Hematologic Malignancies Flashcards
Spongy tissue where development of all types of blood cells takes place
bone marrow
Bones that have active marrow in adults
vertebrae, hip, shoulders, ribs, breast and skull
Cancer of white blood cells. characterized by increased and unregulated growth of myeloid cells in the bone marrow.
Chronic myelogenous leukemia (granulocytic)
Clonal bone marrow stem cell disorder
Associated with characteristic chromosomal translocation- Philadelphia (Ph) chromosome
Chronic myelogenous leukemia (granulocytic)
the result of a reciprocal translocation between chromosome 9 and 22
philadelphia chromosome
result of the translocation is the oncogenic BCR-ABL gene fusion
the mutant tyrosine kinase encoded by the BCR-Abl transcript results in a protein that is “always on” (ie cancer)
Therapeutic options for CML that limits growth of tumor cells and decreases risk of blast crisis. Used to induce remission.
Imatinib mesylate (Gleevec)
Only known cure for CML
allogenic stem cell transplantation
Malignant plasma cell tumor
Plasmacytoma
Where are extramedullary plasmacytomas usually found?
upper respiratory trat
Main signs and symptoms of multiple myeloma
bone lesions, bone pain, anemia
What is the cause of renal dysfunction in late stage multiple myeloma?
amyloid, light chain, or Bence Jones protein deposition in the kidneys
Types of multiple myeloma with no anemia, bone lesions, normal calcium and kidney fxns
MGUS and smoldering MM
Type of multiple myeloma with anemia, bone lesions, high calcium, or abnormal kidney fxn
active MM
What causes bone pain associated with multiple myeloma?
distention of the periosteum by rapidly growing lesion
found in serum or urine or both at time of diagnosis in 97% of patients with MM
M protein
Cancers with the highest prevalence of metastatic bone disease
breast and prostate cancers
primary diagnostic test to detect destructive bony lesions in multiple myeloma
skeletal radiography
Clinical features include: fatigue, infection, organomegaly, extranodal infiltrates
CLL
Overall median survival of patients with CLL
10 yrs
Rai staging of CLL
0- lymphocytosis. 1- lymphocytosis w/lymphadenopathy. 2- lymphocytosis w/hepatomegaly or splenomegaly. 3- lymphocytosis w/anemia. 4- lymphocytosis w/thrombocytopenia
What predicts rapid disease progression of early stage disease?
short lymphocyte doubling time
Prognosis based on bone marrow histology patterns
diffuse marrow infiltration-poor. nodular pattern-good
Intravenous infusion of autologous or allogeneic stem cells. Collected from bone marrow, peripheral blood or umbilical cord blood
Hematopoietic stem cell transplantation
Term that describes a graft source from an identical twin
syngeneic
CI to autologous transplant
disease of blood or bone marrow
From parent, child or sibling. Must have many stem cells to overcome risk of graft rejection. Increased risk of GVHD
Haploidentical Donors
Cryopreserved
Small number of stem cells. Higher incidence of engraftment failure. Degree of matching not as stringent
Umbilical cord blood
clonal stem cell disorder characterised by increased red cell production
Polycythaemia vera
Associated with JAK2 protein mutation
Polycythaemia vera
Presentation includes 55-60yrs, vascular complications, hepatosplenomegaly, erythromyalgia
Polycythaemia vera
Term for increase skin temp, burning sensation, and redness
erythromyalgia
Treatment of Polycythaemia vera
venesection/chemo, low dose aspirin, antihistamines
