99 PV Flashcards
What are the 8 WHO classification of chronic myeloproliferative neoplasm
- CML, bcr-abl positive
- Chronic neutrophilic leukemia
- Chronic eosininophilic leukemia, not otherwise specified
- Polycythemia vera
- Primary myelofibrosis
- Essential thrombocytosis
- Mastocytosis
- Myeloproliferative neoplasms, unclassified
associated with balanced translocation between chromosomes 9 and 22`
CML
associated with a t(15:19)
Chronic neutrophilic leukemia
tyrosine kinase essential for the function of erythropoeitin and thrombopoietin
JAK2
The mutation seen in PV, PMF, and ET
mutation in V617F
True or false. In CML, CNL, there is a high rate of leukemic transformation due to its mutation effects on the granulocyte colony stimulating factor receptor
True.
True or false. In PV, ET, PMF there is rare transformation to leukemia unless there is exposure to mutagenic drugs.
True.
Most common of the chronic myeloproliferative neoplasm
Polycythemia vera
Clonal disorder involving multipotent hematopoetic progenitor cell in which phenotypically normal red cells, granulocytes, and platelets accumulate in the absence of a recognizable physiologic stimulus
Polycythemia vera
True or false. No cosistent cytogenic abnormality is associated with PV unlike CML
True.
central role in the pathogenesis of PV
mutation in the autoinhibitory pseudokinase domain of tyrosine kinase JAK2 that replaces valine with phenylalanine (V617F)
member of an evolutionary well conserved non receptor tyrosine kinase family and serves as cognate tyrosine kinase for the erythropoetin or thrombopoeitin receptors
JAK2
True or false. JAK2V617F is the basis for many of the phenotypic and biochemical characteristics of PV such as elevation of leukocyte alkaline phosphatase (LAP) score
True.
How is PV usually first recognized
incidental discovery of high hemoglobin or hematocrit
With the except of this manifestation, no symptoms distinguish PV from other causes of erythrocytosis
aquagenic pruritus
initial presenting manifestations of PV
isolated thrombocytosis, leukocytosis or splenomegaly
True or false. Systolic hypertension is also a feature of red cell mass elevation
True.
True or false. Uncontrolled erythrocytosis causes hyperviscosity leading to neurologic symptoms such as vertigo, tinnitus, headache, visual disturbances and TIAs.
True.
most commonly affected vessels in PV
cerebral, cardiac, mesenteric vessels
True or false. Venous or arterial thrombosis may be the presenting manifestations of PV
True.
complications of PV manifested as erythema, burning, and pain in the extremities
erythromelalgia
what is mechanism of erythromelalgia
due to increased platelet stickiness
What is unique in PV in contrast to other causes of erythrocytosis
expansion of plasma volume
What is the hemoglobin or hematocrit level to say it is undeniably PV?
hgb more than 20 g/dl or hematocrit more than 60%
3 situations where there is microscopic erythrocytosis
beta thalassemia trait, hypoxic erythrocytosis, and polycythemia vera
other names of stress erythrocytosis
spurious erythrocytosis or Gaisbocks syndrome
how to differential PV from beta thalassemia trait
in beta thalassemia, the RDW is normal while it is elevated in PV
True or false. JAK2 assay has superseded other tests for establishing the diagnosis of PV
True.
True or false. Bone marrow aspirate and biopsy provide no specific diagnostic information because these may be normal or indistinguishable from ET or PMF
True.
True or false. The incidence of H Pylori infection in PV is increased
True.
What is the pathophysiology of pruritus in PV
consequence of mast cell activation by JAK2V617F
True or false. normal hematocrit or hemoglobin with massive splenomegaly should be considered indicative of elevated red cell mass until proven otherwise
True.
True or false. In patients with splenomegaly, true extent of red cell mass elevation is masked
True.
most significant complication of PV
thrombosis
what is the target Hgb and Hct levels in PV to avoid thrombotic complications
Men: Hgb less than 14 g/dL and Hct less than 45%
Women: Hgb less han 12 g/dl and Hct less than 42%
How many phlebotomy or when is it required
Once patient is in iron deficient state, phlebotomy may be done at 3 month interval
serum uric acid value when allopurinol is given
serum uric acid of 10 mg/dL and above
True or false. Due to an elevated platelet count, PV may manifest with acquired vWF deficiency
True.
phosphodiesterase inhibitor that can be given to reduce platelet count with lack of marrow toxicity and protective against venous thrombosis
anagrelide
True or false. Allogeneic bone marrow transplant is promising in PV
False. No role
tyrosine kinase inhibitor for PV patients unresponsive to cytotoxic agent
ruxolitinib
Least common MPN
primary myelofibrosis
clonal disorder characterized by multipotent hematopoietic progenitor cell of unknown etiology characterized by marrow fibrosis, extramedullary hematopoeisis and splenomegaly
Primary myelofibrosis
