Leukemias & Myeloproliferative Diseases Flashcards
Pancytopenia
Low RBCs, WBCs, Platelets
Most childhood leukemia is what type?
Acute Lymphocytic Leukemia (ALL) - 80% of childhood leukemia is ALL but only 20% of adult leukemia is ALL
Where is the malignancy in the cell lineage in the case of ALL?
ALL is a malignancy of progenitor cells in the lymphoid lineage (the lineage leading to B and T Cells)
What causes ALL, AML?
Often exposure to chemo, radiation, benzene toxins, also unknown etiology. smoking in AML.
ALL Labs will show:
Pancytopenia w/ circulating blasts & increased blast B and T cells in the hyper cellular bone marrow; thymus will be enlarged.
Signs and symptoms of ALL, AML:
Rapid onset, bleeding bums/mucosa, epistaxis, infections, pallor, purpura & petechiae, ecchymosis, bone pain / tenderness
ALL Treatment
Chemo / steroids, bone marrow transplant
What is the cell lineage affected in acute myelogenous leukemia?
AML affects myeloid cell progenitors (granulocytes, WBCs, megakaryocytes, RBCs)
What segment of the population usually gets AML?
Adults over 60.
With labs and radiology, how do you differentiate AML from ALL?
ALL patients - enlarged mediastinal mass on x-ray; AML patients will have blast cells that present with AUER RODs (needle-like inclusions in the cytoplasm), cytoplasm w/granules.
What is the most common type of leukemia / who gets it?
Chronic Lymphocytic Leukemia / adults & elderly
What cells are affected in Chronic Lymphocytic Leukemia?
B Cells.
Labs for CLL:
Normal hematocrit and platelet count
Treatment for CLL:
In early stages - observe; in late stages - combination chemotherapy
Who gets Chronic Myeloid Leukemia?
Middle Aged (avg. age of onset is 55)
What occurs in CML?
Over production of myeloid progenitor cells
CML and CLL can both remain ___________________ for years:
asymptomatic and low grade
Etiology of CML:
Philadelphia Chromosome (translocation)
Symptoms of CML:
Night sweats, priapism, fever, blurred vision, splenomegaly, sternal tenderness from bone marrow expansion
Labs in CML:
Left shifted myeloid cells, elevated WBCs
CML can progress to:
AML
Polycythemia Vera is caused by:
Caused by mutation in signaling protein (JAK2)
What occurs in polycythemia vera?
The body overproduces all types of blood cells, particularly RBCs. This leads to thicker blood viscosity.
Symptoms in Polycythemia Vera:
Tinnitus, headache, dizziness, blurred vision, epistaxis, fatigue, pruritis due to increased histamine release, clots, splenomegaly.
EPO level in a patient with polycythemia vera.
Even though all cells are increased, EPO is NOT increased.
Polycythemia Vera labs:
EPO - normal, Increased RBC mass, elevated WBCs and platelets, splenomegaly, elevated hematocrit, high B-12 levels.
Polycythemia Vera Treatment:
Phlebotomy
Essential Thrombocytosis cause:
Unknown
Essential Thrombocytosis labs:
High platelet counts, high megakaryocytes, normal RBC count, most everything else = normal
Complications of Essential Thrombocytosis:
Clotting
Most common symptoms (2) of Essential Thrombocytosis:
Burning of hands, splenomegaly
Myeloproliferative Disorders:
Polycythemia Vera & Essential Thrombocytosis
What disease can follow either of the two Myeloproliferative Diseases?
Myelofibrosis
Myelofibrosis occurs secondary to:
It occurs secondarily to the two myeloproliferative disorders
What causes myelofibrosis?
Increased secretion of platelet derived growth factors and cytokines
Side effects of myelofibrosis:
splenomegaly and hepatomegaly due to extramedullary hematopoiesis in the liver, spleen, and lymph nodes.
Symptoms of Myelofibrosis:
Insidious onset - abdominal fullness, anemia & fatigue, enlarged spleen and liver, fibrosis of bone marrow, thrombocytopenia leads to bleeding, cachectic, bone pain
Lab work of Myelofibrosis always shows:
Anemic Triad
What is the anemic triad?
Tear drop poikilocytosis, leukoerythroblastic blood, giant abnormal platelets
Treatment for Myelofibrosis:
Stem cell transplant (only curative tx), splenectomy (not curative)
Elevated WBCs with the presence of blasts is:
Acute leukemia - medical emergency - call 9-11.
