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Leukemia & Lymphoma Flashcards

1
Q

What is Leukemia?

A

Lymphoid or myeloid neoplasms w/ widespread involvement of BM

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2
Q

Where are the Leukemia tumors cells found?

A

Peripheral blood

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3
Q

What are Lymphomas?

A

Discrete tumor masses arising from LN’s

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4
Q

What is a Leukemoid reaction?

A

Acute inflammatory response to infection

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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
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6
Q

What are the features of Hodgkin’s lymphoma?

A
  • Localized, single group of nodes
  • Extranodal rare
  • Contiguous spread
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7
Q

What is the strongest predictor of prognosis in Hodgkin’s lymphoma?

A

Contiguous spread

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8
Q

What is Hodgkin’s lymphoma characterized by?

A

Reed-Sternberg cells

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9
Q

What is the age group of pts w/ Hodgkin’s lymphoma?

A

Bimodal distribution: young adulthood & >55yo

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10
Q

Who is Hodgkin’s lymphoma MC in?

A

MC in men except for nodular sclerosing type

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11
Q

What are 50% of Hodgkin’s lymphoma assoc w/?

A

EBV

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12
Q

What are the constituitonal (“B”) signs/sx of Hodgkin’s lymphoma?

A
  • Low-grade fever
  • Night sweats
  • Wt loss
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13
Q

What are the features of Non-Hodgkin’s lymphoma?

A
  • Multiple, peripheral nodes
  • Extranodal involvement common
  • Noncontiguous spread
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14
Q

What do the majority of Non-Hodgkin lymphomas involve?

A

B cells (except those of lymphoblastic T cell origin)

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15
Q

What is the peak incidence for certain subtypes of Non-Hodgkin lymphomas?

A

20-40 years of age

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16
Q

What can Non-Hodgkin’s lymphoma be assoc w/?

A

HIV & immunosuppression

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17
Q

What are Reed-Sternberg cells?

A

Distinctive tumor giant cells seen in Hodgkin’s dz

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18
Q

What is the histo of Reed-Sternberg cells?

A

Binucelate or bilobed w/ the 2 halves as mirror images (“owl’s eyes”)

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19
Q

What is the origin of Reed-Sternberg cells?

A

CD30+ & CD15+ B-cell origin

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20
Q

What is assoc w/ a better prognosis in Hodgkin’s dz?

A

Strong stromal or lymphocytic reaction against RS cells

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21
Q

What is the MC form of Hodgkin’s dz w/ the best prognosis?

A

Nodular sclerosing form

(Affects women & men equally)

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22
Q

Which forms of Hodgkin’s dz have poor prognosis?

A

Lymphocyte mixed or depleted forms

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23
Q

Who does Burkitt’s lymphoma occur in?

A

Adolescents or young adults

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24
Q

What are the genetics involved in Burkitt’s lymphoma?

A

t(8;14)–translocation of c-myc (8) & heavy-chain Ig (14)

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25
Q

What is the histo of Burkitt’s lymphoma?

A

“Starry sky” appearance, sheets of lymphocytes w/ interspersed macrophages

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26
Q

What is Burkitt’s lymphoma assoc w/?

A

EBV

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27
Q

What lesions are assoc w/ Burkitt’s lymphoma?

A
  • Jaw lesion in endemic form in Africa
  • Pelvis or abdomen in sporadic form
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28
Q

Who does Diffuse large B-cell lymphoma occur in?

A

Usually older adults, but 20% in children

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29
Q

What is the MC adult Non-Hodgkin lymphoma?

A

Diffuse large B-cell lymphoma

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30
Q

What is the origin of Diffuse large B-cell lymphoma in 20% of cases?

A

Mature T cell

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31
Q

Who gets Mantle cell lymphoma?

A

Older males

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32
Q

What genetics are involved in Mantle cell lymphoma?

A

t(11;14)–translocation of cyclin D1 (11) & heavy-chain Ig (14)

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33
Q

What is the prognosis of Mantle cell lymphoma?

A

Poor

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34
Q

What is the cell surface maker in Mantle cell lymphoma?

A

CD5+

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35
Q

Who gets Follicular lymphoma?

A

Adults

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36
Q

What genetics are involved in Follicular lymphoma?

A

t(14;18)–translocation of heavy-chain Ig (14) & bcl-2 (18)

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37
Q

What are the features of Follicular lymphoma?

A
  • Difficult to cure
  • Indolent course
  • bcl-2 inhibits apoptosis
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38
Q

What genetics are involved in Adult T-cell lymphoma?

A

HTLV-1

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39
Q

What do pts w/ Adult T-cell lymphoma present w/?

A

Cutaneous lesions

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40
Q

What populations are especially affected by Adult T-cell lymphoma?

A

Japan, West Africa & the Caribbean

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41
Q

What is Multiple Myeloma?

A

Monoclonal plasma cell cancer taht arises in the marrow & produces large amounts of IgG (55%) or IgA (25%)

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42
Q

What is the histo appearance of Multiple Myeloma?

A

“Fried egg” appearance

43
Q

What is the MC 1° tumor arising w/in bone in the elderly?

A

Multiple myeloma

(>40-50yo)

45
Q

What is Multiple myeloma assoc w/?

A
  • 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
Q

What is Bence Jones protein?

A

Ig light chains in urine

47
Q

What is Rouleaux formation?

A

RBCs stacked like poker chips in blood smear

48
Q

What is the histo of Multiple myeloma?

A

Numerous plasma cells w/ “clock face” chromatin & intracytoplasmic inclusions containing Ig

49
Q

What is Waldenström’s macrobloulinemia?

A
  • M spike= IgM
  • Hyperviscosity sx
  • No lytic bone lesions
50
Q

What is Monoclonal gammopathy of undetermined significance (MGUS)?

A

Monoclonal expansion of plasma cells w/ M spike

51
Q

What is the asx precursor to Multiple myeloma?

A

MGUS

Develop multiple myeloma at a rate of 1-2%/year

52
Q

What are the features of Leukemias?

A

Unregulated growth of leukocytes in BM→ Inc or dec in # of ciculating leukocytes in blood & marrow failure→ anemia, infections & hemorrhage

53
Q

Where can leukemic cells infiltrate?

A
  • Liver
  • Spleen
  • LN’s
54
Q

What is the age group for Acute lymphocytic leukemia/lymphoma (ALL)?

55
Q

How can T-cell ALL present?

A

Mediastinal mass (leukemic infiltration of the thymus)

56
Q

What is increased in the peripheral blood & BM in ALL?

A

Lymphoblasts

57
Q

What are the cell surface makers of ALL?

A
  • TdT+ (maker pre-T & pre-B cells)
  • CALLA+
58
Q

Which leukemia is most responsive to chemo?

59
Q

Where can ALL spread?

A

CNS & testes

60
Q

Which translocation is assoc w/ a better prognosis in ALL?

61
Q

What is the age group of Small lymphocytic lyphoma (SLL)/ chronic lymphocytic leukemia (CLL)?

62
Q

What are the sx of SLL/CLL?

63
Q

What is the histo of SLL/CLL?

A

Smudge cells in peripheral blood smear AI hemolytic anemia

64
Q

How does CLL differ from SLL?

A

CLL has inc peripheral blood lymphocytosis or BM involvement

65
Q

What is the age group of Hairy cell leukemia?

66
Q

What is Hairy cell leukemia?

A

B cell tumor in the elderly

67
Q

What is the histo of Hairy cell leukemia?

A
  • Cells have filamentous, hair-like projections
  • Stains TRAP (tartrate-resistant acid phosphatase) positive
68
Q

What is the tx for Hairy cell leukemia?

A

Cladribine, an adenosine analog

69
Q

What is the age group of Acute myelogenous leukemia (AML)?

A

Median onset 65 years

70
Q

What is the histo of AML?

A
  • Auer rods
  • Inc circulating myeloblasts on peripheral smear
71
Q

What is the translocation in M3 AML subtype?

72
Q

What is the tx for M3 AML subtype?

A

all-trans retinoic acid (vitamin A)

73
Q

What is a common presentation for M3 AML?

74
Q

What age group has the highest incidence of Chronic myelogenous leukemia (CML)?

75
Q

What is CML defined by?

A

Philadelphia chr (t[9,22], bcr-abl)

76
Q

What are the features of CML?

A
  • Myeloid stem cell prolif
  • Inc neutrophils, metamyelocytes, basophils
  • Splenomegaly
77
Q

What can CML accelerate or transform to?

A

AML or ALL (“blast crisis”)

78
Q

What can immature granulocytes in CML result in?

A

Very low leukocyte alkaline phosphatase

79
Q

What does CML respond to?

A

Imatinib (a small-molecule inhibitor of the bcr-abl tyrosine kinase)

80
Q

What are Auer bodies (rods)?

A

Peroxidase-positive cytoplasmic inclusions in granulocytes & myeloblasts

81
Q

What are Auer rods commonly seen in?

A

Acute promyelocytic leukemia (M3)

82
Q

What can the tx of AML M3 can release?

A

Auer rods→DIC

83
Q

What is Langerhans cell histiocytosis?

A

Prolif disorders of dendritic (Langerhans) cells from monocyte lineage

84
Q

How does Langerhans cell histiocytosis present?

A

In a child as lytic bone lesions & skin rash

85
Q

What are the cells like in Langerhans cell histiocytosis?

A

Functionally lymphocytes via Ag presentation

86
Q

Langerhans cell histiocytosis cells express ___ & ___.

A

S-100 & CD1a

87
Q

What is characteristic of Langerhans cell histiocytosis?

A

Birbeck granules (“tennis rackets” on EM)

88
Q

What are the lab features of Polycythemia vera?

A
  • Inc RBCs, WBCs & platelets
  • Philadelphia chr -
  • JAK2 mutations +
89
Q

What are the lab features of Essential thrombocytosis?

A
  • Inc platelets
  • Philadelphia chr -
  • JAK2 mutations + (30-50%)
90
Q

What are the lab features of Myelofibrosis?

A
  • Dec RBCs
  • Philadelphia chr -
  • JAK2 mutations +
91
Q

What are the lab features of CML?

A
  • Dec RBCs
  • Inc WBCs
  • Inc platelets
  • Philadelphia chr +
  • JAK2 mutations -
92
Q

What is JAK2 involved in?

A

Hematopoietic GF signaling

93
Q

What is Polycythemia vera?

A

ABN clone of hematopoietic stem cells w/ constitutively active JAK2 receptors, proliferate w/o EPO stim

94
Q

What does Polycythemia vera present as?

A

Intense itching after hot shower

95
Q

What is Essential thrombocytosis?

A

Similar to polycythemia vera, but specific for megakaryocytes

96
Q

What is Myelofibrosis?

A

Fibrotic obliteration of BM

Tear drop cell

97
Q

What is the cause of CML?

A

bcr-abl transformation l/t inc cell division & inhibition of apoptosis

98
Q

What are the lab features of Relative Polycythemia?

A

Dec plasma volume

99
Q

What are the lab features of Appropriate Absolute Polycythemia?

A
  • Inc RBC mass
  • Dec O2 saturation
100
Q

Which diseases are assoc w/ Appropriate absolute polycythemia?

A
  • Lung dx
  • Congenital heart dz
  • High altitude
101
Q

What are the lab features of Inappropriate absolute polycythemia?

A

Inc RBC mass

102
Q

Which diseases are w/ Inappropriate absolute polycythemia?

A
  • Renall cell CA
  • Wilm’s tumor
  • Cyst
  • Hepatocellular CA
  • Hydronephrosis
  • Due to ectopic erythropoietin
103
Q

What are the lab features for Polycythemia vera?

A

Inc plasma volume & RBC mass

Hematology & Oncology (7 decks)

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