Clinical Lymphoma

Question 1

Systemic manifestations of lymphoma include ___ (aka “B symptoms)

Local manifestations of lymphoma include __and __ (manifests as early satiety)

Answer 1

Systemic manifestations of lymphoma include fever, night sweats, unintentional weight loss, anorexia, pruritis

Local manifestations of lymphoma include lymphadenopathy and splenomegaly (manifests as early satiety)

Question 2

T/F: You never do a fine needle aspiration to diagnose lymphoma

Answer 2

True. FNA doesn’t preserve the lymph node architecture such that a definitive diagnosis can’t be made.

**Dx is done with:

Excisional biopsy >> demonstrates nodal architecture

Immunohistochemistry >> confirm lymphoid origin of cells/demonstrate clonality with kappa/lambda staining

Flow cytometry

Question 3

Lymphoma can be staged using the Ann Arbor staging system. Describe the different stages 1 thru 4

Answer 3

Stage I: one lymph node above or below the diaphragm

Stage II: two lymph nodes on either side of the diaphragm (either above or below)

Stage III: lymph nodes above and below the diaphgram

Stage IV: extranodal involvement

Question 4

Patients with Hodgkin’s lymphoma can have painless lymphadenopathy, characteristic ___ and ___ (with alcohol consumption)

Answer 4

Patients with Hodgkin’s lymphoma can have painless lymphadenopathy, characteristic pruritis (due to cytokine release) and severe pain (with alcohol consumption) - due to vasodilation within lymph node capsule

Question 5

Treatment of Hodgkin lymphoma consists of the ABVD regimen +/- radiation. Which drugs are part of this regimen?

Answer 5

Adriamycin

Bleomycin

Vinblastine

Dacarbazine

Question 6

What are the side effects of the ABVD drugs?

Answer 6

Adriamycin (aka Doxorubicin) >> cardiotoxicity

Bleomycin >> pulmonary fibrosis

Vinblastine >> severe bone marrow suppression (+ neuropathy but that’s more the other drug)

Da carbizine >> da cytopenias

Question 7

For relapsed disease, pts with Hodgkin Lymphoma can be given salvage chemotherapy (which does what?), ___ (a new mAb) and immunotherapy targeting ___ in Reed-Sternberg cells

Answer 7

For relapsed disease, pts with Hodgkin Lymphoma can be given salvage chemotherapy (which resuces pt from effects of chemo), Brentuximab (a new mAb) and immunotherapy targeting PD-L1 in Reed-Sternberg cells

Question 8

Brentuximab targets ___ which is expressed on the surface of Reed-Sternberg cells. The antibody is conjugated to a chemo agent ___ which directly targets ___ cells

Answer 8

Brentuximab targets CD30 which is expressed on the surface of Reed-Sternberg cells. The antibody is conjugated to a chemo agent (MMAE) which directly targets CD30+ cells

Question 9

Diffuse large B cell lymphoma often presents as a bulky tumor mass, ___ and presence of ___ symptoms. DLBCL also expresses what CD markers? (hint: its the usual for B cell cancers + a new one in this lecture)

Answer 9

Diffuse large B cell lymphoma often presents as a bulky tumor mass, extranodal disease and presence of B symptoms. DLBCL also expresses CD19, CD20 and CD79a

Question 10

The treatment of DLBCL is ___ (hint: the acronym doesn’t match with the letters)

Answer 10

Rx for DLBCL is R-CHOP:

• Rituximab
• Cyclophosphamide
• Dox- HO -rubicin
• Vincristine (vin-cristOned)
• Prednisone

Question 11

What are the side effects of the R-CHOP drugs?

Answer 11

Rituximab (anti-CD20; can cause tumor lysis syndrome/cytokine release syndrome/myelosuppression/infection)

Cyclophosphamide >> cytopenias

Doxorubicin >> cardiotoxicity, alopecia

Vincristine >> neuropathy

Prednisone

Question 12

Patients with HIV, adrenal/kidney/testicular/breast involvement with DLBCL usually get ___ in addition to systemic therapy

Answer 12

Patients with HIV, adrenal/kidney/testicular/breast involvement with DLBCL usually get prophylactic intrathecal chemotherapy in addition to systemic therapy

Question 13

A novel therapy for DLBCL involves the use of CAR-T therapy. What are 2 side effects of this therapy?

Answer 13

Cytokine release syndrome

Neurotoxicity

Question 14

Primary mediastinal large B-cell lymphoma is a subset of DLBCL that presents with ___ typically in young female patients. What is the Rx of this disease?

Answer 14

Primary mediastinal large B-cell lymphoma is a subset of DLBCL that presents with large mediastinal mass typically in young female patients. (comes from thymic medullary B cells)

Rx = infusion chemotherapy sans radiation due to long term side effects

Question 15

Burkitt lymphoma has a high incidence of __ and can be treated with ___

Answer 15

Burkitt lymphoma has a high incidence of tumor lysis syndrome and can be treated with infusional chemotherapy

Question 16

Follicular lymphoma is incurable so Rx is to manage the disease. Two treatment regimens for FL are ___ and ___ (hint: both involve rituximab)

Answer 16

Follicular lymphoma is incurable so Rx is to manage the disease. Two treatment regimens for FL are bendamustine + rituximab, and rituximab + revlimid

Question 17

For relapsed follicular lymphoma, pts get targeted therapy in the form of ___

Answer 17

PI3K inhibitors

Question 18

An absolute lymphocyte count over ___ is indicative of CLL

Answer 18

An absolute lymphocyte count over >5000 is indicative of CLL

Question 19

For CLL/SLL, the presence of ___ (genetic abnormality) indicates high risk, aggressive disease

Answer 19

For CLL/SLL, the presence of del(17p) involving p53 indicates high risk, aggressive disease

Question 20

CLL/SLL uses a disctint Rai staging system. Describe the *technically* 5 stages of this system

Answer 20

Stage 0 – only peripheral blood leukocytosis

Stage I – lymphocytosis + lymphadenopathy

Stage II – lymphocytosis + hepatosplenomegaly

Stage III – Lymphocytosis + anemia

Stage IV – lymphocytosis + thrombocytopenia

Question 21

In general, CLL/SLL can be treated with chemotherapy or targeted therapy.

The FCR regimen (fludarabine, cyclophosphamide and rituximab) is given to __ patients while the BR regimen (___ + ___) is given to older pts

Answer 21

In general, CLL/SLL can be treated with chemotherapy or targeted therapy.

The FCR regimen (fludarabine, cyclophosphamide and rituximab) is given to younger patients while the BR regimen (bendamustine + rituximab) is given to older pts

Question 22

Targeted therapy can also be used to treat CLL/SLL. What are 3 drugs used to treat this and what do they do?

Answer 22

Ibrutinib >> Bruton’s tyrosine kinase (BTK) inhibitor

Idelalisib >> PI3K inhibitor

Venetoclax >> BCL2 inhibitor

Question 23

Splenic marginal zone lymphoma presents with very bad ___ and is strongly ass’d with ___ infection (hint: its one of the Hepatitis viruses)

MALT lymphoma is ass’d with H pylori and ___ infection (leads to ocular adnexa – basically a lymphoma behind the eye)

Answer 23

Splenic marginal zone lymphoma presents with very bad splenomegaly and is strongly ass’d with Hep C infection

MALT lymphoma is ass’d with H pylori and C psittaci infection (leads to ocular adnexa – basically a lymphoma behind the eye)

**for H pylori mediated MALT lymphoma, can also give Rituximab as an alt to antibiotics**

Question 24

Which T cell NHL is associated with HTLV-1 infection and commonly presents with visceral involvement, hypercalcemia, skin lesions, osseous involvement?

Answer 24

Adult T-cell leukemia/lymphoma (T-ALL)

Question 25

___ (lymphoma type) is ass’d with EBV infection and commonly involves the nasopharynx

Answer 25

Extranodal NK/T-cell lymphoma is ass’d with EBV infection and commonly involves the nasopharynx

Question 26

Anaplastic large T cell lymphoma (type of T cell NHL) is characterized by ___ involvement + ___ translocation

Answer 26

Anaplastic large T cell lymphoma (type of T cell NHL) is characterized by visceral involvement + ALK t(2;5) translocation

Rx is with brentuximab (anti-CD30) and CHP chemo

Question 27

Peripheral T cell lymphoma not otherwise specified is a group of heterogeneous diseases that can be treated w/ ___

Answer 27

Peripheral T cell lymphoma not otherwise specified is a group of heterogeneous diseases that can be treated w/ CHOP chemo (note that there’s no R here because that’s for the B cells and this is a T cell disorder)

Question 28

___ is a malignant T cell infiltration of the skin. Rx involves topical or phototherapy or retinoid therapy with ___

Answer 28

Mycosis fungoides is a malignant T cell infiltration of the skin. Rx involves topical or phototherapy or retinoid therapy with Bexarotene

Question 29

___ is characterized by circulating mycosis fungoides cells in the blood and can be treated with Mogamulizumab

Answer 29

Sezary syndrome is characterized by circulating mycosis fungoides cells in the blood and can be treated with Mogamulizumab (anti-CCR4 antibody)