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LymphomaLeukemia Flashcards

1
Q

Name the condition: t(12;21)

A

B-ALL with better prognosis

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1
Q

Name the condition: t(9;22)

A

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

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1
Q

Name the cell type: CD2-8 but not CD10

A

T lymphoblasts (T-ALL)

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2
Q

Name the cell type: CD10, CD19, CD20

A

B lymphoblasts (B-ALL)

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3
Q

Name the condition: Presents as a thymic mass in teenagers

A

T-ALL

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4
Q

Name the cell type: tDt+

A

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

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5
Q

Name the cell type: Myeloperoxidase+ (Auer Rods)

A

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

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6
Q

Name the condition: t(15;17)

A

Acute Promyelocytic Leukemia

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7
Q

Name the disrupted gene: t(15;17)

A

Retinoic Acid Receptor causin promyelocytes to accumulate

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8
Q

Auer rods can activate what

A

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

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9
Q

What is the treatment for Acute Promyelocytic Leukemia

A

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

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10
Q

Swelling of the gums is a symptoms seen in which condition

A

Acute Monocytic Leukemia (AMoL)

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11
Q

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

A

Acute Megakaryoblastic Leukemia (AMKL)

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12
Q

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

A

Acute Lymphoblastic Leukemia (ALL)

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13
Q

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

A

Myelodysplastic syndrome

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14
Q

What do people with Myelodysplastic Syndrome usually die of

A

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

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15
Q

CD5+ B cells are seen in which condition

A

Chronic Lymphocytic Leukemia (CLL)

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16
Q

Name the condition: Smudge Cells

A

Chronic Lymphocytic Leukemia (CLL)

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17
Q

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

A

Hairy Cell Leukemia

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18
Q

Name the condition: Massive splenomegaly due to red pulp expansion

A

Hairy Cell Leukemia

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19
Q

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

A

Adult T-cell Leukemia/Lymphoma (ATL)

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20
Q

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

A

Multiple Myeloma

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21
Q

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

A

Mycosis Fungiodes

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22
Q

Name the involved genes: t(9;22)

A

BCR-ABL fusion with increased tyrosine kinase activity

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23
Q

Treatment for BCR-ABL fusion

A

Imatinib which blocks tyrosine kinase activity

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24
Q

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

A

Normal response to infection

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25
Q

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

A

Chronic Myelogenous Leukemia

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26
Q

Granulocytosis with no increase in basophils indicates what

A

Normal response to infection

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27
Q

Granulocytosis with and increase in basophils indicates what

A

Chronic Myelogenous Leukemia

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28
Q

What is Polycythemia Vera

A

Neoplastic proliferation of mature myeloid cells, but especially RBCs

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29
Q

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

A

Polycythemia Vera

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30
Q

Polycythemia Vera is caused by what mutation

A

JAK2 kinase mutation

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31
Q

Polycythemia with increased EPO and low SaO2 indicates what

A

Reactive polycythemia, as in lung disease

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32
Q

Polycythemia with decreased EPO and normal SaO2 indicates what

A

Polycythemia Vera

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33
Q

Polycythemia with increased EPO and normal SaO2 indicates what

A

Ectopic EPO, as in renal cell carcinoma

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34
Q

What is Essential Thrombocythemia

A

Neoplastic proliferation of mature myeloid cells, but especially platelets

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35
Q

Essential Thrombocythemia is caused by what mutation

A

JAK2 kinase mutation

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36
Q

What besides Essential Thrombocythemia can cause excess platelets in the blood

A

Iron deficiency anemia

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37
Q

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

A

Essential Thrombocythemia

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38
Q

What is Myelofibrosis

A

Neoplastic proliferation of mature myeloid cells, but especially megakaryocytes

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39
Q

Myelofibrosis is caused by what mutation

A

JAK2 kinase mutation

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40
Q

Teare drop cells are commonly seen in which myeloproliferative disorder

A

Myelofibrosis

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41
Q

Name the condition: t(14;18)

A

Follicular Lymphoma

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42
Q

What genes are affected by t(14;18)

A

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.

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43
Q

What does BCL-2 do

A

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

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44
Q

Treatment for Follicular Lymphoma

A

Low-dose chemotherapy of rituximab

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45
Q

What is the target of rituximab

A

CD20 (rituximab is an anti-CD20 monoclonal antibody)

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46
Q

Name the condition: t(11;14)

A

Mantle Cell Lymphoma

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47
Q

What genes are affected by t(11;14)

A

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

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48
Q

What does Cyclin D1 do

A

Cyclin D1 promotes G1/S transition in the cell cycle

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49
Q

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

A

Marginal Zone Lymphoma

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50
Q

Maltoma is a subtype of which lymphoma

A

Marginal Zone Lymphoma

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51
Q

Name the condition: t(8;14)

A

Burkitt Lymphoma

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52
Q

What genes are affected by t(8;14)

A

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

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53
Q

What does c-myc do

A

Nuclear regulator that promotes cell growth

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54
Q

Burkitt Lymphoma is associated with which infection

A

EBV infection

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55
Q

African form of Burkitt Lymphoma presents where

A

In the jaw

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56
Q

Sporadic form of Burkitt Lymphoma presents where

A

In the abdomen

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57
Q

Name this condition: Starry-sky appearance on histology

A

Burkitt Lymphoma

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58
Q

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

A

Reed-Sternberg cell

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59
Q

Name the condition: Reed-Sternberg cell

A

Hodgkin’s Lymphoma

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60
Q

What do Reed-Sternberg cells do

A

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

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61
Q

The most common subtype of Hodgkin’s Lymphoma is

A

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

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62
Q

The classic presentation of Nodular Sclerosis subtype Hodgkin’s Lymphoma

A

Enlarging neck or mediastinal lymphnode in a young adult female

63
Q

What are Lacunar cells

A

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

64
Q

Which subtype of Hodgkin’s lymphoma has the best prognosis

A

Lymphocyte-rich subtype Hodgkin’s Lymphoma

65
Q

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

A

Mixed cellularity subtype Hodgkin’s Lymphoma

66
Q

Which subtype of Hodgkin’s lymphoma has the worst prognosis

A

Lymphocyte-depleted subtype Hodgkin’s Lymphoma

67
Q

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

A

Lymphocyte-depleted subtype Hodgkin’s Lymphoma

68
Q

The most common primary malignancy of bone is

A

Multiple Myeloma

69
Q

What drives the production of plasma cells in Multiple Myeloma

A

High serum IL-6

70
Q

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

A

Neoplastic plasma cells activate osteoclasts

71
Q

Osteoclast activating factor operates through which receptor

A

RANK receptor

72
Q

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

A

An elevated level of monoclonal IgG or IgA

73
Q

What is the most common cause of death in Multiple Myeloma

A

Infection due to loss of antigenic diversity

74
Q

What is and what causes Rouleaux formation

A

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
Q

What causes Primary AL Amyloidosis

A

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

76
Q

Free light chain in the urine is called

A

Bence-Jones Proteins

77
Q

What happens when free light chain deposits in the kidney

A

Renal failure

78
Q

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

A

Monoclonal Gammopathy of Undetermined Significance (MGUS)

79
Q

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

A

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

80
Q

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

A

Waldenstrom Macroglobulinemia

81
Q

What is Waldenstrom Macroglobulinemia

A

B-cell lymphoma with monoclonal IgM production

82
Q

What are Langerhan cells

A

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

83
Q

Neoplastic proliferation of Langerhan cells is called what

A

Langerhan cell histiocytosis

84
Q

What is characteristically seen on EM in Langerhan cell histiocytosis

A

Birbeck (tennis racket) granules

85
Q

Langerhan cell histiocytosis cells are positive for which markers

A

CD1a and S100

86
Q

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

A

Letterer-Siwe disease

87
Q

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.

A

Eosinophilic granuloma

88
Q

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

A

Hand-Schuller-Christian disease

89
Q

Most common leukemia of adults in the western world

A

CLL (Chronic Lymphocytic Leukemia)

90
Q

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

A

DLBCL (Diffuse Large B cell Lymphoma)

91
Q

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

A

Follicular Lymphoma

92
Q

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

A

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

93
Q

Necrotizing Granulomatous histological pattern of lymph nodes indicates

A

TB or fungal infections

94
Q

Non-necrotizing Granulomatous histological pattern of lymph nodes

A

sarcoid or foreign material

95
Q

Stellate microabscesses histological pattern of lymph nodes indicates

A

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

96
Q

Toxoplasmosis pattern histological pattern of lymph nodes indicates

A

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

97
Q

EBV is associated with which lymphoma/leukemia

A

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
Q

HHV8 (KSHV) is associated with which lymphoma/leukemia

A

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

99
Q

H. Pylori is associated with which lymphoma/leukemia

A

Gastric MALT lymphoma

100
Q

Smoking increases the incidence of which white blood cell disorder

A

Acute myeloid leukemia

101
Q

Which broad class of lymphoma spreads in an orderly fashion

A

Hodgkin’s Lymphoma

102
Q

Lymphomas in children

A

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

103
Q

Lymphomas in young adults

A

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
Q

Lymphomas in Middle age

A

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
Q

Lymphomas in elderly

A

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
Q

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

A

Hyperviscosity Syndrome

107
Q

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

A

Sezary’s disease

108
Q

What are Birbeck granules

A

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

109
Q

Birbeck granules are seen in what condition

A

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
Q

Prognosis of Mantle Cell Lymphoma

A

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
Q

Which genes are upregulated in Marginal Zone Lymphoma

A

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

112
Q

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

A

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

113
Q

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

A

Hallmark cells

114
Q

In which condition are Hallmark cells seen

A

Anaplastic large cell lymphoma

115
Q

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

A

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

116
Q

What are Auer rods and what are they seen in

A

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

117
Q

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

A

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

118
Q

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

A

Hypersplenism

119
Q

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

A

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

Good pronosis: ZAP70- and del(13q)

120
Q

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

A

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

121
Q

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

A

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

122
Q

What changes protect tumor cells from anti-tumor immune response

A
  • B2-microglobulin mutations
  • PDL1 upregulation (frequent in thymic DLBCL)
123
Q

What are the cytogenetics of Diffuse B cell Lymphoma

A
  • 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
Q

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

A

Burkitt Lymphoma

125
Q

What is the median survival of patients with Multiple Myeloma

A

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

126
Q

The worst prognostic factor cytogenetically in Multiple Myeloma is what

A

13q deletion

127
Q

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

A

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

128
Q

Hyperviscosity Syndrome due to elevated IgM is seen in what condition

A

Waldenstrom’s Macroglobulinemia (a Lymphoplasmacytic Lymphoma)

129
Q

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

A

Waldenstrom’s Macroglobulinemia (a Lymphoplasmacytic Lymphoma)

130
Q

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

A

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
Q

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

A

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
Q

What is the prognosis of Hairy Cell Leukemia

A

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

133
Q

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

A

Hairy Cell Leukemia

134
Q

t(2;5)

A

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

135
Q

Prognosis of ALK- Anaplastic Large Cell Lymphoma

A

Poor prognosis and seen in older patients

136
Q

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

A

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

137
Q

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

A

Mixed Cellularity type Hodgkin’s Lymphoma

138
Q

What are popcorn cells and what condition are they seen in

A

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
Q

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

A

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

140
Q

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

A

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
Q

What is DiGeorge Syndrome

A

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
Q

How does DiGeorge Syndrome present

A

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

143
Q

Name this condition: Inv(16)

A

Acute Myelomonocytic Leukemia with Eosinophilia

144
Q

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

A

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

145
Q

What is the prognosis of Therapy-Related AML

A

Very poor prognosis

146
Q

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

A

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

147
Q

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

A

Prussian blue stain. The iron is blue

148
Q

What is the prognosis of the Myelodysplastic Syndromes

A

Poor prognosis, generally months to years

149
Q

What is different about the megakaryocytes in Essential Thrombocytosis vs Myelofibrosis

A

The megakaryocytes show more pleomorphism and hyperchromaticity in Myelofibrosis

150
Q

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

A
  • Neutrophil demargination
  • Increased release of neutrophil reserves in marrow
  • Increased neutrophil production by the marrow
151
Q

FLT-3 and c-kit mutations indicate what prognosis

A

Worse prognosis

152
Q

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

A

5q Minus Syndrome

153
Q

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

A

Splenosis

154
Q

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

A

Toxoplasmosis

155
Q

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

A

Flow cytometry

156
Q

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

A

African Ancestry

157
Q

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

A

Mantle cell lymphoma

Heme (7 decks)

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