Myeloproliferative Disorders Flashcards
what are myeloproliferative syndromes characterized as
too many blood cells
too many red blood cells is known as
polycythermia vera
too many platelets
essential thrombocytosis
too many neutrophils
chronic myelogenous or chronic myeloid leukemia
what is polycythemia vera
elevated RBC mass associated with mild increase and platelets
what gene is associated with PCV
Janus kinases 2
JAK2V617F
what are the symptoms of PCV
fatigue, HA, Puritis, flushing, abdominal discomfort d/t splenomegaly
less common symp: TIA, mesenteric or hepatic thrombosis
Epistaxis, easy brusing, digital ischemia
what are the PCV PE findings
ruddy complexion
mild splenomegaly
what is the Dx for PCV
when there is significant mature erythrocytosis (HcT >60%) with some modest increase in mature platelets and neutrophils
WBC and platelets will be normal HcT > 52% males & >48% females but <60%
what is the Dx of PCV?
ABG including carboxy Hgb should be normal
Serum Erythropoietin (low)
splenic US: may show splenomegaly
Fe/TIBC(normal), B12 norm to elevated, folate norm
test peripheral blood for JAK2 mutation
Tx for PCV
lower Hct to avoid symptoms phlebotomize the patient <42% Hct Anagrelide Ruxolitinib only for JAK 2+ NO IRON Anticoag if thrombis is documented
Rx for conditions for hyperurecemia
allopurinol and colchicine
rx for puritis
antihistamines
doxepin
cyproheptadine
essential thrombocytosis has a platelet count of?
600,000 with no other etiologies Need to exclude Iron deficiency bleeding co-existing infection or inflammatory phase or other myleoproliferative d/o
what are the signs and symptoms of essential thrombocytosis
tend to be more common in females
HA or dizziness
Occasionally easy brusing and GI bleeding
PE: norm
what are the lab findings for essential thrombocytosis
Hgb and Hct normal
WBC slightly elevated with normal differential
Platelets must be increased and some giatn platelets are expected
JAK2 V617F
R/O CML
Norm Fe/TIBC, normal ferritin, norm sed rate
when to check if suspucious of something other than ET
BM biopsy to R/O myelofibrosis
check for CML for philadelphia chromosome or BRC/ABL mutation
what is the Tx of ET
based on symptoms
what is CML
characterized by elevated white blood count with spectrum on Immature blood cells normally seen in the bone marrow now present in peripheral blood
Thrombocytosis and anemia are fairly common
Basophilia is almost diagnostic
what is diagnostic of CML
philadelphia chromosome t(9;22)
BCR-ABL-1
what are the signs and symptoms of CML
fatigue, SOB, unexplained weight loss or abdominal discomfort d/t splenomeagly
thrombocytosis >10,000,000
wet purpura, GI bleeding
what are the lab findings for CML
peripheral blood is sent for a leukocyte alkaline
phosphatase which is paradoxically low in CML
sed rate normal
peripheral blood smear should have predominance of bands, metamyeloctyes and myelocyted with <5% blasts
BM hypercellular
what is the tx for CML
Imatinib
what is myelofibrosis with myeloid metaplasia
clonal disorder of the bone marrow characterized by increased marrow reticulum, increased marrow fibrosis, extra-medullary hematopoiesis and splenomegaly
how does MMM present
fatigue, upper abdominal pain and sometime weight loss
thrombocytosis
splenomegaly and extramedually hematopoiesis
what are the labs like for MMM
peripheral smear is a leuko-eryhtroblastic
Myelophthisis
Immature RBC, and WBC with teardrop shaped RBC
50% will have JAK2 V617F
what is the treatment for MMM?
primarily supportive anemia is tx'ed with transfusion Splenectomy or Irradiating the spleen Hydrea or RUxolitinib Allogeneic bone marrow transplantation
aplastic anemia/marrow failure and myelodysplastic syndromes
dz of marrow failure
characterized by cytopenia’s usually more than one cell line
NO splenomegaly or Extramedually hematopoiesis
what causes marrow failure syndromes
toxic bone marrow injury d/t drugs or radiation congenital viral infections thymoma graf vs host dz
pancytopenias are associated with?
cellular marrow secondary to other non marrow etiologies B12 or folate ETOH poisoning Viral infx bacterial sepsis
what is acquired aplastic anemia
pancytopenia associated with bone marrow hypoplasia
what are causes for aplastic anemia
phentoins, chloramphenicol viral infx Hep A,B,C EBV and parvo thymoma Paroxysmal noturnal hemoglobinuria GVH
what will the exam for AAA look like
petechiae, wet purpura, pallor, fever
what will the labs for AAA look like
normocytic or macrocytic granulocytopenia w/o lymphopenia thrombocytopenia retic count low or 0 hypocellular bone marrow with too much fat and little dysplasia
what is the tx of AAA
pt should be treated supportively till definitive dx can be made
Allogeneic bone marrow transplantation is potentially curative
what is myelodysplastic syndromes
disease characterized by pancytopenias abnormally formed RBCs and sometimes immature WBC in peripheral smear
who is affected by myelodysplastic syndromes
older adults > 70 y.o
what is the history of MDS look like
insidious onset of fatigue, SOB and possibly angina
splenomegaly
what is the labs look like for Myelodysplastic syndromes
macrocytosis and abnormally formed RBC and WBC
Bone marrow in hypercellular with dysplastic changes
increase in blasts is bad
what is the tx for MDS
supportive
allogenic bone marrow transplant
azacitidine
