LymphomaLeukemia Flashcards Preview

Heme > LymphomaLeukemia > Flashcards

Flashcards in LymphomaLeukemia Deck (159):
1

Name the condition: t(12;21)

B-ALL with better prognosis

1

Name the condition: t(9;22)

Classically CML, but also a subset of B-ALL more commonly seen in adults and has a poorer prognosis

1

Name the cell type: CD2-8 but not CD10

T lymphoblasts (T-ALL)

2

Name the cell type: CD10, CD19, CD20

B lymphoblasts (B-ALL)

3

Name the condition: Presents as a thymic mass in teenagers

T-ALL

4

Name the cell type: tDt+

Lymphoblasts (as in B-ALL and T-ALL)

5

Name the cell type: Myeloperoxidase+ (Auer Rods)

Myeloblasts (as in AML), and promyelocytes (APL)

6

Name the condition: t(15;17)

Acute Promyelocytic Leukemia

7

Name the disrupted gene: t(15;17)

Retinoic Acid Receptor causin promyelocytes to accumulate

8

Auer rods can activate what

The coagulation cascade, leading to DIC. Promyelocytes have numerous Auer rods, and this is why Acute promyelocytic leukemia is a medical emergency.

9

What is the treatment for Acute Promyelocytic Leukemia

All-Trans Retinoic Acid (ATRA). It binds the disrupted RAR and causes blasts to mature

10

Swelling of the gums is a symptoms seen in which condition

Acute Monocytic Leukemia (AMoL)

11

This condition has an association with Down Syndrome before the age of 5

Acute Megakaryoblastic Leukemia (AMKL)

12

This condition has an association with Down Syndrome after the age of 5

Acute Lymphoblastic Leukemia (ALL)

13

Cytopenia with a hypercellular bone marrow of <20% blasts is characteristic of what

Myelodysplastic syndrome

14

What do people with Myelodysplastic Syndrome usually die of

Infection or bleeding (due to decreased white cells and platelets)

15

CD5+ B cells are seen in which condition

Chronic Lymphocytic Leukemia (CLL)

16

Name the condition: Smudge Cells

Chronic Lymphocytic Leukemia (CLL)

17

Name the condition: Tartrate-resistant acid phosphatase+ (TRAP) cells

Hairy Cell Leukemia

18

Name the condition: Massive splenomegaly due to red pulp expansion

Hairy Cell Leukemia

19

Name the condition: Lytic bone lesions, hypercalcemia, and a rash

Adult T-cell Leukemia/Lymphoma (ATL)

20

Name the condition: Lytic bone lesions and hypercalcemia without a rash

Multiple Myeloma

21

Name the condition: Aggregates of neoplastic T-cells in the epidermis (Pautrier Microabscesses)

Mycosis Fungiodes

22

Name the involved genes: t(9;22)

BCR-ABL fusion with increased tyrosine kinase activity

23

Treatment for BCR-ABL fusion

Imatinib which blocks tyrosine kinase activity

24

Granulocytosis with LAP+ (Leukocyte Alkaline Phosphatase) granulocytes indicates what

Normal response to infection

25

Granulocytosis with LAP- (Leukocyte Alkaline Phosphatase) granulocytes indicates what

Chronic Myelogenous Leukemia

26

Granulocytosis with no increase in basophils indicates what

Normal response to infection

27

Granulocytosis with and increase in basophils indicates what

Chronic Myelogenous Leukemia

28

What is Polycythemia Vera

Neoplastic proliferation of mature myeloid cells, but especially RBCs

29

Most common cause of Budd-Chiari Syndrome (occlusion of the hepatic veins that presents with the classical triad of abdominal pain, ascites and hepatomegaly)

Polycythemia Vera

30

Polycythemia Vera is caused by what mutation

JAK2 kinase mutation

31

Polycythemia with increased EPO and low SaO2 indicates what

Reactive polycythemia, as in lung disease

32

Polycythemia with decreased EPO and normal SaO2 indicates what

Polycythemia Vera

33

Polycythemia with increased EPO and normal SaO2 indicates what

Ectopic EPO, as in renal cell carcinoma

34

What is Essential Thrombocythemia

Neoplastic proliferation of mature myeloid cells, but especially platelets

35

Essential Thrombocythemia is caused by what mutation

JAK2 kinase mutation

36

What besides Essential Thrombocythemia can cause excess platelets in the blood

Iron deficiency anemia

37

The myelodysplastic syndromes can cause marrow fibrosis, hyperuricemia/gout, and can progress to acute leukemia with the exception of which one

Essential Thrombocythemia

38

What is Myelofibrosis

Neoplastic proliferation of mature myeloid cells, but especially megakaryocytes

39

Myelofibrosis is caused by what mutation

JAK2 kinase mutation

40

Teare drop cells are commonly seen in which myeloproliferative disorder

Myelofibrosis

41

Name the condition: t(14;18)

Follicular Lymphoma

42

What genes are affected by t(14;18)

Translocation brings BCL-2 gene (on chromosome 18) into the position of IgH (on chromosome 14), which is highly active, resulting in the overexpression of BCL-2.

43

What does BCL-2 do

It stabilizes the mitochondrial membrane, preventing cytochrome-c from leaking into the cytoplasm from the mitochondria and causing apoptosis. Thus, BCL-2 inhibits apoptosis

44

Treatment for Follicular Lymphoma

Low-dose chemotherapy of rituximab

45

What is the target of rituximab

CD20 (rituximab is an anti-CD20 monoclonal antibody)

46

Name the condition: t(11;14)

Mantle Cell Lymphoma

47

What genes are affected by t(11;14)

Cyclin D1 on chromosome 11 translocates to IgH on chromosome 14, causing the overexpression of cyclin D1

48

What does Cyclin D1 do

Cyclin D1 promotes G1/S transition in the cell cycle

49

What condition is associated with chronic inflammatory states such as Hashimoto's Thyroiditis, Sjogren Syndrome, and H. Pylori Gastritis

Marginal Zone Lymphoma

50

Maltoma is a subtype of which lymphoma

Marginal Zone Lymphoma

51

Name the condition: t(8;14)

Burkitt Lymphoma

52

What genes are affected by t(8;14)

c-myc on chromosome 8 translocates to IgH on chromosome 14 causing the overexpression of c-myc oncogene

53

What does c-myc do

Nuclear regulator that promotes cell growth

54

Burkitt Lymphoma is associated with which infection

EBV infection

55

African form of Burkitt Lymphoma presents where

In the jaw

56

Sporadic form of Burkitt Lymphoma presents where

In the abdomen

57

Name this condition: Starry-sky appearance on histology

Burkitt Lymphoma

58

Name this cell: Large B cell with multilobulated nuclei and prominent nucleoli that is often CD15+ and CD30+

Reed-Sternberg cell

59

Name the condition: Reed-Sternberg cell

Hodgkin's Lymphoma

60

What do Reed-Sternberg cells do

They attract reactive lymphocytes, plasma cells, macrophages, and eosinophils

61

The most common subtype of Hodgkin's Lymphoma is

Nodular Sclerosis subtype Hodgkin's Lymphoma (70%) of cases

62

The classic presentation of Nodular Sclerosis subtype Hodgkin's Lymphoma

Enlarging neck or mediastinal lymphnode in a young adult female

63

What are Lacunar cells

Reed-Sternberg cells when found in the middle of open spaces. This is seen in Nodular Sclerosis subtype Hodgkin's Lymphoma

64

Which subtype of Hodgkin's lymphoma has the best prognosis

Lymphocyte-rich subtype Hodgkin's Lymphoma

65

Which subtype of Hodgkin's lymphoma is associated with abundant eosinophils (the eosinophils are being attracted by IL-5)

Mixed cellularity subtype Hodgkin's Lymphoma

66

Which subtype of Hodgkin's lymphoma has the worst prognosis

Lymphocyte-depleted subtype Hodgkin's Lymphoma

67

Which subtype of Hodgkin's lymphoma is seen in the elderly and in HIV+ individuals

Lymphocyte-depleted subtype Hodgkin's Lymphoma

68

The most common primary malignancy of bone is

Multiple Myeloma

69

What drives the production of plasma cells in Multiple Myeloma

High serum IL-6

70

Why does Multiple Myeloma cause bone lesions and increased risk of fracture

Neoplastic plasma cells activate osteoclasts

71

Osteoclast activating factor operates through which receptor

RANK receptor

72

M-spike on Serum Protein Electrophoresis (SPEP) is most likely due to what

An elevated level of monoclonal IgG or IgA

73

What is the most common cause of death in Multiple Myeloma

Infection due to loss of antigenic diversity

74

What is and what causes Rouleaux formation

It is the stacking of RBCs on blood smear see in Multiple Myeloma due to Increased serum protein that decreases the charge between RBCs

75

What causes Primary AL Amyloidosis

Free light chain produced in Multiple Myeloma enters the blood and forms amyloid deposits

76

Free light chain in the urine is called

Bence-Jones Proteins

77

What happens when free light chain deposits in the kidney

Renal failure

78

Name this condition: The presence of an M-spike without lytic bone lesions, hypercalcemia, AL amyloid, or Bence-Jones Proteins

Monoclonal Gammopathy of Undetermined Significance (MGUS)

79

what percent of 70-year-old individuals of MGUS and how many progress to Multiple Myeloma each year

5% (course packet says 3%, pathoma says 5%) and 1% respectively

80

Name this condition: Generalized lymphadenopathy without lytic bone lesions; Increased serum IgM; Visual and neurologic deficits; Bleeding

Waldenstrom Macroglobulinemia

81

What is Waldenstrom Macroglobulinemia

B-cell lymphoma with monoclonal IgM production

82

What are Langerhan cells

Specialized dendritic cells derived from bone marrow monocytes, and found predominantly in the skin. They present antigen to na‹ve T-cells

83

Neoplastic proliferation of Langerhan cells is called what

Langerhan cell histiocytosis

84

What is characteristically seen on EM in Langerhan cell histiocytosis

Birbeck (tennis racket) granules

85

Langerhan cell histiocytosis cells are positive for which markers

CD1a and S100

86

Name this condition: Malignant proliferation of Langerhan cells classically presents with skin rash and cystic skeletal defects in an infant

Letterer-Siwe disease

87

Name this condition: Benign proliferation of Langerhan cells in bone. The classic presentation is pathologic fracture in an adolescent; skin is not involved. Biopsy shows Langerhan cells with mixed inflammatory cells, including eosinophils.

Eosinophilic granuloma

88

Name this condition: Malignant proliferation of lLangerhan cells that classically present with a scalp rash, lytic skull defects, diabetes insipidus, and exopthalmos in a child

Hand-Schuller-Christian disease

89

Most common leukemia of adults in the western world

CLL (Chronic Lymphocytic Leukemia)

90

The most common type of non-Hodgkin lymphoma among adults.

DLBCL (Diffuse Large B cell Lymphoma)

91

The most common form of indolent non-Hodgkins lymphoma in the U.S.

Follicular Lymphoma

92

HTLV-1 (Human T cell Lymphotrophic Virus-1) is associated with which conditions

HTLV-I-associated myelopathy, Strongyloides stercoralis hyper-infection, and Adult T-cell leukemia/lymphoma

93

Necrotizing Granulomatous histological pattern of lymph nodes indicates

TB or fungal infections

94

Non-necrotizing Granulomatous histological pattern of lymph nodes

sarcoid or foreign material

95

Stellate microabscesses histological pattern of lymph nodes indicates

Bartonella Henselae (Cat scratch), chlamydia, Yersinia, or other bacteria

96

Toxoplasmosis pattern histological pattern of lymph nodes indicates

Follicular hyperplasia, granulomas, or monocytoid B cells. Organisms not found in lymph node

97

EBV is associated with which lymphoma/leukemia

Burkitt lymphoma, 30%-40% of Hodgkin lymphoma, many B-cell lymphomas arising in the setting of T-cell immunodeficiency, and rare NK-cell lymphomas. In some cases, EBV latent membrane protein 1 (LMP1) activates the NF-kB pathway

98

HHV8 (KSHV) is associated with which lymphoma/leukemia

Kaposi Sarcoma and an unusual B-cell lymphoma that presents as a malignant effusion, often in the pleural cavity

99

H. Pylori is associated with which lymphoma/leukemia

Gastric MALT lymphoma

100

Smoking increases the incidence of which white blood cell disorder

Acute myeloid leukemia

101

Which broad class of lymphoma spreads in an orderly fashion

Hodgkin's Lymphoma

102

Lymphomas in children

most commonly Precursor B Acute Lymphoblastic Leukemia (B-ALL), Precursor T Acute Lymphoblastic Leukemia (T-ALL), and Acute Myeloid Leukemia (AML)

103

Lymphomas in young adults

Acute Myeloid Leukemia (AML) more than Acute lymphoblastic leukemia (ALL) and tend to have recurrent translocations with better prognosis, Anaplastic large cell lymphoma (ALK+), Diffuse large B cell lymphoma (DLBCL), Hodgkin?s lymphoma, Primary Mediastina

104

Lymphomas in Middle age

Acute Myeloid Leukemia (AML) more than Acute lymphoblastic leukemia (ALL), Diffuse large B cell lymphoma (DLBCL), Hodgkin?s lymphoma, Myeloproliferative neoplasms, some low grade lymphomas

105

Lymphomas in elderly

Myelodysplastic syndromes (MDS) and Acute Myeloid Leukemia (AML) with multilineage dysplasia, Multiple myeloma, Low grade lymphoma, Diffuse large B cell lymphoma (DLBCL), Myeloproliferative neoplasms

106

Bleeding from mucous membranes, visual disturbances due to retinopathy, and neurologic symptoms ranging from headache and vertigo to seizures and coma are all symptoms of what

Hyperviscosity Syndrome

107

mycosis fungoides (T-cell lymphoma whose primary manifestation is in the skin) with lymphadenopathy is called what

Sezary's disease

108

What are Birbeck granules

Rod shaped or "tennis-racket" cytoplasmic organelles with a central linear density and a striated appearance.

109

Birbeck granules are seen in what condition

They are a characteristic microscopic finding in Langerhans cell histiocytosis (Histiocytosis X), which is one of a group of rare conditions collectively known as histiocytosis

110

Prognosis of Mantle Cell Lymphoma

Poor. Not curable with conventional chemotherapy, and most patients eventually succumb to organ dysfunction caused by tumor infiltration. The blastoid variant and a "proliferative" expression profile are associated with shorter survivals.

111

Which genes are upregulated in Marginal Zone Lymphoma

BCL10 or MALT1, protein components of a signaling complex that activates NF-kB and promotes the growth and survival of B cells.

112

Name this condition: Often presents with massive splenomegaly due to red pulp expansion is the most common and sometimes the only abnormal physical finding

Hairy cell leukemia (CML can also cause red pulp expansion)

113

Name this cell: Large anaplastic cell, sometimes containing horseshoe-shaped nucleus and voluminous cytoplasm

Hallmark cells

114

In which condition are Hallmark cells seen

Anaplastic large cell lymphoma

115

Name the conditions: CD30+, CD15+ v.s. CD30+, CD15-

Hodgkin's Lymphoma v.s. Anaplastic large cell lymphoma

116

What are Auer rods and what are they seen in

Myeloperoxidase crystals that are only present in Myeloblasts (thus seen in Acute Myeloid Leukemia)

117

The ABL tyrosine kinase activity of BCR-Abl is elevated relative to wild-type ABL in what translocation

t(9;22) Translocation known as the Philadelphia chromosome

118

Splenomegaly; A reduction in the number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any combination; a compensatory proliferative response in the bone marrow; and the potential for correction of these abnormalities by splenectomy

Hypersplenism

119

+ and - prognostics of Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma

Poor prognosis: ZAP70+ and del(17p) - the p53 gene or del(11q22)

Good pronosis: ZAP70- and del(13q)

120

Mutations in Blimp1 gene cause what and are seen in what condition

Mutations prevent maturation to plasma cells and are seen in Diffuse Large B cell Lymphoma

121

Increased NFkB signaling seen in Diffuse B cell Lymphoma can result from what mutations

CARD11 mutations (increase signaling through the BCR pathway) and Myd88 mutations (increase signaling through the Toll-like receptor)

122

What changes protect tumor cells from anti-tumor immune response

  • B2-microglobulin mutations
  • PDL1 upregulation (frequent in thymic DLBCL)

123

What are the cytogenetics of Diffuse B cell Lymphoma

  • bcl6 gene mutations in 35% of cases
  • t(14;18) in 20% of cases
  • t(8;14) c-myc translocations in 10% of cases (difficult to cure)

124

Name this condition: Ki67 marker seen in 100% of cases

Burkitt Lymphoma

125

What is the median survival of patients with Multiple Myeloma

3 years with standard (old) treatment. New therapies such as bone marrow transplantation and proteosome inhibitors significantly prolong survival in many patients

126

The worst prognostic factor cytogenetically in Multiple Myeloma is what

13q deletion

127

Nearly all cases of Multiple Myeloma have direct or indirect deregulation of what

1 of the 3 Cyclin Ds (deregulation of Cyclin D1 has better prognosis)

128

Hyperviscosity Syndrome due to elevated IgM is seen in what condition

Waldenstrom's Macroglobulinemia (a Lymphoplasmacytic Lymphoma)

129

Activating mutations in MYD88 (an intermediate in the toll-like receptor signaling pathway) will result in what

Waldenstrom's Macroglobulinemia (a Lymphoplasmacytic Lymphoma)

130

CD5, which is normally expressed on T cells, is expressedn on B cells in what 2 conditions. How do you tell them apart

Mantle cell Lymphoma and Chronic Lymphocytic Leukemia. Mantle cell Lymphoma is CD23- whereas CLL is CD23+. Also Mantle Cell Lymphoma is a Cyclin D1 translocation t(11;14)

131

t(11;18) and t(14;18) causes upregulation of what gene on chromosome 18. What is the result and what condition is this seen in

Upregulation of the MLT1 gene (bcl10 can also be mutated in this condition) leads to activation of NF-kB and is seen in Marginal cell Lymphoma

132

What is the prognosis of Hairy Cell Leukemia

Prognosis is good. Distinct therapy: 90% respond to 2-chlorodeoxyadenosine (2-CDA)

133

CD25, CD11c, and CD103 markers are seen on the surface of cells in what condition

Hairy Cell Leukemia

134

t(2;5)

NPM/ALK translocation seen in ALK+ Anaplastic Large Cell Lymphoma. Common in younger patients with a relatively good prognosis after chemotherapy

135

Prognosis of ALK- Anaplastic Large Cell Lymphoma

Poor prognosis and seen in older patients

136

What causes the hypercalcemia in Adult T-cell Leukemia/Lymphoma

Osteoclast activation (not from marrow infiltration as is the case with plasma cell myeloma)

137

Which subtype of Hodgkin's Lymphoma has a bimodal age distribution, more extensive disease with B symptoms (Fever and night sweats), and association with EBV in 70% of cases

Mixed Cellularity type Hodgkin's Lymphoma

138

What are popcorn cells and what condition are they seen in

Small cells, with a very lobulated nuclei, and small nucleoli. They are a Reed-Sternberg cell variant seen in (Nodular) Lymphocyte Predominant Hodgkin's Lymphoma 

139

What is the prognosis of (Nodular) Lymphocyte Predominant Hodgkin's Lymphoma and is there any association with EBV 

It tends to relapse but prognosis is excellent. No association with EBV

140

How does treatment for (Nodular) Lymphocyte Predominant Hodgkin's Lymphoma differ from classical Hodgkin's Lymphoma

Treatment of (Nodular) Lymphocyte Predominant Hodgkin's Lymphoma is usually much less aggressive and includes the use of rituximab (Anti-CD20 antibody) becuase these cells are CD20+ unlike other types of Hodgkin's Lymphomas

141

What is DiGeorge Syndrome

Failure of the development of the 3rd and 4th pharyngeal pouches leading to thymic hypoplasia or aplasia and a severe lack of cell-mediated immunity. Also there is a failure of parathyroid development, developmental defects of the heart and great vessels

142

How does DiGeorge Syndrome present

Tetany, CHF or cyanosis (heart defects, 22q11 deletion syndrome), Infections (lack of cell-mediated immunity)

143

Name this condition: Inv(16)

Acute Myelomonocytic Leukemia with Eosinophilia

144

What gene fusion causes Acute Myelomonocytic Leukemia with Eosinophilia and what is the prognosis 
 

Inv(16) which causes CBF-beta/MYH11 gene fusion. It has a relatively good prognosis

145

What is the prognosis of Therapy-Related AML

Very poor prognosis

146

What fusion protein is caused by t(8;21)

AML1/ETO fusion protein seen in some AMLs with a good prognosis

147

What stain is used to detect iron and what color does iron appear

Prussian blue stain. The iron is blue

148

What is the prognosis of the Myelodysplastic Syndromes

Poor prognosis, generally months to years

149

What is different about the megakaryocytes in Essential Thrombocytosis vs Myelofibrosis

The megakaryocytes show more pleomorphism and hyperchromaticity in Myelofibrosis

150

What are the mechanisms to increase circulating neutrophils in response to infection

  • Neutrophil demargination
  • Increased release of neutrophil reserves in marrow
  • Increased neutrophil production by the marrow

151

FLT-3 and c-kit mutations indicate what prognosis

Worse prognosis

152

This special category of low grade myelodysplastic syndrome occurs in older females and presents with anemia and increased platelets

5q Minus Syndrome

153

This condition results from implantation of splenic tissue on serosal tissue within the abdomen after traumatic splenic rupture

Splenosis

154

This zoonotic protozoan infection produces lymphadenopathy characterized by follicular hyperplasia, monocytoid B cell hyperplasia, and non-necrotizing granulomas

Toxoplasmosis

155

This lab technique, performed on live cells, allows for the assessment of light chain restriction on the surface of B cells

Flow cytometry

156

Patients with this ancestry have a high incidence of plasma cell myeloma

African Ancestry

157

Patients with this type of lymphoma are frequently found to have lymphomatous polyps in the GI tract called lymphomatoid polyposis

Mantle cell lymphoma