Chronic Leukemia

Question 1

A neoplastic proliferation of mature circulating lymphocytes that is characterized by a high WBC count…

Answer 1

Chronic leukemia

Question 2

A disease of neoplastic proliferation of naïve B cells…

Answer 2

Chronic lymphocytic leukemia

Question 3

What are the two markers that are co-expressed with naïve B cells in chronic lymphocytic leukemia?

Answer 3

CD5 and CD20

Question 4

What is seen on the blood smear of a patient with chronic lymphocytic leukemia?

Answer 4

Increased lymphocytes

Smudge cells

Question 5

Chronic lymphocytic leukemia can involve the lymph nodes causing a lymphadenopathy. What is this called?

Answer 5

Small lymphocytic lymphoma

Question 6

What are the 3 major complications seen with chronic lymphocytic leukemia?

Answer 6

Hypogammaglobulinemia
Autoimmune hemolytic anemia
Richter transformation—a transformation to diffuse large B-cell lymphoma

Question 7

What is Richter transformation?

Answer 7

Chronic lymphocytic leukemia transforming into a diffuse large B-cell lymphoma

Question 8

What marks Richter transformation seen with chronic lymphocytic leukemia clinically?

Answer 8

Enlarging lymph node or spleen

Question 9

What is the most common cause of death seen in patients with chronic lymphocytic leukemia?

Answer 9

Infection

Question 10

Why is infection the most common cause of death in chronic lymphocytic leukemia?

Answer 10

Due to hypogammaglobulinemia

Question 11

A neoplastic proliferation of mature B cells that is characterized by hairy cytoplasmic processes?

Answer 11

Hairy cell leukemia

Question 12

Hairy cell leukemia cells are positive for what?

Answer 12

Tartrate-resistant acid phosphatase (TRAP)

Question 13

What are the clinical features seen with hairy cell leukemia?

Answer 13

Splenomegaly

Dry tap on bone marrow aspiration

Question 14

What causes the splenomegaly seen with hairy cell leukemia?

Answer 14

Accumulation of hairy cells in RED PULP

This is different from other leukemias because the cells usually accumulate in the white pulp

Question 15

What is the best treatment for hairy cell leukemia?

Answer 15

2-CDA—cladribine

Question 16

What is the mechanism of action for 2-CDA (cladribine) when treating hairy cell leukemia?

Answer 16

2-CDA is an adenosine deaminase inhibitor and causes adenosine to accumulate in the neoplastic B cells causing death of the cells

Question 17

A disease involving neoplastic proliferation of mature CD4+ T cells?

Answer 17

Adult T-cell leukemia/Lymphoma

Question 18

What marker is associated with adult T-cell leukemia/lymphoma?

Answer 18

HTLV-1

Question 19

Adult T-cell leukemia/lymphoma is most commonly seen in what areas of the world?

Answer 19

Japan

Caribbean

Question 20

What are the clinical features seen with Adult T-cell leukemia/lymphoma?

Answer 20

Rash
Generalized lymphadenopathy
Hepatosplenomegaly
Lytic bone lesions with hypercalcemia—don’t confuse with multiple myeloma!

Question 21

A neoplastic proliferation of mature CD4+ T cells that infiltrate the skin?

Answer 21

Mycosis fungoides

Question 22

What is produced by mycosis fungoides due the infiltration of the skin?

Answer 22

Localized skin rash, plaques, and nodules

Question 23

What are pautrier microabscesses seen with mycosis fungoides?

Answer 23

Aggregates of neoplastic cells in the epidermis

Question 24

Mycosis fungoides cells can spread to involve the blood. This pathogenesis causes what syndrome to occur?

Answer 24

Sezary syndrome

Question 25

What characterizes Sezary syndrome?

Answer 25

Lymphocytes with cerebriform nuclei (Sezary cells) on blood smear