HEME ONC WHITES

Question 1

How is the M-spike of Waldenstrom’s Macroglobulinemia different from the M-spike of Multiple Myeloma?

Answer 1

M spike means “Monoclonal Ab” in Wald that Mab is IgM (hence, hyperviscosity) and in Multiple Myeloma that Mab is IgG usually or IgA

Question 1

What is constitutively expressed in polycythemia vera?

Answer 1

JAK2

Question 2

Why are neoplastic cells in Langerhans histiocytosis S-100 (+)? What primary CNS tumor is S-100 (+)?

Answer 2

Because they are dendritic cells of neural crest origin; Schwannomas are also S-100 positive

Question 2

Bence-Jones Protein

Answer 2

Multiple Myeloma

Question 3

Which infection are 50% of Hodgkin’s lymphoma patients infected with?

Answer 3

EBV

Question 3

What cell has a fried egg appearance in hematologic histology? Brain histology?

Answer 3

heme = neoplastic plasma cell of multiple myeloma; brain = oligodendrocyte whether normal or in oligodendroglioma

Question 4

What age groups are affected by ALL?

Answer 4

Young kids

Question 5

Hodgkin’s lymphoma has a good prognosis if there is a strong lymphocytic response against which cells?

Answer 5

Reed-Sternberg (of B cell origin)

Question 6

How does Hodgkin’s lymphoma differe anatomically from NHL?

Answer 6

Hodgkin’s involves a localized set of lymph nodes and the spread is contiguous whereas with NHL the pattern is diffuse and the spread is non-contiguous

Question 6

What age is affected by CLL/SLL? What is the pathognomonic cell?

Answer 6

60 or older; smudge cell

Question 6

TdT (+)

Answer 6

ALL

Question 7

What causes Adult T-cell lymphoma? Name 3 geographic areas most affected.

Answer 7

HTLV-1; Japan, West Africa, and Caribbean

Question 8

What is MGUS? What is the risk of progression to multiple myeloma?

Answer 8

A plasma cell expansion with monoclonal antibody (M spike) production but asymptomatic i.e. no CRAB. There is a 1-2% per year risk of going on to multiple myeloma

Question 9

What is affected (anatomically) in the endemic (African) form of Burkitt’s vs. the sporadic form?

Answer 9

Endemic (jaw); sporadic (pelvis or abdomen)

Question 10

What is a good way to differentiate CML from a leukemoid reaction?

Answer 10

Both have a high WBC count but CML has low leukocyte alkaline phosphatase and leukemoid reaction has high leukocyte alkaline phosphatase

Question 10

bcl-2

Answer 10

follicular lymphoma

Question 11

Type of anemia associated with myelofibrosis

Answer 11

Myelophthisic anemia, teardrop cells = dacryocytes

Question 12

What age group is most afflicted by AML?

Answer 12

65 years and older

Question 13

Auer rods

Answer 13

AML M3

Question 14

What is involved in t(12;21)?

Answer 14

This translocation is involved with a better prognosis of ALL

Question 15

What is a leukemoid reaction?

Answer 15

This is an acute inflammatory response to infection it involves increased levels of WBC’s and bands as well as leukocyte alkaline phosphatase

Question 15

What age group is most likely to be affected by chronic myelogenous leukemia?

Answer 15

30-60

Question 16

What cluster of differentiation marker is involved in the neoplastic cells os mycosis fungoides/Sezary syndrome? What “abscesses” are associated?

Answer 16

CD4; Pautrier’s microabscesses

Question 16

What is the CRAB criteria for multiple myeloma?

Answer 16

Hypercalcemia, Renal insufficiency, Anemia (because all the myeloid is going into making the neoplastic cells), and Bone lesions (lytic) and back pain

Question 16

What type of amyloidosis is multiple myeloma associated with?

Answer 16

primary (light chain amyloidosis)

Question 16

2 things that Langerhans histiocytosis cells express

Answer 16

S-100 and CD1a

Question 16

What involves t(14;18)?

Answer 16

Follicular lymphoma 14 = Heavy Chain Ig and 18 = bcl-2

Question 17

Tennis Racket appearance on EM

Answer 17

Birbeck Granules of Langerhans Histiocytosis

Question 18

What is involved in t(15;17)?

Answer 18

AML M3

Question 19

What is the translocation in follicular lymphoma?

Answer 19

t(14;18)--14 is heavy chain Ig, and 18 is bcl-2

Question 20

Why are leukemic patients susceptible to anemia, infections, and bleeding?

Answer 20

Because all of the effort of the blood cell line is put into making the neoplastic cells which are useless, really. This occurs at the expense of RBC's (Anemia), WBC's (infections) and platelets (bleeding)

Question 21

Why is M3 AML amenable to Tx with all-trans-retinoic acid?

Answer 21

It induces the differentiation of myeloblasts

Question 22

How is essential thrombocytosis similar/different from polycythemia vera?

Answer 22

Essential thrombocytosis is specific for megakaryocytes... BOTH ARE MYELOPROLIFERATIVE DISORDERS

Question 22

cyclin D1

Answer 22

mantle cell lymphoma

Question 24

What is the biggest concern with CML?

Answer 24

That there may be a blast crisis and it will revert to AML or even ALL

Question 25

These are peroxidase positive cytoplasmic inclusions in granulocytes and myeloblasts that are present, particularly in AML M3

Answer 25

Auer rods

Question 26

Who is likely to get mantle cell and follicular lymphoma?

Answer 26

Adults and mantle cell is most likely in older males

Question 27

How is SLL different from CLL? What type of anemia are these associated with?

Answer 27

SLL is small lymphocytic lymphoma and CLL is chronic lymphocytic leukemia; so CLL involves the blood and bone marrow whereas SLL only invoves lymph nodes, but they are basically the same and treated the same; associated with warm and cold autoimmune hemolytic anemia

Question 28

Reed-Sternberg cells have which cluster of differentiation markers (2)?

Answer 28

CD30 and CD15

Question 30

CD1a (+) and S-100 (+)

Answer 30

Langerhans histiocytosis

Question 31

What is the age distribution for Hodgkin's lymphoma?

Answer 31

BIMODAL, it tends to appear in young adulthood and after 55 years and is more common in males except for the nodular sclerosing subtype

Question 33

What kind of molecule is the bcr-abl fusion protein?

Answer 33

tyrosine kinase turned on that leads to expansion of myeloid lines

Question 34

Which translocation is involved in Acute Myelogenous Leukemia?

Answer 34

t(15;17)

Question 35

Who is affected by Burkitt's lymphoma?

Answer 35

Usually adolescents and young adults

Question 37

Which type of leukemia can present as a mediastinal mass?

Answer 37

T-cell ALL as the neoplastic T cells can infiltrate the thymus

Question 39

Smudge cells

Answer 39

SLL/CLL

Question 41

How is CML defined?

Answer 41

Presence of t(9;22) the Philadelphia Chromosome? Low leukocyte alk phos, and myeloid stem cell proliferation

Question 42

TRAP (+)

Answer 42

Hairy Cell Leukemia

Question 43

Which type of Hodgkin's lymphoma has the best prognosis?

Answer 43

Nodular Sclerosing

Question 45

What immunoglobulins are the neoplastic plasma cells of multiple myeloma most likely to produce?

Answer 45

IgG (55%) and IgA (25%)

Question 47

What is the strongest prognostic indicator for Hodgkin's lymphoma?

Answer 47

STAGE, this is because it has contiguous spread, so the further the spread the worse the Dz

Question 48

What is the most common type of NHL in adults?

Answer 48

Diffuse Large B-Cell lymphoma

Question 48

What does ALL stand for? What marker is present? Which translocation is associated with a better prognosis?

Answer 48

Acute lymphoBLASTIC leukemia/lymphoma; TdT is present as it is a marker for lymphoblasts also CALLA (+); t(12;21) is associated with best prognosis

Question 50

CALLA (+)

Answer 50

ALL

Question 52

Why would treating AML M3 lead to DIC?

Answer 52

The release of auer rods turns on the coagulation cascade

Question 53

What are 2 places that ALL is most likely to spread?

Answer 53

CNS and testes

Question 54

What is involved in t(9;22)

Answer 54

Philadelphia chromosome of CML (bcr-abl fusion protein that is a tyrosine kinase)

Question 55

What is the main difference between leukemia and lymphoma?

Answer 55

Leukemia involves the bone marrow which results in "spillage" of neoplastic WBC's into the blood and lymphoma is what arises in a lymph node

Question 57

Describe the genetics (translocation) involved in Burkitt's lymphoma

Answer 57

t(8;14) which is c-myc from chromosome 8 and heavy-chain Ig from chromosome 14

Question 58

Who gets hairy cell leukemia? How do you stain for it?

Answer 58

Elderly men; TRAP positive--Tartrate Resistant Acid Phosphatase

Question 60

Which type of lymphoma (Hodgkin's/NHL) is more likely to be associated with immunosuppression?

Answer 60

NHL

Question 61

What drug does CML respond to?

Answer 61

Imatinib (an inhibitor of bcr-abl tyrosine kinase)

Question 62

What involves t(11;14)?

Answer 62

mantle cell lymphoma; 11= cyclin D1 and 14 = heavy chain Ig

Question 63

What is the treatment for hairy cell leukemia?

Answer 63

Cladribine, an adenosine analog --could this be because the same reason that adenosine deaminase deficiency causes SCID???

Question 64

Birbeck Granules

Answer 64

Langerhans histiocytosis

Question 65

JAK2 constitutively expressed

Answer 65

Polycythemia vera and sometimes in essential thrombocytosis and myelofibrosis... i.e. in myeloproliferative disorders

Question 66

What translocation occurs in Mantle Cell lymphoma? Prognosis?

Answer 66

t(11;14); cyclin D1 (11) and heavy chain Ig (14) --POOR PROGNOSIS

Question 68

What is a very common presentation of M3 AML?

Answer 68

DIC--auer rods can leak out and induce it

Question 69

Neoplastic cells in Mantle Cell lymphoma are positive for this cluster of differentiation marker

Answer 69

CD5

Question 70

Which heme/onc issue can present in a child with lytic lesions and skin rash?

Answer 70

Langerhans histiocytosis

Question 71

What involves t(8;14)?

Answer 71

Burkitt's lymphoma 8 = c-myc and 14 = heavy chain Ig