Lymphoid Leukemias

Question 1

When looking at a sample what is key in determining if it is reactive or neoplastic?

Answer 1

If the cells are various shapes and sizes, then most likely they are polyclonal and it is reactive
If the cells are very similar in size and shape looking almost the same, then likely neoplastic.

Question 2

How can you tell if cells are monoclonal or polyclonal?

Answer 2

• Kappa AND lambda light chains?
• Variable IgH gene?
• Aberrancy markers?

Question 3

What is the marker for an immature B-cell? Mature?

Answer 3

• CD10 - immature

- CD19 / CD20 - Mature B-cells

Question 4

What are the surface markers found with a myeloid origin acute leukemia?

Answer 4

CD13, CD14, CD15, CD33, CD117

Question 5

What is the difference in epidemiology of AML and ALL?

Answer 5

AML = Adults, Auer Rods, Myelodysplasia, Recurrent cytogenic abnormalities, myelodysplasia associated with age or therapy.

ALL = Children, Lymphoblast, B-cell&raquo_space; T-cell

Question 6

What indicate a good prognosis of B-ALL?

Answer 6

Children between 2-10
Hyperdiploidy
t(12;21)

Question 7

What indicates a bad prognosis in B-ALL?

Answer 7

Age: Younger than 2, Older than 10
Philadelphia Chromosome -- (9;22)
Hypodiploidy
Very High WBC count 100+
CSF Involvement

Question 8

What markers with flow cytometry can distinguish between B-ALL and T-ALL?

Answer 8

T-ALL will have CD1a and CD3

Question 9

What special procedure needs to be done with T-ALL treatment?

Answer 9

Chemotherapy needs to be injected directly into the CSF and testicles, since they are isolated and unable to get treated intravenously.

Question 10

How would T-ALL most likely present?

Answer 10

Most commonly adolescent males present with a thymic mass in their mediastinum.

Question 11

What is the first line therapy for ADULT patients with a Philadelphia chromosome ALL (mostly B)?

Answer 11

Dasatinib – tyrosine kinase inhibitor

Question 12

What are the antigenic markers for CLL/SLL?

Answer 12

CD20+
CD23+
CD19+
CD5+

Question 13

What are the clinical features of CLL/SLL?

Answer 13

Typically contains both bone marrow disease and lymph node disease that is asymptomatic and indolent. Can develope autoimmune hemolysis (broken B-cells)

Question 14

What determines Rai Staging system for CLL/SLL?

Answer 14

• Lymphocytosis
• Adenopathy
• Organomegaly
• Anemia / Thrombocytopenia
• *Each symptom you get a level**

Question 15

What are good indicators of prognosis of CLL/SLL?

Answer 15

• Low Rai Stage
• Mutated IGHV (this causes mature B-cells to proliferate in the germinal center in lymph nodes)
• Deletion of 13q

Question 16

What are bad prognosis of CLL/SLL?

Answer 16

• High Staging
• Non-mutated IGHV gene
• CD38/ZAP-70
• Deleted 11q or 17p

Question 17

How do you manage a patient with CLL/SLL since it is an indolent disease the progresses slowly?

Answer 17

Watch the disease parameters and cell counts, if symptoms progress or worsen is when treatment should happen. Over time the neoplastic cells overtake the bone marrow causing the more severe symptoms.

Question 18

What is the best treatment for progressing CLL?

Answer 18

BTK Inhibitor – prevents B-cell signaling

Rituximab – CD20 antibody

Question 19

What is characteristic of Hairy Cell Leukemia?

Answer 19

• protruding of cytoplasm of the cell
  +TRAP protein
• bone marrow fibrosis, causing pancytopenia and splenomegaly due to it trying to produce the lacking cells in the blood.
• “dry tap” of bone marrow aspiration, since fibrosis

Question 20

What are the unique surface markers of hair cell leukemia?

Answer 20

CD19/CD20 – mature B-cells
CD11c
CD22
Bright+

Question 21

What might a patient diagnosed with Adult T-cell leukemia/lymphoma be positive for?

Answer 21

HTLV-1 virus – endemic in Japan, West Africa, and Caribbean.

Question 22

What is a unique histological feature of Adult T-cell leukemia/lymphoma?

Answer 22

Lymphocytes that appear to have a lobulated nuclei, considered “flower” cells.

Question 23

What are common clinical features of ATLL?

Answer 23

• skin lesions (b/c T-cells!)
• hepatosplenomegaly
• LAD
• hypercalcemia?

Question 24

If a patient has cutaneous patches and plaques with generalized erythema and the histology reveals “cerebriform” nuclei, what is the likely diagnosis?

Answer 24

Mycosis Fungoides

Question 25

If patient has Mycosis Fungoides, what additional symptoms needs to be present to become diagnosed with Sezary Syndrome?

Answer 25

Lymphadenopathy and “Cerebriform”lymphocytes present in the blood.

Question 26

What T-cell leukemia is most common associated with Rheumatoid arthritis?

Answer 26

T-cell Large granular lymphocytic leukemia (LGLL)

Question 27

What is a histologically significant finding correlating to LGLL?

Answer 27

Lymphocytes with eosinophilic granules present