6.3 Chronic Leukemia

Question 1

What cells proliferate in chronic leukemia, what is the characteristic blood finding, how does the disease come on, who gets it?

Answer 1

Proliferation of mature circulating lymphocytes
Blood shows high WBC count
Insidious onset
Usually older adults

Question 2

What cells are neoplastic in CLL? Cell markers?

Answer 2

naive B cells that co-express CD5 and CD20

Question 3

What is the most common leukemia overall?

Answer 3

CLL

Question 4

What is seen on blood smear for CLL?

Answer 4

Increased lymphocytes and Smudge cells

Question 5

What systemic sign might be present with CLL?

Answer 5

Generalized LAD signals involvement of the lymph nodes

Sometimes called small lymphocytic lymphoma

Question 6

Complications of CLL

Answer 6

Hypogammaglobulinemia: causes infection–most common cause of death
Autoimmune hemolytic anemia
Transformation to diffuse large B cell lymphoma (Richter transformation) presents as enlarging lymph node or spleen

Question 7

What should you be worried about if a patient with CLL starts to have an enlarging lymph node or spleen?

Answer 7

Richter transformation

This is a transformation to diffuse large B cell lymphoma

Question 8

Hairy cell leukemia

Answer 8

Type of CLL
Proliferation of mature B cells
Characterized by hairy cytoplasmic processes
Cells positive for tartate-resistant acid phosphate (TRAP)
Clinical: splenomegaly from hairy cell accumulation in red-pulp, “dry tap” marrow aspiration from marrow fibrosis, LAD generally absent
Excellent response to 2-CDA (cladribine), an adenosine deaminase inhibitor so adenosine accumulates in neoplastic B cells

Question 9

Adult T-cell Leukemia/Lymphoma

Answer 9

Type of CLL
Neoplastic mature CD4+ T cells
Assoc. c HTLV-1, common in Japan and Caribbean
Clinical: rash, generalized LAD, HSM, lytic (punched out) bone lesions with hypercalcemia

Question 10

Mycosis Fungoides

Answer 10

Neoplastic mature CD4+ T cells infiltrate the skin causing localized rash, plaques, and nodules
Aggregates of cells in the epidermis are called Pautrier microabscesses
Cells can spread to blood causing Sezary syndrome
Characteristic lymphocytes with cerebriform nuclei (Sezary Cells) on blood smear

Question 11

What form of CLL has cerebriform nuclei?

Answer 11

Mycosis Fungoides

These are called Sezary Cells

Question 12

What leukemia has smudge cells?

Answer 12

CLL