Lymphoma

Question 1

Lymphomas are malignant neoplasms that originate from the ___.

Answer 1

Lymphoid tissues

Question 2

What are the 2 distinct categories of lymphoma? How are they different?

Answer 2

Hodgkin and non-Hodgkin; different morphologic characteristics, clinical behavior, response to Rx

Question 3

How can you distinguish between types of lymphoma?

Answer 3

Surgical biopsy (cannot differentiate on clinical grounds)

Question 4

In the diagnosis of a suspected lymphoma, what kind of biopsy should be done and why?

Answer 4

Excisional biopsy of the entire lymph node or nodes is imperative, as the architecture has a bearing on dx and treatment

Question 5

Population typically affected by Hodgkin lymphoma?

Answer 5

May occur at any age, but generally a disease of young adults; prevalence in women peaks in the 3rd decade and then falls, while it remains fairly constant in men

Question 6

Pathologic dx findings of Hodgkin lymphoma?

Answer 6

Reed-Sternberg cells in an appropriate cellular background of reactive leukocytes and fibrosis

Question 7

What determines the subtype of Hodgkin disease?

Answer 7

Pattern of lymphocytic infiltrate

Question 8

Modern therapy has wallowed for a cure of over ___% of patients with Hodgkin lymphoma.

Answer 8

70

Question 9

Presentation of patients with Hodgkin lymphoma?

Answer 9

Most present with enlarged but painless lymph nodes, typically in the lower neck or supraclavicular region

On occasion, mediastinal masses are associated with cough or dyspnea or discovered on routine CXR

About 25% have systemic symptoms (B symptoms - weight loss, pruritus, fever, drenching night sweats)

Question 10

What is the most important prognostic factor in Hodgkin lymphoma?

Answer 10

Disease stage

Question 11

What is the major means of staging intrathoracic and intra-abdominal disease?

Answer 11

CT chest, abdomen, pelvis

Question 12

___ is also part of the staging evaluation of patients with bony symptoms or cytopenias.

Answer 12

BM biopsy

Question 13

What has recently become a standard staging tool both before treatment and at completion?

Answer 13

Fluorodeoxyglucose F 18 (FDG-PET) scan

Question 14

In the past, what was used to stage abdominal disease in Hodgkin lymphoma?

Answer 14

Staging laparotomy with splenectomy, wedge liver biopsy, and dissection of the para-aortic, iliac, splenic, hilar, and hepatic portal lymph nodes

Question 15

For patients with favorable prognosis stage I-II Hodgkin lymphoma, treatment typically involves what?

Answer 15

Combination of ABVD chemo (doxorubin, bleomycin, vinblastine, dacarbazine) in combination with involved field irradiation

(Unfavorable -> more cycles)

Question 16

For patients with stage III-IV Hodgkin lymphoma (advanced stage), treatment involves what?

Answer 16

Newer regimens include escalated BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone) and Stanford V (doxorubicin, vinblastine, mechlorethamine, vincristine bleomycin, etoposide, prednisone)

Consolidation radiotherapy may be considered with ABVD or BEACOPP;e ssential with Stanford V

Question 17

Approximately ___% of patients are refractory to initial therapy and ___% will relapse after complete remission. What are the next steps?

Answer 17

5-10; 10-30

Salvage therapy with an alternate chemo regimen

High-dose chemo and autologous HCT if early relapse (within 12 months), second relapse, or a generalized systemic relapse, even afer 12 months

Question 18

Compare the prevalence of NHL to Hodgkin vis a vis age.

Answer 18

As opposed to HL, the prevalence of NHL rises with age.

Question 19

Risk factors for NHL?

Answer 19

Congenital disorders such as ataxia-telangiectasia, Wiskott-Aldrich syndrome, celiac disease
Acquired conditions such as prior chemo or radiation, immunosuppressive therapy, EBV infection, HIV infection, HTLV-1 infection, H. pylori gastritis, Hashimoto, and Sjogren syndrome

Question 20

NHL may originate from what types of cells?

Answer 20

B, T, or histiocytes

Question 21

Presentation of NHL?

Answer 21

Classically presents as non-tender enlargement of LN, but nearly 1/3 of all cases originate outside the LN - these extranodal malignancies develop in organs that normally have nests of lymphoid tissue (muocsal surfaces, bone marrow, skin)

Question 22

What is the goal of staging evaluation in NHL?

Answer 22

Distinguish patients who have localized disease from those with disseminated disease

Question 23

After pathologic diagnosis, the staging evaluation for NHL consists of what?

Answer 23

Detailed H&P
Routine labs
BM biopsy
CT nekc, chest, abdomen, pelvis
Evaluation of CSF if diffuse large-cell NHL with bone marrow involvement, high LDH, or multiple extranodal sites of disease
FDG-PET scans

Question 24

Rx indolent localized lymphoma?

Answer 24

Radiation therapy only with curative intent

Question 25

Rx indolent disseminated lymphoma?

Answer 25

Current therapies are rarely curative, goal of treatment is palliation of symptoms; “watch and wait” approach in asymptomatic patients

Symptomatic - rituximab + alkylator chemotherapy - high response rates and can alleviate symptoms

Question 26

Rx aggressive low-risk lymphoma?

Answer 26

CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) + rituximab

Radiotherapy after chemo for areas of bulky disease

Question 27

Rx aggressive high-risk lymphoma?

Answer 27

More intensive chemo + rituximab and potentially high-dose therapy with HCT