Lymphomas

Question 1

lymphoma

Answer 1

malignancy of lymphoid tx

**3rd most common malignancy in childhood

Question 2

Etiology of lymphoma

Answer 2

• Etiology unknown
• Epstein-Barr virus may play a role
  o Hodgkin disease
  o Non-Hodgkin lymphoma

Question 3

Hodgkin DZ

Answer 3

association w/ EBV

Question 4

Hodgkin DZ epidemiology

Answer 4

Bimodal peaks
o	Adolescence / young adult years (15-30)
(Rare before age 10)
o	Age 50+
o	Boys more than girls in childhood

Question 5

CF of Hodgkin DZ

Answer 5

o Painless, rubbery, cervical lymphadenopathy

• Often supraclavicular and cervical areas
• Pain in the affected node after alcohol ingestion may occur

o Mediastinal mass causing cough or SOB

Question 6

What are the “B” sx?

Answer 6

fever higher than 38°C for 3 consecutive days (w/o infxn)

drenching night sweats, unexplained weight loss > 10% during the prior 6 months

Question 7

dx of hodgkin’s lymphoma

Answer 7

o Tissue biopsy
o Pleural or peritoneal fluid evaluation may be helpful
o May be accompanied by fever, weight loss
o Staged by Ann Arbor system

Question 8

what is the pathologic hallmark of Hodgkin’s lymphoma?

Answer 8

Reed-Sternberg Cells

Question 9

TX of Hodgkin’s

Answer 9

o Combination of chemotherapy and low-dose, involved field radiation therapy

Question 10

Prognosis of Hodgkin’s?

Answer 10

o Three risk groups

• Low, medium, high
• Excellent prognosis
• Overall survival rate 90%
• Close to 100% in low-risk patients

Question 11

Non-Hodgkin Lymphoma (NHL)

Answer 11

• Neoplastic proliferation of immature lymphoid cells (unlike malignant lymphoid cells of ALL, they accumulate outside the bone marrow)

Question 12

what is NHL associated w/?

Answer 12

• Associated with congenital or acquired immunodeficiency states
  o B-cell
  o T-cell
  o Large-Cell (Burkitt Lymphoma)

Question 13

epidemiology of NHL

Answer 13

• Incidence increases with age
  o More common in whites than African-Americans
  o More common in males than females
  o Peaks between 7-11 years old

Question 14

Clinical Findings of NHL

Answer 14

o Lymphadenopathy
o Sometimes abdominal pain
o Systemic symptoms (fever, night sweats, weight loss, etc) occur but are less common than with Hodgkin’s

isolated lymph nodes, and ski

Question 15

what are common extralymphatic sites of NHL?

Answer 15

GI tract, skin, bone, bone marrow

Question 16

sx of B cell involvement in NHL

Answer 16

involves bone, isolated lymph nodes and skin

Question 17

sx of T cell involvemnt

Answer 17

mediastinal mass (think potential SVC syndrome)

Question 18

Burkitt and NHL?

Answer 18

rapidly expanding jaw lesion, usually carries EBV

*** less likely to present w/ abdominal fullness

Question 19

dx eval of NHL

Answer 19

tissue bx, serum LDH is useful

Question 20

tx of NHL

Answer 20

o Distant noncontinuous metastases common
o Systemic chemotherapy mandatory
o Highly chemo-sensitive

Question 21

prognosis of NHL

Answer 21

related to stage

overall 3 yr survival rates 70-90%

Question 22

Polycythemia Vera

Answer 22

slowly progressive bone marrow disorder characterized by increased numbers of RBC and increasd total blood volume

Question 23

what is diagnostic criteira of polycythemia vera?

Answer 23

• JAK2 mutation!

- splenomegaly, normal arterial oxygen saturation, and an elevated red cell mass

Question 24

PP of PCV?

Answer 24

• Unregulated expansion of red cell mass causes hyperviscosity, which leads to decreased cerebral blood flow

Question 25

what are secondary causes of PCV>

Answer 25

include chronic hypoxia, often caused by cigarette smoking, and renal tumors

Question 26

what are complications of PCV?

Answer 26

- thrombosis | - bleeding, pUD, GI bleed

Question 27

epidemiology of PCV

Answer 27

- Median age at presentation is 60 years, and 60% of patients are male. Median survival time for patients with polycythemia vera is 11-15 years

Question 28

what can PCV convert to overtime?

Answer 28

myelofibrosis, CML, or rarely AML

Question 29

SX of PCV

Answer 29

increased blood viscosity and volume (i.e., headache, dizziness, fullness in the head and face, weakness, fatigue, tinnitus, blurred vision), burning pain, and redness of the extremities. Rarely stroke

Question 30

what is a classic sx of PCV?

Answer 30

pruritis after bathing

Question 31

what can be the most common presenting complaint?

Answer 31

epistaxis

Question 32

PE findings of PCV?

Answer 32

systolic hypertension, engorged retinal veins, and splenomegaly

Question 33

what is the most common complication of PCV?

Answer 33

Thrombosis

Question 34

what does absence of splenomegaly suggest?

Answer 34

secondary polycythemia

Question 35

laboratory findings of PCV

Answer 35

o At sea level, hematocrit levels in polycythemia vera are typically greater than 54% in males and greater than 51% in females EPO levels are low, but red cell morphology is normal hyperuricemia can also develop

Question 36

peripheral smear of PCV

Answer 36

neutrophilic leukocytosis, increased basophils and eosinophils, and increased numbers of large, bizarre platelets

Question 37

tx of PCV

Answer 37

phlebotomy!!! myelosuppressive tx w/ hydroxyurea may be indicated low dose ASA reduces risk of thrombosis