphoma

61
Q

Non-Hodgkin lymphoma Morphology: germinal center B cell lymphoma, lymph node architectural effacement, closely packed follicles with scant mantle zone

A

Follicular Lymphoma

62
Q

Non-Hodgkin lymphoma Morphology: scant, agranular cytoplasm; monoclonal mature lymphocytes, lymph node architectural effacement

63
Q

Non-Hodgkin lymphoma Morphology: small to medium B lymphocytic neoplasm, effacement of lymph node architecture, starry sky appearance

A

Mantle Cell Lymphoma

64
Q

Non-Hodgkin lymphoma Morphology: starry sky pattern, abundant basophilic cytoplasm, multiple basophilic medium-sized nucleoli

A

Burkitt Lymphoma

65
Q

Non-Hodgkin lymphoma most likely to be seen in children

A

Burkitt Lymphoma

66
Q

Plasma Cell Malignancy: 75% of lesions occur in upper respiratory tract

A

Extraosseus Plasmacytoma

67
Q

Plasma Cell Malignancy: bone marrow-based, multifocal plasma cell neoplasm

A

Plasma Cell Myeloma (PCM)

68
Q

Plasma Cell Malignancy: bone pain in back or extremities, weakness and fatigue

A

Plasma Cell Myeloma (PCM)

69
Q

Plasma Cell Malignancy: high M protein in urine

A

Plasma Cell Myeloma (PCM)

70
Q

Plasma Cell Malignancy: localized plasma cell tumors in tissues outside bone marrow

A

Extraosseus Plasmacytoma

71
Q

Plasma Cell Malignancy: localized tumor of bone, consisting of clonal plasma cells

A

Solitary Plasmacytoma of Bone

72
Q

Plasma Cell Malignancy: male predominance

A

Extraosseus Plasmacytoma

73
Q

Plasma Cell Malignancy: Marrow plasmacytosis <10%, no lytic bone lesions

A

Monoclonal Gammopathy of Undetermined Significance (MGUS)

74
Q

Plasma Cell Malignancy: Monoclonal antibody with no evidence of PCM or other causes

A

Monoclonal Gammopathy of Undetermined Significance (MGUS)

75
Q

Plasma Cell Malignancy: most common gammopathy

A

Monoclonal Gammopathy of Undetermined Significance (MGUS)

76
Q

Plasma Cell Malignancy: Rouleaux formation present in peripheral smear

A

Plasma Cell Myeloma (PCM)

77
Q

Plasma Cell Malignancy: single bone lesion

A

Solitary Plasmacytoma of Bone

78
Q

Plasma Cell Malignancy: Symptoms of PCM (4)

A

Hypercalcemia, Renal Insufficiency, Anemia, Bone lesions

79
Q

Proliferation of one or more myeloid lineages with increases in one or more blood cell types

A

Myeloproliferative Neoplasm

80
Q

RBC precursors with nuclear budding, irregularly-shaped nuclei, lack of coordination between nuclear and cytoplasmic maturation, increased ring sideroblasts

A

Dyserythropoiesis

81
Q

Refractory Anemia with Excess Blasts-__ (RAEB-__): ____% blasts in marrow or ____% blasts in peripheral blood; median survival of 16 months

A

1; 1; 5-9; 2-5

82
Q

Refractory Anemia with Excess Blasts-__ (RAEB-__): ____% blasts in marrow or ____% blasts in peripheral blood; median survival of 9 months

A

2; 2; 10-19; 5-19

83
Q

RS Cells: Common Features (4)

A

Large (up to 100mcm), multiple nuclei or large loblulated nucleus, large eosinophilic nucleolus, ample cytoplasm

84
Q

RS Cells: Important Antigens

A

CD15+, CD30+, CD45(-)

85
Q

Types of High Grade MDS

A

Refractory Anemia with Excess Blasts -1 and -2 (RAEB-1 and RAEB-2)

Blood and Lymph (14 decks)

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