Leukemia & Lymphoma Flashcards
(103 cards)
1
Q
What is Leukemia?
A
Lymphoid or myeloid neoplasms w/ widespread involvement of BM
2
Q
Where are the Leukemia tumors cells found?
A
Peripheral blood
3
Q
What are Lymphomas?
A
Discrete tumor masses arising from LN’s
4
Q
What is a Leukemoid reaction?
A
Acute inflammatory response to infection
5
Q
What are the lab features of Leukemoid reaction?
A
- Inc WBC count
- Inc neutrophils & neutrophil precursors such as band cells (left shift)
- Inc leukocyte alkaline phosphatase
6
Q
What are the features of Hodgkin’s lymphoma?
A
- Localized, single group of nodes
- Extranodal rare
- Contiguous spread
7
Q
What is the strongest predictor of prognosis in Hodgkin’s lymphoma?
A
Contiguous spread
8
Q
What is Hodgkin’s lymphoma characterized by?
A
Reed-Sternberg cells
9
Q
What is the age group of pts w/ Hodgkin’s lymphoma?
A
Bimodal distribution: young adulthood & >55yo
10
Q
Who is Hodgkin’s lymphoma MC in?
A
MC in men except for nodular sclerosing type
11
Q
What are 50% of Hodgkin’s lymphoma assoc w/?
A
EBV
12
Q
What are the constituitonal (“B”) signs/sx of Hodgkin’s lymphoma?
A
- Low-grade fever
- Night sweats
- Wt loss
13
Q
What are the features of Non-Hodgkin’s lymphoma?
A
- Multiple, peripheral nodes
- Extranodal involvement common
- Noncontiguous spread
14
Q
What do the majority of Non-Hodgkin lymphomas involve?
A
B cells (except those of lymphoblastic T cell origin)
15
Q
What is the peak incidence for certain subtypes of Non-Hodgkin lymphomas?
A
20-40 years of age
16
Q
What can Non-Hodgkin’s lymphoma be assoc w/?
A
HIV & immunosuppression
17
Q
What are Reed-Sternberg cells?
A
Distinctive tumor giant cells seen in Hodgkin’s dz
18
Q
What is the histo of Reed-Sternberg cells?
A
Binucelate or bilobed w/ the 2 halves as mirror images (“owl’s eyes”)
19
Q
What is the origin of Reed-Sternberg cells?
A
CD30+ & CD15+ B-cell origin
20
Q
What is assoc w/ a better prognosis in Hodgkin’s dz?
A
Strong stromal or lymphocytic reaction against RS cells
21
Q
What is the MC form of Hodgkin’s dz w/ the best prognosis?
A
Nodular sclerosing form
(Affects women & men equally)
22
Q
Which forms of Hodgkin’s dz have poor prognosis?
A
Lymphocyte mixed or depleted forms
23
Q
Who does Burkitt’s lymphoma occur in?
A
Adolescents or young adults
24
Q
What are the genetics involved in Burkitt’s lymphoma?
A
t(8;14)–translocation of c-myc (8) & heavy-chain Ig (14)
25
What is the histo of Burkitt's lymphoma?
"Starry sky" appearance, sheets of lymphocytes w/ interspersed macrophages
26
What is Burkitt's lymphoma assoc w/?
EBV
27
What lesions are assoc w/ Burkitt's lymphoma?
* Jaw lesion in endemic form in Africa
* Pelvis or abdomen in sporadic form
28
Who does Diffuse large B-cell lymphoma occur in?
Usually older adults, but 20% in children
29
What is the MC adult Non-Hodgkin lymphoma?
Diffuse large B-cell lymphoma
30
What is the origin of Diffuse large B-cell lymphoma in 20% of cases?
Mature T cell
31
Who gets Mantle cell lymphoma?
Older males
32
What genetics are involved in Mantle cell lymphoma?
t(11;14)--translocation of cyclin D1 (11) & heavy-chain Ig (14)
33
What is the prognosis of Mantle cell lymphoma?
Poor
34
What is the cell surface maker in Mantle cell lymphoma?
CD5+
35
Who gets Follicular lymphoma?
Adults
36
What genetics are involved in Follicular lymphoma?
t(14;18)--translocation of heavy-chain Ig (14) & *bcl*-2 (18)
37
What are the features of Follicular lymphoma?
* Difficult to cure
* Indolent course
* *bcl*-2 inhibits apoptosis
38
What genetics are involved in Adult T-cell lymphoma?
HTLV-1
39
What do pts w/ Adult T-cell lymphoma present w/?
Cutaneous lesions
40
What populations are especially affected by Adult T-cell lymphoma?
Japan, West Africa & the Caribbean
41
What is Multiple Myeloma?
Monoclonal plasma cell cancer taht arises in the marrow & produces large amounts of IgG (55%) or IgA (25%)
42
What is the histo appearance of Multiple Myeloma?
"Fried egg" appearance
43
What is the MC 1° tumor arising w/in bone in the elderly?
Multiple myeloma
| (\>40-50yo)
44
45
What is Multiple myeloma assoc w/?
* Inc susceptibility to infection
* Primary amyloidosis (AL)
* Punched-out lytic bone lesions on x-ray
* M spike on protein electrophoresis
* Bence Jones protein
* Rouleux formation
46
What is Bence Jones protein?
Ig light chains in urine
47
What is Rouleaux formation?
RBCs stacked like poker chips in blood smear
48
What is the histo of Multiple myeloma?
Numerous plasma cells w/ "clock face" chromatin & intracytoplasmic inclusions containing Ig
49
What is Waldenström's macrobloulinemia?
* M spike= IgM
* Hyperviscosity sx
* No lytic bone lesions
50
What is Monoclonal gammopathy of undetermined significance (MGUS)?
Monoclonal expansion of plasma cells w/ M spike
51
What is the asx precursor to Multiple myeloma?
MGUS
Develop multiple myeloma at a rate of 1-2%/year
52
What are the features of Leukemias?
Unregulated growth of leukocytes in BM→ Inc or dec in # of ciculating leukocytes in blood & marrow failure→ anemia, infections & hemorrhage
53
Where can leukemic cells infiltrate?
* Liver
* Spleen
* LN's
54
What is the age group for Acute lymphocytic leukemia/lymphoma (ALL)?
\<15yo
55
How can T-cell ALL present?
Mediastinal mass (leukemic infiltration of the thymus)
56
What is increased in the peripheral blood & BM in ALL?
Lymphoblasts
57
What are the cell surface makers of ALL?
* TdT+ (maker pre-T & pre-B cells)
* CALLA+
58
Which leukemia is most responsive to chemo?
ALL
59
Where can ALL spread?
CNS & testes
60
Which translocation is assoc w/ a better prognosis in ALL?
t(12;21)
61
What is the age group of Small lymphocytic lyphoma (SLL)/ chronic lymphocytic leukemia (CLL)?
\>60yo
62
What are the sx of SLL/CLL?
Often asx
63
What is the histo of SLL/CLL?
Smudge cells in peripheral blood smear AI hemolytic anemia
64
How does CLL differ from SLL?
CLL has inc peripheral blood lymphocytosis or BM involvement
65
What is the age group of Hairy cell leukemia?
Adults
66
What is Hairy cell leukemia?
B cell tumor in the elderly
67
What is the histo of Hairy cell leukemia?
* Cells have filamentous, hair-like projections
* Stains TRAP (tartrate-resistant acid phosphatase) positive
68
What is the tx for Hairy cell leukemia?
Cladribine, an adenosine analog
69
What is the age group of Acute myelogenous leukemia (AML)?
Median onset 65 years
70
What is the histo of AML?
* Auer rods
* Inc circulating myeloblasts on peripheral smear
71
What is the translocation in M3 AML subtype?
t(15;17)
72
What is the tx for M3 AML subtype?
all-*trans* retinoic acid (vitamin A)
73
What is a common presentation for M3 AML?
DIC
74
What age group has the highest incidence of Chronic myelogenous leukemia (CML)?
30-60yo
75
What is CML defined by?
Philadelphia chr (t[9,22], *bcr-abl*)
76
What are the features of CML?
* Myeloid stem cell prolif
* Inc neutrophils, metamyelocytes, basophils
* Splenomegaly
77
What can CML accelerate or transform to?
AML or ALL ("blast crisis")
78
What can immature granulocytes in CML result in?
Very low leukocyte alkaline phosphatase
79
What does CML respond to?
Imatinib (a small-molecule inhibitor of the *bcr-abl* tyrosine kinase)
80
What are Auer bodies (rods)?
Peroxidase-positive cytoplasmic inclusions in granulocytes & myeloblasts
81
What are Auer rods commonly seen in?
Acute promyelocytic leukemia (M3)
82
What can the tx of AML M3 can release?
Auer rods→DIC
83
What is Langerhans cell histiocytosis?
Prolif disorders of dendritic (Langerhans) cells from monocyte lineage
84
How does Langerhans cell histiocytosis present?
In a child as lytic bone lesions & skin rash
85
What are the cells like in Langerhans cell histiocytosis?
Functionally lymphocytes via Ag presentation
86
Langerhans cell histiocytosis cells express ___ & \_\_\_.
S-100 & CD1a
87
What is characteristic of Langerhans cell histiocytosis?
Birbeck granules ("tennis rackets" on EM)
88
What are the lab features of Polycythemia vera?
* Inc RBCs, WBCs & platelets
* Philadelphia chr -
* JAK2 mutations +
89
What are the lab features of Essential thrombocytosis?
* Inc platelets
* Philadelphia chr -
* JAK2 mutations + (30-50%)
90
What are the lab features of Myelofibrosis?
* Dec RBCs
* Philadelphia chr -
* JAK2 mutations +
91
What are the lab features of CML?
* Dec RBCs
* Inc WBCs
* Inc platelets
* Philadelphia chr +
* JAK2 mutations -
92
What is JAK2 involved in?
Hematopoietic GF signaling
93
What is Polycythemia vera?
ABN clone of hematopoietic stem cells w/ constitutively active JAK2 receptors, proliferate w/o EPO stim
94
What does Polycythemia vera present as?
Intense itching after hot shower
95
What is Essential thrombocytosis?
Similar to polycythemia vera, but specific for megakaryocytes
96
What is Myelofibrosis?
Fibrotic obliteration of BM
Tear drop cell
97
What is the cause of CML?
*bcr-abl* transformation l/t inc cell division & inhibition of apoptosis
98
What are the lab features of Relative Polycythemia?
Dec plasma volume
99
What are the lab features of Appropriate Absolute Polycythemia?
* Inc RBC mass
* Dec O2 saturation
100
Which diseases are assoc w/ Appropriate absolute polycythemia?
* Lung dx
* Congenital heart dz
* High altitude
101
What are the lab features of Inappropriate absolute polycythemia?
Inc RBC mass
102
Which diseases are w/ Inappropriate absolute polycythemia?
* Renall cell CA
* Wilm's tumor
* Cyst
* Hepatocellular CA
* Hydronephrosis
* Due to ectopic erythropoietin
103
What are the lab features for Polycythemia vera?
Inc plasma volume & RBC mass
