Chronic Leukemias and Plasma Cell Disorders

Question 1

What are the 3 main chronic leukemias?

Answer 1

Chronic Lymphocytic Leukemia
Hairy cell leukemia
Adult T cell Leukemia/Lymphoma

Question 2

Chronic Lymphocytic Leukemia (CLL)

In what parts of the body does it happen?

Answer 2

Involves blood, bone marrow, and lymph nodes

Question 3

Chronic Lymphocytic Leukemia (CLL)

What patients does this tend to happen in?

Answer 3

Elderly patients

May have immune dysfunction and hypogammaglobulinemia

Question 4

Chronic Lymphocytic Leukemia (CLL)

Pathogenesis

Answer 4

You get a ton of abnormal B cells that are useless in fighting infection. They suppress normal B cell proliferation.

May also lead to autoimmune hemolytic anemia

Question 5

Chronic Lymphocytic Leukemia (CLL)

How can you assess prognosis?

Answer 5

Look for mutations in the IGH gene

Those with IGH mutations have a favorable outcome

Question 6

Chronic Lymphocytic Leukemia (CLL)

What do the cells look like?

Answer 6

Mostly small round lymphocytes with condensed chromatin

May also see Smudge cells due to increased fragility (their appearance is an artifact of the blood smear)

Question 7

Hairy Cell Leukemia

How do the cells appear?

Answer 7

They have cytoplasmic projections (hair-like)

Question 8

Hairy Cell Leukemia

What are some usual presenting symptoms?

Answer 8

Pancytopenia
Splenomegaly (red pulp expansion)
Hepatomegaly
Infections

Question 9

Hairy Cell Leukemia

What cytochemical stain should you use for diagnosis?

Answer 9

TRAP

Tartrate Resistant Acid Phosphatase

Question 10

Hairy Cell Leukemia

Prognosis

Answer 10

Excellent response to gentle chemo

Question 11

Adult T Cell Leukemia/Lymphoma

What type of cells tend to be involved? What characteristic appearance will they maintain?

Answer 11

CD4+ T cells (they will have convoluted nuclei with a floret shape)

Question 12

Adult T Cell Leukemia/Lymphoma

What infection is it commonly associated with?

Answer 12

HTLV-1 infection

Question 13

What is the most common plasma cell disorder?

Answer 13

MGUS

Monoclonal Gammopathy of Undertermined Significance

Question 14

Multiple Myeloma

What is the basic pathogenesis?

Answer 14

Monoclonal plasma cell proliferation involving the bone marrow and typically the skeleton at many sites

Question 15

Multiple Myeloma

What drives the proliferation of plasma cells?

Answer 15

Cytokines, like IL-6

Question 16

Multiple Myeloma

Diagnostic Criteria

Answer 16

M protein - monoclonal protein in serum or urine (IgG or IgA)
Plasmacytoma (tissue based tumor of plasma cells)

CRAB
Hypercalcemia
Renal insufficiency
Anemia
Bone lesions (lytic)

Question 17

Multiple Myeloma

What lab tests would you run to assess? What would you see in normal? Multiple myeloma?

Answer 17

Serum protein electrophoresis

Normal - smear due to many Abs of many sizes

Multiple Myeloma- one sharp band due to monoclonal expansion

You may run a second step with antibody binding to see the isotype of the heavy and light chains.

Question 18

Multiple Myeloma

Why does the patient get hypercalcemia?

Answer 18

Bone resorption (also leads to more fractures)

Question 19

Multiple Myeloma

Why does the patient get infections?

Answer 19

Recurrent infections occur because they are only making one type of Ab

Question 20

Multiple Myeloma

What is Bence-Jones proteinuria?

Answer 20

Light chains are toxic to the tubular epithelium, eventually lead to proteinuria

Question 21

Monoclonal Gammopathy of Undertermined Significance (MGUS)

How should you treat them?

Answer 21

Don’t treat them, just follow them over time and analyze with serum protein electrophoresis and renal function assessment. You should begin treatment once the monoclonal protein levels start to increase