Myelodysplasias, Leukemia & Multiple Myeloma

Question 1

what has proliferation WITHOUT differentiation?

Answer 1

Acute myeloid leukemia
acute lymphoblastic leukemia

Question 2

increased proliferation WITH differentiation?

Answer 2

chronic myeloid leukemia
polycythemia vera- red cells
essential thrombocythemia- platelets
myelofibrosis- stromal tissue

Question 3

clinical features of acute leukemia?

Answer 3

• anemia (pallor, fatigue, dyspnea)
  neutropenia (fever, infections)
  thrombocytopenia (bruises, bleeding)
• organ infiltration
  bone pain, lymphadenopathy, meningeal signs, testicular swelling, skin rash, pulmonary infiltrates

Question 4

• often patients are asymptomatic at diagnosis
• marked leukocytosis common: may cause
  weight loss, massive splenomegaly, gout, anemia
• thrombocytosis, may cause:
  unusual clotting or bleeding

Answer 4

chronic myeloid leukemia

Question 5

Defined as increased red cell volume
JAK 2 present in 100%

Answer 5

polycythemia vera

Question 6

secondary polycythemia vera symptoms

Answer 6

hypoxia, sleep apnea, smoking, tumors that secrete erythropoitin

Question 7

treatment of polycythemia vera

Answer 7

Reduce hematocrit
* phlebotomy
* hydroxurea

Prevent thrombosis
* aspirin daily
* keep platelet count under 400/ul

Question 8

defined by increased platelet count
JAK 2 present 50% of the time
most common clinical features: abnormal clotting, abnormal bleeding, erythromelalgia (burning sensation in the hands)

Answer 8

essential thrombocytopenia

Question 9

treatment of essential thrombocytemia?

Answer 9

reduce platelet count
* hydroxyurea
* anagrelide

Prevent thrombosis
* reduce risk factors (smoking, hypertension)
* aspirin

Question 10

• defined by fibrosis of the bone marrow
• JAK 2 present in 50%
• often with massive splenomegaly
• blood smear is characteristic- teardrop
• anemia, elevated WBC, platelets early
• anemia, lower WBC, platelets late
• worst pronosis of 3 MPD

Answer 10

myelofibrosis

Question 11

treatment of myelofibrosis?

Answer 11

improve hemoglobin
* transfusions, erythropoietin, androgens, thalidomide

control WBC, platelets, hypermetabolism
* hydroxyurea

control spleen size
* ruxolitnib, hydroxyurea, radiation, surgery

cure
* hematopoietic cell transplantation

Question 12

• pancytopenia (diverse presentation)
  anemia, thrombocytopenia neutropenia
• ineffective hematopoiesis
  paradox of hypercellular marrow, but low blood counts
• caused by stem cell damage (risk increases with age)
• potential evolution to AML
  for many but not all

Answer 12

Myelodysplastic syndrome

Question 13

treatment options for lower risk MDS? high risk?

Answer 13

low risk
* observation
* growth factors

high risk
* chemotherapy
* stem cell transplant

Question 14

clonal growth leads to bone marrow infiltration (like leukemia) and overproduction of non-functioning antibodies

Answer 14

myeloma

Question 15

clinical features of myeloma

Answer 15

proteinurina
monoclonal protein in blood/and or urine
lytic bone lesions and fractures
anemia
recurrent infections
renal failure
hypercalcemia

Question 16

diagnosis of myeloma

Answer 16

• bone marrow with 10% clonal plasma cells (or plasmacytoma outside of the marrow)
• monoclonal protein in the blood and or urine
• myeloma related end organ damage (CRAB criteria)
  hypercalcemia
  renal insufficiency
  anemia
  bone lesions