Chapter 11

Question 1

Hodgkin’s Lymphoma

Answer 1

• represents about 30% of malignant lymphoma
• can occur across the lifespan
• Higher incidence in males with a worse prognosis
• about a 5 year survival rate in 85% of treated hodgkins

Question 2

Pathogenesis of Hodgkin’s Lymphoma

Answer 2

• malignant disorder of the lymph nodes
• characterized by reed-sternberg cells
• originates from B cells in germinal centers of lymph nodes
• usually metastasizes along contiguous lymphatic pathways

Question 3

Reed-Sternberg characteristics

Answer 3

• epstein barr virus frequently found in genome of transformed reed-sternberg cells
• usually present in single node or localized node chain
• constitute about 2% of cells in lymph node tumor

Question 4

Types of Hodgkins

Answer 4

• rare lymphocyte predominance type (5%)

- classical type (cHD)(95%)

Question 5

Clinical manifestations of Hodgkin’s Disease

Answer 5

• depend on origin site and dissemination stage ( early stages are often asypmtomatic)
• painless lymphadenopathy (abnormally sized lymph nodes)
• possibly with fever, night sweats, pruritus (itching), weight loss, malaise (discomfort)
• lymph node enlargement above diaphragm, cervival nodes are the most common site
• nodes below the diaphragm less common primary site
• disease spreads from site of origin to other lymph nodes/lymphatic tissue

Question 6

Prognosis of Hodgkins Disease

Answer 6

• Ann Arbor staging system
• uses presence/absence of certain clinical systems AND locations of affected nodes
• 4 stages and 4 modifying characteristics

Question 7

Treatment of Hodgkins Disease

Answer 7

• stages dictate treatment modality
• localized tumor: radiation therapy
• Disseminated disease: chemotherapy

Question 8

Non Hodgkin’s Lymphoma

Answer 8

• do not have Reed - Sternberg Cells
• Majority arise from lymph nodes, but can originate in any lymphoid tissue
• 95% occur in older adults
• Most arise from B cells, T cells, or NK cells

Question 9

Types of Non Hodgkin’s

Answer 9

• indolent: longer survival times

- Aggressive: disseminated at presentation, generally poor prognosis

Question 10

Pathogenesis of Non Hodgkins

Answer 10

• tumor cells derived from single mutant precursor cell and are clonal
• Viruses suspected in development of some lymphomas
• 5 year survival rate 50%

Question 11

Clinical Manifestations of Non Hodgkins

Answer 11

• painless lymphadenopathy, fever, night sweats, weight loss, malaise, puritus
• extranodal involvement occurs early
• may present with infiltrative disease of the skin, GI tract, bone, or bone marrow

Question 12

Complications of Non Hodgkins

Answer 12

• two very serious: superior vena cava obstruction, spinal cord compression
• other complications: infection, bone, metastasis, joint effusions

Question 13

Staging of Non Hodgkins

Answer 13

uses same staging system as hopkins

Question 14

Treatment of Non Hodgkins

Answer 14

• radiation
• chemotherapy
• tissue specific therapies: monoclonal antibodies & BMT
• treatment determined by stage

Question 15

Hairy Cell Leukemia

Answer 15

• rare chronic type of leukemia
• 2% of adult leukemia but highly treatable
• B cell phenotype
• Peculiar cells with hair like projections on their surface

Question 16

Pathogenesis & Clinical Manifestations of Hairy Cell Leukemia

Answer 16

• hairy cells in peripheral blood
• reduced numbers in granulocytes, platelets, red blood cells
• splenomegaly (90%) (enlarged slpeen)

Question 17

Treatment of Hair Cell Leukemia

Answer 17

• begins when patient is symptomatic (enlarged spleen, recurrent infection, bleeding disorder, anemia)
• CR rate of 80% with appropriate chemotherapy protocol

Question 18

Multiple Myeloma (Plasma Cell Myeloma)

Answer 18

• malignant disorder of mature, antibody secreting B lymphocytes (plasma cells)
• occurs exclusively in adults
• men > women

Question 19

Pathogenesis of Multiple Myeloma

Answer 19

• malignant plasma cells invade bone and form multiple tumor sites
• may also target other tissues (lymph nodes, liver, spleen, kidneys)
• abnormalities in chromosome structure/number

Question 20

Clinical Manifestations of multiple Myeloma

Answer 20

• diagnosis based on monoclonal antibody peak, presence of Bence Jones Protein, Hypercalcemia, evidence of bone lesions
• bone marrow biopsy confirms diagnosis

Question 21

Bence Jones Protein

Answer 21

malignant plasma cells produce light chain antibody fragments that accumulate in blood and urine; can accumulate in kidneys and damage them

Question 22

Bone marrow Biopsy

Answer 22

• plasma cells occupt 20% to 95% of bone

- minimum of 10% to 15% for diagnosis

Question 23

Monoclonal gammopathy of undermined significance (MGUS)

Answer 23

-test for the presence of an abnormal protein (paraprotein) in the blood.

Question 24

Signs and Symptoms of Multiple Myeloma

Answer 24

• protein in urine
• high serum calcium levels
• first symptom usually bone pain
• anemia
• recurrent infections
• bleeding tendencies
• renal insufficiency (50% of cases)
• Honeycomb appearance in bones

Question 25

Treatment of Multiple Myeloma

Answer 25

• stem cell transplant
• pharmacologic management for renal dysfunction and bone pain
• Antineoplastic agents
• chemo

Question 26

Chronic Lymphoid Leukemia

Answer 26

• accounts for 30% of all leukemia cases in US
• 95% malignant B Cell precursors
• 5% associated with more aggressive T cell transformation
  \

Question 27

Symptoms of CLL

Answer 27

• fatigue, weight loss, anorexia

- increased susceptibility to infections

Question 28

Pathogenesis and Clinical Manifestations of CLL

Answer 28

• malignant lymphocytes invade lymphoid tissue and bone marrow; disrupts function
• CLL cells characterized by defective apoptosis and longer lifespan
• derived from mature peripheral B cells

Question 29

Complete Remission (CR)

Answer 29

• return to normal hematopoiesis with normal red cell, neutrophil, and platelet count; no detectable neoplastic cells
• not a cure

Question 30

Chemotherapy treatment phases

Answer 30

• remission induction: eliminate all detectable neoplastic cells and achieve CR
• Post remission or consolodation: begins after CR is attained to eliminate any undetected cells that may have escaped initial induction-phase treatment
• Remission maintenance: used to manage some neoplasms to prolong the remission interval

Question 31

Leukocytosis and Steroids

Answer 31

glucocorticoids cause an increase in WBC count, primarily PMNs