PMF eventually progresses to the fibrotic stage, in which the bone marrow has ____, the peripheral blood has _____, and other organs enlarge because of ____.
reticulin fibrosis; leukoerythroblastosis; extramedullary hematopoiesis
PV should always be considered in a pt with a thrombosis of the ____, ____, or _____ vein.
mesenteric, portal, splenic
What are the s/s of PV?
thrombosis of the mesenteric, portal, or splenic vein; headaches; dizziness; visual probs; paresthesias; plethora; itching; splenomegaly; hepatomegaly
Before diagnosing MDS, you should rule out non-neoplastic causes of 2a myelodysplasia: ____, ______, _____, and _____.
certain drugs (chemo); B12, folic acid, and other deficiencies; viral infection; heavy metal (arsenic) and other toxin exposures
What is PV?
an MPN characterized by increased RBC mass (erythrocytosis) accompanied by increased neutrophils and platelets (trilineage hyperplasia)
What are ring sideroblasts?
RBCs that have iron accumulated in the mitochondria that encircle the nucleus- stains blue
What does dysgranulopoiesis look like?
nuclear hypolobation; neutrophils with bilobed nuclei called pseudo-Pelger-Huet cells; hypogranularity
What are the 2 types of high grade MDS?
1. refractory anemia with excess blasts-1 (RAEB-1) 2. refractory anemia with excess blasts-2 (RAEB-2)
What is the initial phase of CML, and what are its characteristics?
the chronic phase; normal blasts, increased neutrophils, basophils, and (usu) platelets
What is PMF?
proliferation of the granulocytic and megakaryocytic lineages with eventual progression to myelofibrosis
MPNs are usually associated with abnormalities of genes (translocations, point mutations) that encode _____.
cytoplasmic or receptor protein tyrosine kinase (PTKs)
Untreated MPNs lead to _____, _____, and _____.
1. AML or ALL 2. myelodysplasia with ineffective hematopoiesis (MDS) 3. marrow fibrosis and failure
What is common to all MPNs?
increase in 1+ cell types; hypercellular marrow; spleno- or hepatomegaly; insidious onset usually detected by CBC
What is the prognosis for refractory anemia with excess blasts-1 (RAEB-1)? Median survival? Transformation?
poor; median survival 16 mos; 25% of patients transform to AML
Dysplastic changes in at least ____ % of cells in one or more lineages is diagnostic for MDS.
What is the bone marrow like in ET?
atypical megakaryocytes are even larger and more bizarre than PMF
____ mutations can be found in 50% of pts with ET.
What is the incidence rate of MPNs?
What is seen on the PV bone marrow biopsy?
trilineage hyperplasia; clusters of large, bizarre megakaryocytes
What constitutes the BCR-ABL1 gene fusion?
translocation (9;22)(q34;q11.2) on the derivative chromosome 22 (Philadelphia chromosome)
What is the most serious complication of PV?
venous or arterial thrombi
What does dysmegakaryopoiesis look like?
megakaryocytes have hypolobated or non-lobated nuclei; hyperchromatic nuclei; small size
How are PV and PMF different?
no erythrocytosis in PMF
Where does extramedullary hematopoiesis occur?
spleen, liver, lymph nodes
____ eventually progresses to the fibrotic stage, in which the bone marrow has reticulin fibrosis, the peripheral blood has leukoerythroblastosis, and other organs enlarge because of extramedullary hematopoiesis.
Who gets 1a/idiopathic MDS, and what is the incidence rate?
people over 50; avg age at diagnosis is 70; 3-5/100,000
In the prefibrotic stage of PMF, the marrow is _____, showing a granulocytic and megakaryocytic proliferation.
What is the 2nd phase of CML? What are its characteristics?
the blast phase; essentially acute leukemia (>20% blasts in the blood)
Who gets 2a/t-MDS?
persons exposed to alkylating agents or IR 2-8 years later
Which cells make up the blast phase in CML?
myeloblasts (70%) and lymphoblasts (30%)