6-3: Chronic Leukemia

Question 1

Chronic leukemia is referring to a … proliferation of

Answer 1

proliferation CD8 or CD4 t-cells

MATURE T-CELLS circulating around in large numbers

Question 2

Chronic leukemia is seen in which age group?

Answer 2

Older adult (bc it has a rather slow onset)

Question 3

Chronic Lymphocytic leukemia (CLL)

• proliferation of what cell type?
• what markers are expressed?

Answer 3

• prolieration of naive B-cells

- co-express CD5 and CD20

Question 4

Chronic lymphocytic leukemia on blood smear…

Answer 4

increased lymphocytes

smudge cells

Question 5

*Smudge cells characteristic of which proliferative disease?

Answer 5

*CLL - chronic lymphocytic leukemia

Question 6

*CD5 and CD20 are co-expressed on what cell type in which disease?

Answer 6

*on naive B-cells

Chronic lymphocytic leukemia

Question 7

If Chronic lymphocytic leukemia involves a lymph node (lymph node becomes swollen) disease is named…

Answer 7

small lymphocytic LYMPHOMA (bc there is now a mass - not just blood)

Question 8

Complications of CLL…

Answer 8

• hypogammaglobinemia - B-cell NEVER MATURE INTO PLASMA CELLS == LOW immunoglobulin == infection risk
• autoimmune hemolytic anemia - the B-cells dont mature into plasma cells to produce Ig’s but if they do they usually screw it up and cause some autoimmune rxn
• transformation to diffuse large B-cell lymphoma - enlarging lymph node or enlarging spleen

Question 9

CLL deaths are most commonly caused by…

Answer 9

infection

Question 10

Hairy cell leukemia-

• proliferation of what cells?
• cells are positive for what enzyme?

Answer 10

• Proliferation of mature B-cell

- cells are positive for TRAP (tartrate resistant acid phosphatase)

Question 11

Proliferation of mature B-cell diseases:

Answer 11

• CLL (leukemia or lymphoma)

- hairy cell leukemia

Question 12

“Hairy cells” in red pulp of spleen is characteristic of…

Answer 12

Hairy cell leukemia – enlarges the spleen

Question 13

“dry tap” in bone marrow is characteristic of what disease?

Answer 13

hairy cell leukemia

dry tap = marrow fibrosis

Question 14

hairy cell leukemia clinical features:

Answer 14

• splenomegaly (red pulp)
• dry tap with bone marrow aspiration
• lymphadenopathy is ABSENT!

Question 15

red pulp enlargement disease vs white pulp enlargement disease and associated cells?

Answer 15

• red pulp = hairy cell leukemia due to mature B-cells

- White pulp = mononucleosis (EBV)

Question 16

TRAP menomic thingy for hairy cell

Answer 16

B-cells are TRAPPED in the spleen (red pulp, they are TRAPPED in the bone marrow (dry tap) and therefore they cant go to where you normally expect them - lymph nodes.

Question 17

Hairy cell leukemia prognosis and Tx

Answer 17

• Drug = 2-CDA and there is an excellent response

- adenosine deaminase inhibitor = adenosine accumulates to toxic levels in neoplastic B-cells

Question 18

Adult T-cell Leukemia Lymphoma (ATLL) -

• Proliferation of what cells?
• Associated with what virus?
• where in the world is this common?

Answer 18

• neoplastic proliferation of mature CD4 T-cells
• HTLV-I association (human tcell leukemia virus type I)
• Japan and Caribbean

Question 19

Adult T-cell Leukemia Lymphoma (ATLL) -

Clinical features

Answer 19

• RASH
• generalized LAD (lymphadenopathy) and HSH (hepatosplenomegally)
• -lytic bone lesions with hypercalcemia

Question 20

T-cell leukemias generally like to do what?

Answer 20

Go to skin and cause a rash

Question 21

patient has lytic bone lesions vs patient with lytic bone lesions and rash?

Answer 21

just bone lesions = multiple myeloma

bone lesions with rash = ATLL

Question 22

Mycosis fungoides -

• proliferation of what cell type?
• what happens/clinical presenation?

Answer 22

• neoplastic prolif of amture CD4 t-cells
• t-cells involved so the cells infiltrate skin producting rash, plaques, or nodules –> t-cells in epidermis are called Pautrier microabscesses

Question 23

*Pautrier microabscesses are associated with?

Answer 23

*T-cell neoplasms that aggregate in the epidermis

Question 24

If T-cell neoplasm spreads to the blood its called?

Answer 24

Sezary syndrome

Question 25

Blood smear characteristics of Sezary syndrome?

Answer 25

lymphocytes with cerebriform nuclei - bunch of lobes like a brain