Putthoff/Robbins 3

Question 1

What are the 5 plasma cell neoplasms/disorders?

Answer 1

MM, LL, Heavy Chain Disease, Primary or immunocyte associated amyloidosis, and MGUS.

Question 2

What is the phenomenon in MM with RBCs?

Answer 2

Rouleaux formation of RBCs

Question 3

What does definitive diagnosis of MM require? What are 3 specific things we need for diagnosis?

Answer 3

BM examination

BM shows greater than 30% plasma cells with atypia
Greater than 3 gm/dL of Ig
More than 6 mg/dL of urinary bence jones proteins

Question 4

Two types of heavy chain disease and what is significant to remember about each one?

Answer 4

Most common form is IgA or alpha. GI tract involvement

Second most common form is IgG or Franklin disease/gamma. Palatal edema.

Question 5

What are the two variants of MM? How many of these cats progress to MM?

Answer 5

Plasmacytoma which is more of a mass in bone almost always progresses to MM. Mass outside of bone can be respected.
Smoldering myeloma is defined by a lack of symptoms but still has the M spike. 75% progress to MM

Question 6

What is a clinical feature that MM shares with LL?

Answer 6

Hyper viscosity

Question 7

Two CD markers for MM, but also plasma cell tumors in general?

Answer 7

CD138 and CD56

Question 8

What is the most common plasma cell disorder?

Answer 8

MGUS

Question 9

What patient population is MGUS commonly seen in and how often do these guys progress to MM? By definition, what is MGUS?

Answer 9

5% of 70 year olds
1% per year progress to MM
Asymptomatic and less than 3gm of M protein

Question 10

Virtually all cases of lymphoplasmacytic lymphoma are associated with what mutation?

Answer 10

Acquired MYD88

Question 11

What two things are not commonly seen in LL?

Answer 11

Bone lesions and secretion of Ig light chains

Question 12

3 clinical signs/symptoms found in mantle cell lymphoma? How is the prognosis of Mantle cell?

Answer 12

Painless LAD, spleen/BM/liver and then GI

Poor

Question 13

What is the unusual phenomenon found in mantle cell lymphomas?

Answer 13

Lympomatoid polyposis

Question 14

What is the specific transformation associated with marginal zone lymphoma?

Answer 14

Polyclonal to monoclonal

Question 15

What other lymphomas follow this polyclonal to monoclonal transformation?

Answer 15

EBV induced lymphomas

Question 16

What is the classic patient with hair cell leukemia?

Answer 16

Middle aged white male

Question 17

What is the most common symptom of hairy cell leukemia?
What are three other symptoms that can commonly arise as well?
Prognosis of Hairy?

Answer 17

Massive spleen
Pancytopenia, splenic dysfunction and infections…commonly atypical mycobacterial ones
Excellent

Question 18

Where are peripheral T cell tumors most common in the world?

Answer 18

Asia

Question 19

What are the 6 peripheral T cell and NK cell neoplasms?

Answer 19

Peripheral T cell lymphoma
Anaplastic Large Cell Lymphoma
Adult T cell leukemia
Mycosis fungus
Large granular lymphocytic leukemia
Extranodal NK/t cell lymphoma

Question 20

What is the mutation in anaplastic large cell lymphomas, what is the typical patient, how does the tumor present, and what is the prognosis?

Answer 20

ALK gene mutaiton
Children and young adults
Soft tissue masses
Very good prognosis even though T cell lymphomas typically have horrible prognosis

Question 21

What CD marker is anaplastic?

Answer 21

30+

Question 22

Extranodal NK/T cell lymphoma is highly associated with what?

Answer 22

EBV

Question 23

What is the most common place anaplastic large cell lymphomas like to cluster? Where do extranodal NKt cell lymphomas like to invade and cause?

Answer 23

Venules and sinuses

Small vessels and necrosis

Question 24

What is the most common cancer diagnosed in teens 15-19?

Answer 24

HL

Question 25

Nodular sclerosis is not commonly associated with what? Mixed cellularity is commonly associated with what? Lymphocyte rich is associated with what in 40% of cases? Which HL type is associated with EBV over 90% of the time? Which HL type is found in HIV patients most of the time? Which type is EBV not associated with?

Answer 25

EBV
EBV
EBV
Lymphocyte depleted
Lymphocyte depleted
Lymphocyte predominance