Myeloproliferative Disorders Flashcards
Myeloproliferative disorders (MPDs)
includes leukemias & disorders
MPDs are not clearly malignant but are characterized by neoplastic proliferation of hematopoietic precursors
affects the myeloid line
acquired, chronic disorders that may transform into acute leukemias
CML Variants - Juvenile CML
rare
10-14 yrs old
similar to adult CML
CML Variants - Chronic Neutrophilic leukemia
sustained increase in neutrophils VERY rare median age - 67 yrs most patients are asymptomatic Ph chromosome NEGATIVE!!!
CML Variants - Chronic Neutrophilic Leukemia lab findings
neutrophilia >80%
increased LAP
poor prognosis
CML Variants - Chronic Eosinophilic leukemia (CEL)
Ph chromosome negative
middle aged men
release of eosinophilic granules cause damage to digits & organs
30-70% eos
CML Variants - Chronic basophilic leukemia
rarest variant of CML
40-80% basophils
Polycythemia Vera (PV) General Characteristics
AKA polycythemia rubra vera, primary polycythemia
unregulated proliferation of the erythroid elements in the BM
increase RBCs in PB
Epidemiology of PV
relatively rare
20-80 yrs
frequently in men than women
more common in whites from jewish descent
Eitology of PV
acquired mutation
EPO hypersensitivity
Symptoms of PV
related to the thickened blood
red coloration, especially of the face
vascular occlusion or thrombosis (clots)
myocardial infarction - cardiovascular diseases
Lab findings of PV
erythrocytosis elevated hgb elevated hct no feathered edge thrombocytosis - giant platelets LAP increased
Treatment of PV
no cure
therapeutic phlebotomy
myelosuppressive drugs
Complications of PV
may lead to AML
cardiovascular disease
Prognosis of PV
median survival w/ treatment: 11-15 yrs
w/o treatment - 18 months
major cause of death is strokes / heart attack
Secondary Polycythemia
increased EPO due to tissue hypoxia or due to renal tumors
Relative Polycythemia
mild polycythemia due to dehydration resulting in hemoconcentration
will have normal WBC & PLT counts
Essential Thrombocythemia (ET)
large # of abnormal platelets that cause clotting & bleeding / hemorrhaging.
Hallmark of ET
thrombocytosis greater than 600 x 10 ^9
all other cells in the blood are normal
Eitology of ET
acquired mutation
cause unknown
Epidemiology of ET
rare disorder
older age group (65)
affects males & females equally
Symptoms of ET
often asymptomatic
thrombosis
hemorrhage from nose, gums, GI tract
Lab findings of ET in PB
thrombocytosis > 600
megakaryocytes
giant, bizarre platelets
Lab findings of ET in BM
hypercellular
large increase in abnormal megakaryocytes (increased size, clustering, increased lobules)
treatment of ET
anticoagulants & drugs to inhibit platelet function
Prognosis of ET
10+ years
common cause of death are clots & bleeding
Chronic Idiopathic Myelofibrosis (CIMF)
AKA agnogenic myeloid metaplasia (AMM), myelofibrosis w/ myeloid metaplasia (MMM), and primary fibrosis (WHO 2008)
scarring of the bone marrow
CIMF pathophysiology
scarring of bone marrow causing extramedullary hematopoiesis
Fibroblasts are collagen producing cells that eventually disrupt normal architecture of the marrow & replaces hematopoietic tissue
Hallmark of CIMF
bone marrow is replaced by collagen - DRY TAP aspirate
Epidemiology of CIMF
uncommon disease
very chronic - gradual onset
older patients > 50 yrs
Symptoms of CIMF
may be asymptomatic
pain in extremities
splenomegaly & hepatomegaly
Lab findings of CIMF
normo, normo anemia striking aniso & poik TEAR DROP CELLS basophilic stippling left shift expected platelet count is variable!!!
lab findings in BM of CIMF
DRY TAP- no aspirate
hypercellular
presence of fibrosis
