Chronic Leukemia

Question 1

What is the big translocation and gene involved in CML?

Answer 1

9:22 translocation

BCR-ABL fusion gene

Question 2

What does the accelerated phase of CML present with unusually?

Answer 2

Leukocytosis, splenomegaly, extreme thrombocytopenia or thrombocytosis.

Question 3

4 clinical symptoms/signs patients present with when they have CML?

Answer 3

Fatigue, lethargy, low grade fever, and weight loss.

Question 4

3 things on physical exam to see for CML?

Answer 4

Pallor skin, splenomegaly, and no LAD usually.

Question 5

What is the family of drugs to use for CML, why, and what is a specific drug to use?

Answer 5

Tyrosine kinase inhibitors because the fusion gene increases tyrosine kinase activity way too much
Imatinib

Question 6

What is the alternative to tyrosine kinase inhibitors and in what setting would we use it?

Answer 6

BMT
This is the most definitive option to cure disease
Might be used to initiate therapy in kids, but typically used in patients who are resistant to tyrosine kinase inhibitors.

Question 7

When does CML become a very poor prognosis?

Answer 7

disease transforming into the Accelerated phase or blast phase

Question 8

What are signs of accelerated phase and blast crisis for CML?

Answer 8

Increasing leukocyte counts, basophilia, fever and an enlarging spleen

Question 9

3 complications of CLL and what is a key laboratory finding?

Answer 9

Infections from encapsulated pathogens
Autoimmune thrombocytopenia and anemia
Transformation to DLBCL

Question 10

2 requirements for diagnosing CLL? What are the three markers of the B cells?

Answer 10

Lymphocyte count over 5k in the absent of a viral infection
Clonality of circulating b lymphocytes
5, 19 and 20

Question 11

Stage 0 - 4 of CLL for the Rai Staging?

Answer 11

0 - lymphocytosis
1 - plus LAD
2 - plus hepatosplenomegaly 
3 - plus anemia (Hb less than 11)
4 - plus thrombocytopenia

Question 12

What percentage of patients are found in each stage and what is the prognosis for each?

Answer 12

Stage 0 - 25% greater than 10 year survival
Stage 1 and 2 - 50% of patients 6-9
Stage 4 and 4 - 25% less than 2 years

Question 13

What is the Binet system based on?

Answer 13

Number of lymph nodes involved

Question 14

What are some ways we treat CLL?

Answer 14

Chemo, prednisone or anti CD20 for the autoimmune part
IVIG for the low immunoglobulin
Get vaccines for recurrent infections

Question 15

What are three options of meds to initiate therapy for CLL?

Answer 15

Purine analogs, alkylating agents, and monoclonal antibodies

Question 16

When do we consider stem cell transplantation for CLL patients?

Answer 16

High risk genetic features like 17p deletion and 11q deletion

Question 17

How do we best define/characterize hairy cell leukemia?

Answer 17

Patients have splenomegaly and diffuse bone marrow involvement and the clinical picture is dominated by symptoms of the enlarged spleen and pancytopenia

Question 18

What are three cd markers for hairy cell leukemia and which one is an excellent marker for disease activity?

Answer 18

22,25,103

Question 19

Two mutations most cases of hairy cell leukemia have?

Answer 19

BRAF and V600E

Question 20

4 treatment options for hairy cell leukemia?

Answer 20

Splenectomy
Nucleosides but can lead to further immunosuppression and increase opportunistic infections
Interferon alpha, but not as effective as nucleosides
BRAF inhibitors like vemurafenib