HEME ONC WHITES Flashcards

1
Q

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

A

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

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1
Q

What is constitutively expressed in polycythemia vera?

A

JAK2

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2
Q

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

A

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

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2
Q

Bence-Jones Protein

A

Multiple Myeloma

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3
Q

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

A

EBV

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3
Q

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

A

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

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4
Q

What age groups are affected by ALL?

A

Young kids

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5
Q

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

A

Reed-Sternberg (of B cell origin)

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6
Q

How does Hodgkin’s lymphoma differe anatomically from NHL?

A

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

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6
Q

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

A

60 or older; smudge cell

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6
Q

TdT (+)

A

ALL

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7
Q

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

A

HTLV-1; Japan, West Africa, and Caribbean

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8
Q

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

A

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

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9
Q

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

A

Endemic (jaw); sporadic (pelvis or abdomen)

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10
Q

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

A

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

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10
Q

bcl-2

A

follicular lymphoma

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11
Q

Type of anemia associated with myelofibrosis

A

Myelophthisic anemia, teardrop cells = dacryocytes

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12
Q

What age group is most afflicted by AML?

A

65 years and older

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13
Q

Auer rods

A

AML M3

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14
Q

What is involved in t(12;21)?

A

This translocation is involved with a better prognosis of ALL

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15
Q

What is a leukemoid reaction?

A

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

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15
Q

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

A

30-60

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16
Q

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

A

CD4; Pautrier’s microabscesses

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16
Q

What is the CRAB criteria for multiple myeloma?

A

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

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16
Q

What type of amyloidosis is multiple myeloma associated with?

A

primary (light chain amyloidosis)

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16
Q

2 things that Langerhans histiocytosis cells express

A

S-100 and CD1a

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16
Q

What involves t(14;18)?

A

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

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17
Q

Tennis Racket appearance on EM

A

Birbeck Granules of Langerhans Histiocytosis

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18
Q

What is involved in t(15;17)?

A

AML M3

19
Q

What is the translocation in follicular lymphoma?

A

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

20
Q

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

A

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)

21
Q

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

A

It induces the differentiation of myeloblasts

22
Q

How is essential thrombocytosis similar/different from polycythemia vera?

A

Essential thrombocytosis is specific for megakaryocytes… BOTH ARE MYELOPROLIFERATIVE DISORDERS

22
Q

cyclin D1

A

mantle cell lymphoma

24
Q

What is the biggest concern with CML?

A

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

25
Q

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

A

Auer rods

26
Q

Who is likely to get mantle cell and follicular lymphoma?

A

Adults and mantle cell is most likely in older males

27
Q

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

A

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

28
Q

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

A

CD30 and CD15

30
Q

CD1a (+) and S-100 (+)

A

Langerhans histiocytosis

31
Q

What is the age distribution for Hodgkin’s lymphoma?

A

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

33
Q

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

A

tyrosine kinase turned on that leads to expansion of myeloid lines

34
Q

Which translocation is involved in Acute Myelogenous Leukemia?

A

t(15;17)

35
Q

Who is affected by Burkitt’s lymphoma?

A

Usually adolescents and young adults

37
Q

Which type of leukemia can present as a mediastinal mass?

A

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

39
Q

Smudge cells

A

SLL/CLL

41
Q

How is CML defined?

A

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

42
Q

TRAP (+)

A

Hairy Cell Leukemia

43
Q

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

A

Nodular Sclerosing

45
Q

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

A

IgG (55%) and IgA (25%)

47
Q

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

A

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

48
Q

What is the most common type of NHL in adults?

A

Diffuse Large B-Cell lymphoma

48
Q

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

A

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

50
Q

CALLA (+)

A

ALL

52
Q

Why would treating AML M3 lead to DIC?

A

The release of auer rods turns on the coagulation cascade

53
Q

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

A

CNS and testes

54
Q

What is involved in t(9;22)

A

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

55
Q

What is the main difference between leukemia and lymphoma?

A

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

57
Q

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

A

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

58
Q

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

A

Elderly men; TRAP positive–Tartrate Resistant Acid Phosphatase

60
Q

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

A

NHL

61
Q

What drug does CML respond to?

A

Imatinib (an inhibitor of bcr-abl tyrosine kinase)

62
Q

What involves t(11;14)?

A

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

63
Q

What is the treatment for hairy cell leukemia?

A

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

64
Q

Birbeck Granules

A

Langerhans histiocytosis

65
Q

JAK2 constitutively expressed

A

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

66
Q

What translocation occurs in Mantle Cell lymphoma? Prognosis?

A

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

68
Q

What is a very common presentation of M3 AML?

A

DIC–auer rods can leak out and induce it

69
Q

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

A

CD5

70
Q

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

A

Langerhans histiocytosis

71
Q

What involves t(8;14)?

A

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