Myeloproliferative Disorders Flashcards
<strong>Predominant cell type in peripheral blood and bone marrow?</strong>
-– Extreme elevation of blasts and promyelocytes in peripheral blood. In bone marrow there is 90-100% celularity with M:E=10:1 or 50:1, <20% blast (except in blast crisis), normoblasts, platelets(sometimes abnormal fxn.), and megakaryoblasts increased
<strong>Symtoms? </strong>fever, weakness, bleeding, pallor, tender over lower sternum, splenomegaly, petechiae and ecchymoses
<strong>Therapy?</strong> Gleevec: the only drug designed based on the understanding of the molecular basis of disease.
<strong>Onset? </strong>Middle age men and women
Prognosis? A 2011 followup of 832 patients using imatinib who achieved a stable cytogenetic response found an overall survival rate of 95.2% after 8 years, which is similar to the rate in the general population. Less than 1% of patients died because of leukemia progression.
<strong>Exta info?</strong>
•Philadelphia chromosome is present
–T(9:22)
–“bcr-abl”
–Tyrosine kinase with autophosphorylation: Growth factor receptor, always “on”.
–Additional mutations cause progression to accelerated or blast crisis phase (three stages), and can become AML or ALL
Chronic Myeloid Leukemia
Predominant cell type in peripheral blood and bone marrow?
Symtoms?
Onset?
Therapy?
Prognosis?
<strong>Predominant cell type in peripheral blood and bone marrow?</strong> in bone marrow: fibroblast proliferation which messes up normal structure and replaces normal hematopoetic tissue (fibrosis). In peripheral blood: Aniso / poikilo, Basophilic stippling, Norm / norm anemia, Dacrocytes (as spleen enlarges), Reticulocytosis, WBCs high / low / normal, Blasts <5%, LAP is elevated, Plts high / low / normal, and Med leukoerythroblastic anemia.
<strong>Symtoms?</strong> Weakness and loss of appetite. Extramedulary hematopoesis may lead to hepatosplenomegaly and hematopoetic infiltrates in kidney, adrenal glands, peritoneal surfaces, skin, lymph nodes and spinal cord.
<strong>Age/Onset? </strong>Insidous. Men and women over 50.
<strong>Therapy? </strong>Splenectomy can be palliative
<strong>Prognosis?</strong> 5 year survival from diagnosis.
<strong>Extra info?</strong> Can terminate as ALL or AML
Chronic Idiopathic Myelofibrosis
Predominant cell type in peripheral blood and bone marrow?
Symtoms?
Age/Onset?
Therapy?
Prognosis?
Diagnosis of PV consists of :
–RBC mass of: 36 ml/kg in men, 32 ml/kg in women.
–Normal O2 sat
–Splenomegaly
If one of the three above are missing any two of what three conditions will substitute?
–Thrombocytosis (>400)
–Leukocytosis (>12) w/o fever or infection
–Increased LAP, B12 and unbound B12 binding capacity
Predominant cell type in peripheral blood and bone marrow? Bone marrow makes too many red blood cells/may also result in the overproduction of white blood cells and platelets (seen in peripheral blood). BM up to 100% cellularity.
Sypmtoms? Splenomegaly common, itching, possible hypersplenism and possible pancytopenia. Can see trio of: BM fibrosis, splenomegaly, and anemia w/ teardrop poikilocytes.
Therapy? Phlebotomy
Prognosis? Stable phase lasts a long time. Spent phase or progression to acute leukemia can occur.
Extra Info? Stem cells are >> sensitive to erythropoetin
Polycythemia Vera
Predominant cell type in peripheral blood and bone marrow?
Symtoms?
Age/Onset?
Therapy?
Prognosis?
Extra Info?
<strong>Predominant cell type in peripheral blood and bone marrow?</strong> In peripheral blood: >>PLTs, >>change in plt (size, grannularity, shape), WBCs normal, and RBCs norm / norm unless there has been bleeding. In bone marrow lots of megakeryoctes (hypercellular), increase in ALL precursors, and reticulin fibers may increase.
<strong>Symtoms? </strong>bleeding, blood clots, headache, nausea, vomiting, abdominal pain, visual disturbances, dizziness, fainting, splenomegaly, and numbness in the extremities,
<strong>Age/Onset? </strong>Men and women >60 yrs old (mostly women)
<strong>Therapy? </strong>Suppression of plt production through radiation or drugs is palliative but brings increased risk of leukemic transformation
<strong>Prognosis? </strong>Survival can be long after diagnosis.
Plts can occlude small vessels causing
Erythromalalgia
Transient ischemic attacks
Seizures
Cerebral or Myocardial Infarct
<strong>Extra Info? </strong>
Plts can occlude small vessels causing: Erythromalalgia, Transient ischemic attacks, Seizures, and Cerebral or Myocardial Infarct
Essential Thrombocythemia
Predominant cell type in peripheral blood and bone marrow?
Symtoms?
Age/Onset?
Therapy?
Prognosis?
Extra Info?
What six factors are are considered for ET diagnosis?
Diagnosis
–Thrombocytosis >600
–Hgb < 13 (normal RBC mass)
–Sufficient iron stores
–Ph negative
–BM is not fibrotic
–No reason to suspect reactive thrombocytosis
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Compare and contrast the three stages of CML (Chronic Myelogenous Leukemia).
Chronic phase: Approximately 85% of patients with CML are in the chronic phase at the time of diagnosis. Patients are usually asymptomatic or have only mild symptoms of fatigue, left side pain, joint and/or hip pain, or abdominal fullness. The duration of chronic phase is variable and depends on how early the disease was diagnosed as well as the therapies used. In the absence of treatment, the disease progresses to an accelerated phase.
Accelerated Phase: Increased splenomegaly, Rising leukocytosis and Prominent basophilia. 10–19% myeloblasts in the blood or bone marrow, >20% basophils in the blood or bone marrow, Platelet count <100,000, unrelated to therapy, and Platelet count >1,000,000, unresponsive to therapy
Blast Crisis: >20% blasts in BM or PB, and Not called AML because of preexisting CML.
Compare and contrast proliferative disorders with acute leukemias.
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