LymphomaLeukemia Flashcards
(159 cards)
1
Q
Name the condition: t(12;21)
A
B-ALL with better prognosis
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
1
Q
Name the cell type: CD2-8 but not CD10
A
T lymphoblasts (T-ALL)
2
Q
Name the cell type: CD10, CD19, CD20
A
B lymphoblasts (B-ALL)
3
Q
Name the condition: Presents as a thymic mass in teenagers
A
T-ALL
4
Q
Name the cell type: tDt+
A
Lymphoblasts (as in B-ALL and T-ALL)
5
Q
Name the cell type: Myeloperoxidase+ (Auer Rods)
A
Myeloblasts (as in AML), and promyelocytes (APL)
6
Q
Name the condition: t(15;17)
A
Acute Promyelocytic Leukemia
7
Q
Name the disrupted gene: t(15;17)
A
Retinoic Acid Receptor causin promyelocytes to accumulate
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.
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
10
Q
Swelling of the gums is a symptoms seen in which condition
A
Acute Monocytic Leukemia (AMoL)
11
Q
This condition has an association with Down Syndrome before the age of 5
A
Acute Megakaryoblastic Leukemia (AMKL)
12
Q
This condition has an association with Down Syndrome after the age of 5
A
Acute Lymphoblastic Leukemia (ALL)
13
Q
Cytopenia with a hypercellular bone marrow of <20% blasts is characteristic of what
A
Myelodysplastic syndrome
14
Q
What do people with Myelodysplastic Syndrome usually die of
A
Infection or bleeding (due to decreased white cells and platelets)
15
Q
CD5+ B cells are seen in which condition
A
Chronic Lymphocytic Leukemia (CLL)
16
Q
Name the condition: Smudge Cells
A
Chronic Lymphocytic Leukemia (CLL)
17
Q
Name the condition: Tartrate-resistant acid phosphatase+ (TRAP) cells
A
Hairy Cell Leukemia
18
Q
Name the condition: Massive splenomegaly due to red pulp expansion
A
Hairy Cell Leukemia
19
Q
Name the condition: Lytic bone lesions, hypercalcemia, and a rash
A
Adult T-cell Leukemia/Lymphoma (ATL)
20
Q
Name the condition: Lytic bone lesions and hypercalcemia without a rash
A
Multiple Myeloma
21
Q
Name the condition: Aggregates of neoplastic T-cells in the epidermis (Pautrier Microabscesses)
A
Mycosis Fungiodes
22
Q
Name the involved genes: t(9;22)
A
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
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Treatment for Follicular Lymphoma
Low-dose chemotherapy of rituximab
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What is the target of rituximab
CD20 (rituximab is an anti-CD20 monoclonal antibody)
46
Name the condition: t(11;14)
Mantle Cell Lymphoma
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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
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What does c-myc do
Nuclear regulator that promotes cell growth
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Burkitt Lymphoma is associated with which infection
EBV infection
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African form of Burkitt Lymphoma presents where
In the jaw
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Sporadic form of Burkitt Lymphoma presents where
In the abdomen
57
Name this condition: Starry-sky appearance on histology
Burkitt Lymphoma
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Name this cell: Large B cell with multilobulated nuclei and prominent nucleoli that is often CD15+ and CD30+
Reed-Sternberg cell
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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
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The classic presentation of Nodular Sclerosis subtype Hodgkin's Lymphoma
Enlarging neck or mediastinal lymphnode in a young adult female
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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
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Which subtype of Hodgkin's lymphoma has the best prognosis
Lymphocyte-rich subtype Hodgkin's Lymphoma
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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
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Which subtype of Hodgkin's lymphoma has the worst prognosis
Lymphocyte-depleted subtype Hodgkin's Lymphoma
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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
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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
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Osteoclast activating factor operates through which receptor
RANK receptor
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M-spike on Serum Protein Electrophoresis (SPEP) is most likely due to what
An elevated level of monoclonal IgG or IgA
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What is the most common cause of death in Multiple Myeloma
Infection due to loss of antigenic diversity
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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
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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
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What happens when free light chain deposits in the kidney
Renal failure
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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)
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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
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Langerhan cell histiocytosis cells are positive for which markers
CD1a and S100
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Name this condition: Malignant proliferation of Langerhan cells classically presents with skin rash and cystic skeletal defects in an infant
Letterer-Siwe disease
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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
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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
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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)
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What changes protect tumor cells from anti-tumor immune response
* B2-microglobulin mutations
* PDL1 upregulation (frequent in thymic DLBCL)
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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
