Lecture 38 - Non-Hodgkins, MGUS, and Multiple Myeloma

Question 1

Unlike with Hodgkin Lymphoma, the tumor cell mass in Non-Hodgkin is comprised mostly of _____ cells.

Answer 1

Neoplastic

Question 2

_____ Non-Hodgkin lymphoma typically occurs in the elderly. Characteristic cells are small with _____ nuclei and are CD__+, BCL___+, and BCL___+ (associated with rearrangement of chromosomes 14 and 18). BM biopsy will show ______ lymphoid aggregates.

Answer 2

Follicular

Cleaved nuclei

CD10+

BCL6+

BCL2+

Paratrabecular

Question 3

_____ cell Non-Hodgkin Lymphma occurs mostly in elderly males. These patients may show a translocation between chromosomes 11 and 14, leading to high level expression of BCL__ (aka Cyclin___). These cells are also CD5+. Keep in mind CLL presents basically the same, but it will not be BCL__+.

Answer 3

Mantle cell Non-Hodgkin Lymphoma

BCL1 (aka CyclinD1)

BCL1+

Question 4

Diffuse large B-cell Non-Hodgkin Lymphoma patients typically present with a rapidly enlarging mass at a _____ site. The B-cells in this disease are about 3-4 times _____ than resting lymphocytes –> this is what differentiates it from ______ lymphoma, because both can be CD___+, BCL___+, and BCL___+.

Keep in mind Diffuse Large B-cell patients who show a double hit (MYC and BCL___ or BCL___) or are double expressors (MYC and BCL___+) cannot be treated effectively.

Answer 4

Single site

Larger

Follicular

CD10+

BCL6+

BCL2+

BCL6 or BCL2

BCL2+

Question 5

Burkitt Lymphoma is associated with _____ infections, and the disease usually arises at _____ sites. Keep in mind there are 3 subtypes of Burkitt:

1. Endemic –> occurs in sub-Saharan Africa and patients present with _____ or _____ masses.
2. Sporadic –> seen in the US and is NOT associated with ____ infection. These patients present with large ______ tumors.
3. Immunodeficiency-associated –> typically in patients with _____.

The gold standard for identification of Burkitt lymphoma is c-Myc+ that results from rearrangement of chromosomes ____ and ____. Also look out for a “____ ____” appearance on hematopathology, with cells showing round nuclei and 3-4 _____.

Answer 5

EBV infections

Extranodal

1. Maxillary or Mandibular
2. EBV infection
   Abdominal tumors
3. HIV

8 and 14

Starry Night

Nucleoli

Question 6

Extranodal marginal zone lymphoma/MALToma is more common in _____ (men or women?). Patients typically present with swelling of the ______ glands, thyroid, or orbit. These malignancies originate in marginal zone B-cells of MALT, so it should make sense in 70% of cases you’ll see a tumor in the ______. These B-cells are CD___- and CD____-, which supports the diagnosis. Patients with an 11;18 translocation are not responsive to _______ treatment, which is an issue because ___ ______ infection is ALWAY associated with the MALT aspect of this cancer.

Answer 6

Women

Salivary

Stomach

CD5- and CD10-

Antibiotics

H. pylori

Question 7

Anaplastic large cell lymphoma involves which lymphocytes?

It is more common in young adults in their teens/20s and presents in lymph nodes, ___ (most common), and other extranodal sites. If it is nodal (in the lymph nodes), which area of the lymph node do you expect to see it?

The affected cells in this lymphoma appear large, with _____ shaped nuclei. These cells are CD30+, ____5-, and CD45+. A __;__ translocation can be used diagnostically.

Answer 7

T-cells

Skin (raised red lesion)

Sinusoidal area

Horseshoe shaped nuclei

Pax5-

2;5 translocation

Question 8

Mycosis Fungoides also affects ___-cells and has a slow indolent course over decades. Patients present with flat, red, scaly patches on their _____ (generally, the cancer does not spread beyond the ____). The progression of this cancer shows three stages: ___-___-___. The cells in this cancer show markers CD___+ and CD___-. Keep in mind cells in _____ syndrome will have the same markers, but they will have a cerebraform appearance. Patients will present with diffuse skin rash (braille looking skin)

Answer 8

T-cells

Skin

Skin

Patch-plaque-tumor

CD4+

CD7-

Szary Syndrome

Question 9

Monoclonal Gammopathy is a ____ cell disorder. It can be identified by monoclonal immunoglobulin (___ protein) in blood and immunoglobulin light chain in urine (___ ____ protein.)

Answer 9

Plasma cell

M protein

Bence Jones Protein

Question 10

MGUS, Smoldering Myeloma, and Multiple Myeloma are all forms of Plasma cell disorders.

MGUS is the least severe, with M protein levels at < ___ g/dL, absence of end organ damage, and < ___% plasma cells in the BM.

Smoldering myeloma shows M protein levels > ___ g/dL and 10-60% plasma cells in BM, but end organ damage is also ABSENT.

Multiple Myeloma is similar to Smoldering myeloma, but it’s important to look in the urine for ___ ___ protein, and end organ damage WILL be observable.

Answer 10

< 3g/dL

< 10%

> 3g/dL

Bence Jones protein

Question 11

Serum Protein Electrophoresis is used to detect ___ protein, but a Serum Immunofixation must be used to determine which Ig it is.

Answer 11

M protein