Chronic Leukemias

Question 1

Plasma cell dyscrasias

Answer 1

Neoplastic plasma cells that almost always secrete monoclonal immunoglobulin
- serve as tumor markers

Question 2

Most deadly plasma cell dyscrasias

Answer 2

Multiple myeloma

Question 3

M protein

Answer 3

Monoclonal immunoglobulin in reference to a myeloma

• complete M proteins are very heavy and are restricted to plasma/extracellular fluid. Are also excluded from urine unless kidney is damaged
• often contain excess immunoglobulin light chains*

Question 4

Terms used to describe abnormal IGs associated with plasma cell neoplasms

Answer 4

Monoclonal gammopathy

Dysproteinemia

Paraproteinemia

Question 5

Clinicopathologic entities associated with M proteins

Answer 5

Multiple myeloma

Monoclonal gammopathy of undetermined significance (MGUS)

Primary amyloidosis

Waldenstrom macroglobulinemia

Question 6

How to differentiate CLL from SLL?

Answer 6

Check peripheral blood lymphocyte counts

> 5000 cells/uL = CLL

<5000 cells/uL = SLL

Question 7

Richter transformation

Answer 7

Occurs in aggressive forms of CLL

Transform tumor cells into similar to DLBCL’s

Much more aggressive and kill quicker (<1yr)

Question 8

What diseases can produce M proteins?

Answer 8

Multiple myeloma

MGUS

Primary amyloidosis

Waldenstrom macroglobulinemia

Question 9

Multiple myeloma immunophenotype

Answer 9

Lyric lesions throughout bone

Numerous plasma cells in bone marrow with cytoplasmic droplets

lots of IgG and IgA (mostly IgA)

Produces bence jones and the above M proteins

High levels of cyclin D1 and D3 genes

Question 10

Bence jones proteins

Answer 10

Light chains only aggregations generated by plasma cells

Seen in multiple myeloma

Question 11

CLL/SLL immunophenotype

Answer 11

High levels of BCL2

BTK presence

Smudge cells and pro-lymphocyte are present

CD5 and CD20

Trisomy 12 is common as well as the following deletions
11, 13 and 17

Question 12

Hairy cell leukemia immunophenotype

Answer 12

B cells w/ pale blue, agranular cytoplasm, hairlike cytoplasmic projections

BRAF activation

CD20, CD11c and CD103

Question 13

What is the most immature cell in the myeloid series?

Answer 13

Myeloblast

Question 14

Lineage of myeloid series

Answer 14

Myeloblast (immature)

Promyelocyte (basophilic cytoplasm w/ azurophilic granules generation)

Myelocytes (specific granules for basophil, neutrophil and eosinophil generate)

Metamyelocyte ( increasing numbers of granules in the cytoplasm

Band cell (elongated nucleus but not lobed yet)

Segmented granulocytes (maturation)

Question 15

What are the primary myeloproliferative disorders

Answer 15

CML

Polycythemia Vera

Primary Myelofibrosis

Essential thrombocytosis

Question 16

CML immunophenotype

Answer 16

T(9;22)

BCR-ABL gene and proteins

Leukocyte count higher than 100,000 cells

Hypercellular megakaryocytic bone marrow

Spleen looks hypertrophic and like red bone marrow

Philadelphia chromosome is present t(9;22)

Question 17

Blast crisis

Answer 17

Last and most aggressive stage of CML marked by large amount of blast cells without an accelerated phase

Question 18

Polycythemia Vera (PV) immunophenotype

Answer 18

Activating point mutations in JAK2

Absolute increase in red cell mass

Low serum erythropoietin

Hyperceullar bone marrow w/ possible marrow fibrosis

Pancytocytosis

Question 19

Spent phase

Answer 19

Phase of PV that resembles primary Myelofibrosis

Needs to be treated with JAK2 inhibitors

Question 20

Treatments for PV

Answer 20

Phlebotomy (1st line)

JAK2 (only in spent phase)