leukemias/lymphomas 2

Question 0

What are Bence Jones proteins?

Answer 0

Free light chains (incomplete immunoglobulins) secreted by neoplastic Plasma cells in PLASMA CELL DYSCRASIAS

Can also secrete Ig

Question 1

Most common tumor arising from bone

symptoms (3)

Answer 1

Multiple Myeloma

bone pain
pathological fractures
recurrent infections due to lack of functional Ig
Renal insufficiency due to light chain accumulation

Question 2

Immunoglobulins secreted in Multiple Myeloma

Answer 2

IgG (66%)

IgA (25%)

Question 3

Difference between hemophilia A and B

Answer 3

A: factor VIII deficiency
B: factor IX deficiency

Question 4

Burkitt’s lymphoma translocation and oncogene present

Answer 4

t(8;14)

c-myc and heavy-chain IgG

Question 5

Follicular lymphoma translocation and oncogene involved

Answer 5

t(14;18) of heavy chain IgG and bcl-2

Question 6

Multiple Myeloma assocations

Answer 6

large production of IgG leading to sticky RBCs and thus...
Rouleaux formation
IgG light chains in urine (Bence Jones)
M-spike on protein electrophoresis
inhibited Calcitonin leading to...
punched out lytic bone lesions
Hypercalcemia

susceptibility to infection

Question 7

How to distinguish Multiple Myeloma from Waldenstrom’s macroglobulinemia

Answer 7

Both have M-spike, but Waldenstrom’s is only attributed to IgM
both have hyperviscosity syndrome but
Waldenstrom’s does not have lytic bone lesions

Question 8

Percentage accumulation of blasts to be defined as Acute Leukemia

Answer 8

20% in bone marrow

presents with anemia, thrombocytopenia, and neutropenia

Question 9

What is TDT

Answer 9

Distinguishes between AML and ALL

is a DNA polymerase only found in lymphoblasts (not myeloblasts, not in mature lymphocytes)

Question 10

Key marker for the presence of myeloblasts (AML)

Answer 10

Myeloperoxidase

which can cyrstallize into Auer Rods

Question 11

Translocation associated with Acute Promyelocytic Leukemia

Answer 11

t(15;17)

RAR receptor is disrupted (retanoic acid receptor)
results in accumulation of promyelocytes

Question 12

Why is promyelocytic leukemia considered a medical emergency?

Answer 12

Auer Rods (from accumulation of promyelocytes) can cause DIC

Question 13

Treatment for Acute Promyelocytic Leukemia

Answer 13

giving ATRA (All Trans Retanoic Acid) which binds RAR (retanoic acid receptor) causing promyelocytes to mature into neutrophils.

Question 14

Which myeloblasts lack MPO (myeloperoxidase)

Answer 14

megakaryoblasts

because MPO is used for oxygen dependent killing

Question 15

Down Syndrome associated with

Answer 15

before age 5: AML (megakaryoblastic)

after age 5: ALL

Question 16

What is Myelodysplastic syndrome?

Answer 16

Cytopenias with hypercellular bone marrow
Results from radiation or alkylating agents
Blasts is LESS THAN 20%
death from infection or bleeding