Chapter 18: Immunoproliferative Diseases

Question 1

Immunoproliferative Diseases

Answer 1

Malignancies involving lymphoid cells

Question 2

What does immunoproliferative diseases include?

Answer 2

○ Leukemias- Malignant cells in bone marrow and peripheral blood
○ Lymphomas- Malignant cells arise in lymphoid tissues
○ Plasma cell dyscrasias- Malignant or premalignant conditions of the plasma cells

Question 3

WHO classification

Answer 3

the most widely accepted system of Hematologic Malignancies

Question 4

What is the WHO system based on?

Answer 4

○ Cell lineage
○ Immunologic markers
○ Genetic features
○ Morphology and cytochemical staining

Question 5

Acute Lymphocytic Leukemia (ALL)

Answer 5

Poorly differentiated lymphoid precursor cells (blasts) in bone marrow and peripheral blood

Question 6

Pre-B-cell ALL expressing CALLA

Answer 6

○ Most common type of ALL
○ Positive for CD10
○ Most common genetic marker is TEL-AML-1

Question 7

Chronic Lymphocytic Leukemia (CLL)

Answer 7

○ Most common leukemia in adults
○ Involves small, mature lymphocytes
○ Positive for CD19 and weakly expresses CD20

Question 8

Hairy Cell Leukemia

Answer 8

○ Malignant lymphocytes with irregular cytoplasmic projections
○ Positive for CD19, CD20, CD22
○ CD103 = highly specific marker

Question 9

Hodgkin Lymphoma (HL)

Answer 9

○ Hodgkin and Reed Sternberg (RS) cells in lymph nodes and other lymphoid organs
○ Positive for CD30 and mostly positive for CD15
○ CD20 weak or absent

Question 10

Non-Hodgkin Lymphoma (NHL)

Answer 10

○ Mostly in patients older than age 60 and associated with immunosuppression
○ Heterogeneous group of neoplasms

Question 11

Plasma Cell Dyscrasias

Answer 11

○ Also known as monoclonal gammopathies
○ Disorders involving an abnormal proliferation of the plasma cells
○ Characterized by a clone of identical plasma cells and overproduction of a single immunoglobulin component

Question 12

Multiple Myeloma

Answer 12

○ Malignancy of mature plasma cells
○ CD38+, CD56+, CD138+
Lytic bone lesions produce bone pain and hypercalcemia

Question 13

Monoclonal Igs in Multiple Myeloma

Answer 13

○ Produced by clone of malignant plasma cells
○ Present in serum (greater than 3 g/dL) and/or urine
○ IgG most common (about 50% of cases) Monoclonal immunoglobulin

Question 14

Waldenström Macroglobulinemia

Answer 14

○ Malignant proliferation of IgM-producing B cells or plasma cells that infiltrate bone marrow, spleen, and lymph nodes
○ lymphoplasmacytic lymphoma
○ Monoclonal IgM in serum

Question 15

Heavy Chain Diseases

Answer 15

Rare B-cell lymphomas characterized by production of free monoclonal heavy chains

Question 16

Alpha heavy chain disease

Answer 16

○ the most common type of Heavy chain disease
○ Seen mostly in Mediterranean region
○ Lymphoma that involves MALT

Question 17

Serum Protein Electrophoresis (SPE)

Answer 17

○ Technique is used in initial identification of monoclonal Ig
○ Serum proteins are separated by size and charge
○ Most Igs migrate in gamma globulin region

Question 18

Immunosubtraction (Immunotyping)

Answer 18

○ Technique uses capillary electrophoresis to identify monoclonal Ig
○ Antisera is added to patient serum
Binding of antisera to patient’s monoclonal Ig components changes electrophoretic mobility

Question 19

Serum Free Light Chain Analysis (sFLC)

Answer 19

Automated, latex-enhanced immunoassays

Question 20

Cytogenetics

Answer 20

Traditional karyotyping and FISH are used to identify chromosome abnormalities

Question 21

Molecular Testing

Answer 21

○ More sensitive than cytogenetics
○ Used to detect: Microdeletions, Clonal rearrangements ,of Ig genes, Clonal rearrangements of TCR genes