October 6, 2015 - CLL

Question 1

Non-Hodgkin Lymphoma

Answer 1

Comprose most lymphomas.

B cell lymphomas account for 85% of NHL, while T cell lymphomas consist of about 15% of NHL.

Question 2

Small Lymphocytic Lymphoma

Answer 2

The same disease as CLL. This just does not have the lymphocytosis.

If you look at the bone marrow, it is identical to CLL.

Question 3

Chronic Lymphocytic Leukemia

Answer 3

A neoplastic disease characterized by the clonal proliferation and accumulation of small, mature-appearing immunologically incompetent B lymphocytes in the blood, marow, lymph nodes, and spleen. Don’t make useful antibodies, but are small and non-sticky so they aren’t overtly harmful either.

Accounts for 40% of all leukemias in patients older than 65. It is the most common lymphoid malignancy in the western world. Incidence increases with age… Median age of diagnosis is 72… CLL is statistically VERY unlikely in young patients.

Question 4

Smudge Cells

Answer 4

Consistent with CLL

Smudge cells in the presence of lymphocytosis is highly suggestive of CLL.

Question 5

Family History and CLL

Answer 5

Tightly linked. One of the most heritable blood cancers.

Does not indicate severity, however.

Question 6

B Cell Ontogency

Answer 6

Stem cells

Pre-B cells

Immature B cells

Mature B cells

Plasma cells

Question 7

B Cell Ontology and B Cell Malignancies

Answer 7

Stem cell - ALL

Pre-B cell - cALL

Immature B cell - NHL

Mature B cell - CLL/HCL

Plasma cell - WM/MM

Question 8

Diagnostic Test for CLL

Answer 8

Flow cytometry of the peripheral blood.

You want to look at the lymphocyte differential. You would expect to see skewed Kappa/Lambda ratio as well as elevated CD19+ count if it is CLL.

You need to have at least > 5 x 10⁹ / L B lymphocytes in peripheral blood for > 3 months to be classified as CLL. If less, it is called “monoclonal B cell lymphocytosis”, which may turn into CLL later.

Question 9

Diagnostic Test for T Cell Lymphoma

Answer 9

PCR

They have the TCR, not the kappa/lambda chains

Question 10

Threshold for CLL Diagnosis

Answer 10

Minimum needed to confirm diagnosis is > 5x10⁹ /L B lymphocytes in the peripheral blood for more than 3 months.

Less than this is called “monoclonal B cell lymphocytosis”, which may turn into CLL at a later date.

Question 11

CLL Natural History

Answer 11

Elevated lymphocytes in peripheral blood and bone marrow. (0)

Lymphocytosis with enlarged nodes. (1)

Lymphocytosis with enlarged spleen/liver. (2)

Lymphocytosis with anemia. (3)

Lymphocytosis with thrombocytopenia. (4)

Question 12

Indications for Treatment in CLL

Answer 12

Lymphocyte doubling time of less than 6 months

Systemic symptoms

Symptomatic lymphadenopathy or splenomegaly

Significant anemia or thrombocytopenia

Question 13

New Therapies for CLL

Answer 13

Monoclonal antibodies (rituximab, alemtuzumab)

Tyrosine kinase inhibitors

Question 14

Watch and Waiting

Answer 14

Often used in CLL.

Early treatment does not improve survival. Oftentimes things are not serious.