Hematopoietic Malignancies Putting it Together

Question 1

what are the two major differences between acute and chronic leukemias?

Answer 1

the clinical course of the disease and the maturity of the white blood cell that is involved

Question 2

what is acute leukemia?

Answer 2

sudden onset, devastating symptoms, and quickly fatal if not treated.

bone marrow and blood full of blasts or very immature cells

may be lymphoid or myeloid

Question 3

what is chronic leukemia?

Answer 3

slow development over time (may be asymptomatic at diagnosis)

bone marrow and blood contain more mature-appearing malignant cells

may be lymphoid or myeloid

Question 4

what does the blood smear of AML vs. CML look like?

Answer 4

AML: a cluster of blasts with just a thin rim of cytoplasm surrounding nuclei with fine chromatin and prominent nucleoli

CML: a spread of maturing neutrophilic cells: promyelocytes, myelocytes, metamyelocytes, and multiple segmented neutrophils

Question 5

what’s the difference between benign follicular hyperplasia and follicular lymphoma?

Answer 5

go look at the picture!

1. benign follicular hyperplasia

• Normal nodal architecture maintained
• Loosely-packed follicles of varying size and shape
• Well-defined mantle zone
• Germinal centers contain tingible-body macrophages

2. Follicular lymphoma

• Normal nodal architecture obliterated
• Densely-packed, uniform follicles
• Mantle zone absent
• Follicles do not have tingible-body macrophages

Question 6

Can you name three features of follicular lymphoma that differentiate it from benign follicular hyperplasia?

Answer 6

Densely packed follicles, obliteration of normal architecture, and absence of tingible bodies in follicles.

Question 7

what is are the characteristics of benign follicular hyperplasia?

Answer 7

• Normal nodal architecture maintained
• Loosely-packed follicles of varying size and shape
• Well-defined mantle zone
• Germinal centers contain tingible-body macrophages

Question 8

what are the characteristics of follicular lymphoma?

Answer 8

• Normal nodal architecture obliterated
• Densely-packed, uniform follicles
• Mantle zone absent
• Follicles do not have tingible-body macrophages

Question 9

what are the two major differences between low grade and high grade lymphomas?

Answer 9

the clinical course of the disease and the type of cell involved

Question 10

low grade vs. high grade lymphomas

Answer 10

1. low grade:

• Slow-growing; long, indolent course
• Composed of small, mature-appearing lymphocytes
• Treatment often delayed until patient develops severe symptoms

2. high grade:

• Very rapidly growing; aggressive course
• Composed of large, highly mitotically active cells
• Treatment should be immediate and aggressive

Question 11

what are the characteristics of low grade lymphomas?

Answer 11

• Slow-growing; long, indolent course
• Composed of small, mature-appearing lymphocytes
• Treatment often delayed until patient develops severe symptoms

Question 12

what are the characteristics of high grade lymphomas?

Answer 12

• Very rapidly growing; aggressive course
• Composed of large, highly mitotically active cells
• Treatment should be immediate and aggressive

Question 13

which disease has a t(8;21)?

Answer 13

present in many cases of AML-M2 (acute myeloid leukemia with maturation)

it confers a favorable prognosis in adults, but not in children.

Question 14

which disease has a t(15;17)?

Answer 14

present in (and diagnostic of ) all cases of AML-M3 (acute promyelocytic leukemia)

treatment is all-trans retinoic acid (ATRA), which is usually started right away and results in an excellent prognosis.

to remember the translocation, remind yourself that retinoic acid is also a treatment in acne. Teenagers tend to get acne around 15 or 17 years old. Now remember that the translocation for APL is 15;17.

Question 15

which disease has inv(16)?

Answer 15

oresent in many cases of AML-M4 (acute myelomonocytic leukemia)

these cases usually have large eosinophils with dark granules seen on the smear

cases of AML-M4 with this translocation have a favorable prognosis with therapy

Question 16

which diseases have 11q23?

Answer 16

Seen mostly in AML variants with a monocytic component, such as AML- M4 and AML-M5

there are over 100 types of specific rearrangement and translocation patterns – so no need to try to remember the specific partner chromosomes in this one!

In general, 11q23 rearrangements carry a very poor prognosis

Question 17

which disease has an FLT-3 mutation?

Answer 17

present in 30% of cases of AML, with an increased frequency in AML-M3

FLT3 is a receptor tyrosine kinase that stimulates unregulated cell proliferation

this mutation is associated with a high tumor burden, poor response to therapy, frequent relapse, and overall poor survival

Question 18

which disease a t(9:22)?

Answer 18

Present in all cases of chronic myeloid leukemia (CML). this translocation is called the Philadelphia chromosome and results in a BCR-ABL fusion protein, which is a powerful tyrosine kinase causing unregulated cell growth.

prognosis has improved dramatically since the development of tyrosine kinase inhibitors

the Philadelphia chromosome is also seen in some cases of ALL and indicates a poor prognosis

tell yourself that the P in Philadelphia chromosome looks like the 9 in t(9;22)

Question 19

which disease has t(11;14)?

Answer 19

present in most cases of mantle cell lymphoma

tell yourself that the word ‘cell’ should remind you of chromosome 11.

mantle cell lymphoma = mantle ce11 = t(11;14)

Question 20

which disease has t(14;18)?

Answer 20

present in most cases of follicular lymphoma

tell yourself that there are 18 letters in the word follicular lymphoma, which just so happens to involve chromosome 18 – t(14;18). Also, follicular sounds like fourteen.

Question 21

which disease has t(8;14)?

Answer 21

present in most cases of Burkitt lymphoma

Tell yourself that the ‘B’ in Burkitt’s looks like an 8. Burkitt’s lymphoma = 8ukitt’s = t(8;14)

Question 22

What kind of WBC predominates in acute leukemias?

Answer 22

blasts

Question 23

which of the following histologic features would be seen in a case of follicular lymphoma?

Answer 23

obliterated nodal architecture