BIIC Pathology Lecture 8_Chronic Leukemia

Question 1

What demographic is most at risk for Multiple Myeloma?

Answer 1

African american males over the age of 70

Question 2

What are 4 the common Clinical features of multipul myeloma?

Answer 2

1) Lytic leasions that result from increased osteoclast activity (leads to hypercalcemia) 2) Monoclolony of antibodies 3) Free kapa and gamma light chains in the urin 4) Bacterial infections because of monoclonal Antibodies

Question 3

What mutation causes Multiple Myeloma?

Answer 3

Ig locus on chromosome 14 fuses to Dq or Gs cycline genes. This pushes cells from G1 to S-Phase

Question 4

Why does Multiple Myeloma lead to normocytic anemia?

Answer 4

Because lytic lesions take over medulary space and displaces bone marrow

Question 5

How do CLL and SLL cells stay alive?

Answer 5

High levels of BCL2. Elevated BCL2 levels come about by a mechanisium other than the t(14:18) mutation.

Question 6

Review what a smudge cell looks like.

Answer 6

Question 7

What unique surface protein is found on B cells?

Answer 7

CD5 (typically a t cell protein)

Question 8

What are the chromosomal abnormalities typical of Chronic Lymphocytic Leukemia?

Answer 8

the most commonly trisomy 12
and deletions involving portions of chromosomes 11, 13, and 17.

NOTE: Translocations are rate for CLL

Question 9

What is the only way to cure CLL and SLL?

Answer 9

Cure may only be achieved with hematopoietic stem cell transplantation

Question 10

What causes hairy cell leukemia?

Answer 10

activating
mutations in the serine/threonine kinase BRAF,

Question 11

What cell protein markers are unique to Hairy cell leukemia?

Answer 11

CD11c and CD103

Question 12

What demographic is most at risk for Hairy Cell Leukemia? How is the prognosis?

Answer 12

Older males. The prognosis is excelent.

Question 13

What is a Myeloproliferative neoplasms?

Answer 13

It is a condition characterized by the disregulated growth of mature CMP line cells

Question 14

What are the 4 Myeloproliferative Disorders from class?

Answer 14

1. Chronic myeloid leukemia (CML)
2. Polycythemia vera
3. Primary myelofibrosis
4. Essential thrombocytosis

Question 15

What mutations causes CML?

Answer 15

t(9;22) that creates the BCR-ABL oncogene that is constitutivly active

Question 16

What pharmacology innervention can be used to treat CML?

Answer 16

tyrosine kinase inhibitors, eg,
imatinib

Question 17

What causes Polycythemia Vera?

Answer 17

point mutations in the tyrosine kinase JAK2 that makes JAK2 over active

Question 18

How can PV be distinguished fromrelative polycythemia?

Answer 18

polycythemia vera is associated with low levels of
serum erythropoietin, reflecting the growth factorindependence
of the neoplastic clone.

Question 19

What causes Primary Myelofibrosis

Answer 19

JAK-STAT activation

Question 20

What does PMF cause?

Answer 20

Primary Myelofibrosis results in the fibrosis of the medulary space. This leads to reduced hemeatopoesis, anemia, Progresive spleenomegaly, and bizarre shaped red blood cells (poiklocytes and teardrops)

Question 21

What causes Essential thrombocytosis (ET) and how does it manifest?

Answer 21

ET is caused to by an activating point mutation in JAK2 or MPL. It causes a hyperproliferation of platelets without polycythemia (rule out PV) and without marrow fibrosis (rule out PMF)