Chapter 11- Hodgkin Lymphoma & Myeloid Neoplasms

Question 1

Hodgkin lymphoma (HL) 
Pathologic Cells

Answer 1

B cells

Question 2

Hodgkin lymphoma (HL) 
Patients

Answer 2

Bimodal age
distribution: young
adults (15-34 years)
and older adults
(>55 years)

Question 3

Hodgkin lymphoma (HL) 
Unique Features

Answer 3

More predicable than NHLs,

Reed-Sternberg cells, arises from a single node, spreads to nearby nodes in a predicable manner, painless lymphadenopathy, fever,
cachexia, pruritus, anemia.

Radiation is a risk for other cancers, previous EBV infection is associated with 70% of cases

Question 4

Hodgkin lymphoma (HL) 
Prognosis

Answer 4

Chemotherapy is very effective at managing HL, nearly all live to see
another 5 years after diagnosis and approximately 50% are cured.

Question 5

Acute myeloid leukemia
(AML)
Pathologic Cells

Answer 5

Immature myeloblasts that lose the ability to differentiate

Question 6

Acute myeloid leukemia
(AML)
Patients

Answer 6

Adults, average age

of Dx. is 50 years

Question 7

Acute myeloid leukemia
(AML)
Unique Features

Answer 7

Aggressive, various mutations stops myeloblast differentiation, myeloblasts replace marrow and suppress hematopoiesis, resembles ALL and AML (pancytopenia),
Auer rods

Question 8

Acute myeloid leukemia
(AML)
Prognosis

Answer 8

Poor prognosis even with Tx. (chemotherapy, stem cell transplant, transfusion), average long-term survival is 15-30%

Question 9

Myelodysplastic syndromes

Pathologic Cells

Answer 9

Myeloid stem cells that retain ability to differentiate

Question 10

Myelodysplastic syndromes

Patients

Answer 10

Older adults,
average age of Dx. is
age 50-70

Question 11

Myelodysplastic syndromes

Unique Features

Answer 11

Marrow is replaced by
myeloblasts that retain
capacity to differentiate,
hematopoiesis is disordered and at least one cytopenia is
found in peripheral blood, up to 40% eventually transform
into AML

Question 12

Myelodysplastic syndromes

Prognosis

Answer 12

Median survival is 9 years after diagnosis, very poor prognosis once transitioned to AML

Question 13

Chronic myeloproliferative
disorders (i-iv)
Patients

Answer 13

Adults & older adults

Question 14

Chronic myeloproliferative
disorders (i-iv)
Unique Features

Answer 14

RBC/granulocyte/platelet
proportions are abnormal with an increase of at least one whole blood cell in circulation, may progress into a spent phase, pancytopenia,
splenomegaly

Question 15

Chronic myeloproliferative
disorders
i. Chronic myelogenous
leukemia (CML)

Pathologic Cells

Answer 15

Myeloid stem cells that retain ability to differentiate

Question 16

Chronic myeloproliferative
disorders
i. Chronic myelogenous
leukemia (CML)

Patients

Answer 16

Adults, most commonly age 30-50

Question 17

Chronic myeloproliferative
disorders
i. Chronic myelogenous
leukemia (CML)

Unique Features

Answer 17

Philadelphia chromosome
t(9,22), severe granulocytosis,
severe extramedullary
hematopoiesis causes pulp of spleen to resemble marrow
with severe splenomegaly,
anemia, 50% enter into
accelerated phase (blast crisis)
resembles ALL

Question 18

Chronic myeloproliferative
disorders
i. Chronic myelogenous
leukemia (CML)

Prognosis

Answer 18

Remission is common with tyrosine
kinase inhibitor medications, 70%
cured with stem cell
transplantation, 3-year survival if transitions into aggressive leukemia (blast crisis)

Question 19

Chronic myeloproliferative
disorders
ii. Polycythemia vera

Pathologic Cells

Answer 19

RBCs, increased

production

Question 20

Chronic myeloproliferative
disorders
ii. Polycythemia vera

Patients

Answer 20

Patients with JAK2
mutations develop
PCV

Question 21

Chronic myeloproliferative
disorders
ii. Polycythemia vera

Unique Features

Answer 21

Itching skin or risk for 30% have thrombotic

complications: stroke, M.I., organ infarction (spleen, kidneys)

Question 22

Chronic myeloproliferative
disorders
ii. Polycythemia vera

Prognosis

Answer 22

PCV: low levels of EPO, patients live 1.5-3 years without medical management, but are expected to live 10-20 years after diagnosis with management

Question 23

Chronic myeloproliferative
disorders
iii. Primary myelofibrosis

Pathologic Cells

Answer 23

Myeloid stem cells that retain ability to differentiate

Question 24

Chronic myeloproliferative
disorders
iii. Primary myelofibrosis

Patients

Answer 24

Adults, > 50 years

Question 25

Chronic myeloproliferative
disorders
iii. Primary myelofibrosis

Unique Features

Answer 25

Anemia, thrombocytopenia,
poikilocytes, severe
extramedullary hematopoiesis,
massive splenomegaly,
diagnoses in the late stages

Question 26

Chronic myeloproliferative
disorders
iii. Primary myelofibrosis

Prognosis

Answer 26

Poor prognosis because it’s commonly diagnosed late: 4-5 years after Dx.

Question 27

Chronic myeloproliferative
disorders
iv. Essential
thrombocythemia

Pathologic Cells

Answer 27

Myeloid stem cells that retain ability to differentiate

Question 28

Chronic myeloproliferative
disorders
iv. Essential
thrombocythemia

Patients

Answer 28

Adults & older adults

Question 29

Chronic myeloproliferative
disorders
iv. Essential
thrombocythemia

Unique Features

Answer 29

Increased platelet production, most are asymptomatic,

splenomegaly, thrombosis or hemorrhage

Question 30

Chronic myeloproliferative
disorders
iv. Essential
thrombocythemia

Prognosis

Answer 30

No impact on lifespan with medical management of thrombocytes