Myelodysplasias, Leukemia & Multiple Myeloma Flashcards
what has proliferation WITHOUT differentiation?
Acute myeloid leukemia
acute lymphoblastic leukemia
increased proliferation WITH differentiation?
chronic myeloid leukemia
polycythemia vera- red cells
essential thrombocythemia- platelets
myelofibrosis- stromal tissue
clinical features of acute leukemia?
- anemia (pallor, fatigue, dyspnea)
neutropenia (fever, infections)
thrombocytopenia (bruises, bleeding) - organ infiltration
bone pain, lymphadenopathy, meningeal signs, testicular swelling, skin rash, pulmonary infiltrates
- often patients are asymptomatic at diagnosis
- marked leukocytosis common: may cause
weight loss, massive splenomegaly, gout, anemia - thrombocytosis, may cause:
unusual clotting or bleeding
chronic myeloid leukemia
Defined as increased red cell volume
JAK 2 present in 100%
polycythemia vera
secondary polycythemia vera symptoms
hypoxia, sleep apnea, smoking, tumors that secrete erythropoitin
treatment of polycythemia vera
Reduce hematocrit
* phlebotomy
* hydroxurea
Prevent thrombosis
* aspirin daily
* keep platelet count under 400/ul
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)
essential thrombocytopenia
treatment of essential thrombocytemia?
reduce platelet count
* hydroxyurea
* anagrelide
Prevent thrombosis
* reduce risk factors (smoking, hypertension)
* aspirin
- 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
myelofibrosis
treatment of myelofibrosis?
improve hemoglobin
* transfusions, erythropoietin, androgens, thalidomide
control WBC, platelets, hypermetabolism
* hydroxyurea
control spleen size
* ruxolitnib, hydroxyurea, radiation, surgery
cure
* hematopoietic cell transplantation
- 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
Myelodysplastic syndrome
treatment options for lower risk MDS? high risk?
low risk
* observation
* growth factors
high risk
* chemotherapy
* stem cell transplant
clonal growth leads to bone marrow infiltration (like leukemia) and overproduction of non-functioning antibodies
myeloma
clinical features of myeloma
proteinurina
monoclonal protein in blood/and or urine
lytic bone lesions and fractures
anemia
recurrent infections
renal failure
hypercalcemia
diagnosis of myeloma
- 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
