100% CORRECT
chronic myeloid leukemia (CML) chromosomal abnormality Answer -
Philadelphia chromosome (~95% of cases)
polycythemia vera (PV) chromosomal abnormality Answer - JAK2V617F
mutation (most cases)
chronic myeloproliferative disorders Answer - -chronic myeloid leukemia (CML)
-polycythemia vera (PV)
-essential thrombocythemia (ET)
-primary myelofibrosis (PMF)
-chronic neutrophilic leukemia (CNL)
-chronic myelomonocytic leukemia (CMML)
-juvenile myelomonocytic leukemia (JMML)
polycythemia vera major criteria (WHO) Answer - -hemoglobin >16.5 g/dL
(men), >16 g/dL (women)
-Hct >49% (men), >48% (women)
-increased RCM >35 mL/kg (men), >31 mL/kg (women)
-BM biopsy with hypercellularity and panmyelosis
-presence of JAK2V617F or JAK2 Exon 12 mutations
, polycythemia vera minor criteria (WHO) Answer - subnormal serum EPO levels
chronic myeloid leukemia (CML) Answer - -etiology: a single genetic
translocation in a pluripotent hematopoietic stem cell causes clonal
overproduction of immature neutrophils, usually diagnosed between age 45-55
-chromosomal abnormality: Philadelphia chromosome
-morphology: myeloblasts, promyelovytes, bands, segmented neutrophils (all
stages of neutrophil development) seen in the peripheral blood, nRBCs
present, *gaucher-like macrophages present*, very small megakaryocytes
present
-symptoms: infections with Strep pneumoniae, anemia, bleeding,
splenomegaly
-PB: RBCs normal to inc, retics normal, total WBCs increased, granulocytes
increased (neutrophils, basophils, eosinophils), PLTs normal to inc, *LAP stain
decreased*
-BM: hypercellular, increased granulopoiesis, decreased erythropoiesis,
megakaryopoiesis increased, reticulin fibers increased
chronic myeloid leukemia (CML) stages Answer - chronic phase (3-4 years) →
accelerated phase → blastic phase → possible transition to acute leukemia
(poor prognosis)
chronic myeloid leukemia (CML) treatments Answer - -BM transplant or SCT=
CURE
-*gleevec* (imatnib mesylate)
-sprycel, dasigna, bosulib, iclusig