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Hematology/Oncology > Myeloproliferative Disorders > Flashcards

Myeloproliferative Disorders Flashcards

1
Q

what are myeloproliferative syndromes characterized as

A

too many blood cells

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2
Q

too many red blood cells is known as

A

polycythermia vera

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3
Q

too many platelets

A

essential thrombocytosis

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4
Q

too many neutrophils

A

chronic myelogenous or chronic myeloid leukemia

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5
Q

what is polycythemia vera

A

elevated RBC mass associated with mild increase and platelets

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6
Q

what gene is associated with PCV

A

Janus kinases 2

JAK2V617F

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7
Q

what are the symptoms of PCV

A

fatigue, HA, Puritis, flushing, abdominal discomfort d/t splenomegaly
less common symp: TIA, mesenteric or hepatic thrombosis
Epistaxis, easy brusing, digital ischemia

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8
Q

what are the PCV PE findings

A

ruddy complexion

mild splenomegaly

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9
Q

what is the Dx for PCV

A

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%

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10
Q

what is the Dx of PCV?

A

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

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11
Q

Tx for PCV

A 
lower Hct to avoid symptoms 
phlebotomize the patient <42% Hct
Anagrelide
Ruxolitinib only for JAK 2+
NO IRON
Anticoag if thrombis is documented
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12
Q

Rx for conditions for hyperurecemia

A

allopurinol and colchicine

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13
Q

rx for puritis

A

antihistamines
doxepin
cyproheptadine

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14
Q

essential thrombocytosis has a platelet count of?

A 
600,000 with no other etiologies
Need to exclude 
Iron deficiency
bleeding
co-existing infection or inflammatory phase or other myleoproliferative d/o
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15
Q

what are the signs and symptoms of essential thrombocytosis

A

tend to be more common in females
HA or dizziness
Occasionally easy brusing and GI bleeding
PE: norm

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16
Q

what are the lab findings for essential thrombocytosis

A

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

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17
Q

when to check if suspucious of something other than ET

A

BM biopsy to R/O myelofibrosis

check for CML for philadelphia chromosome or BRC/ABL mutation

18
Q

what is the Tx of ET

A

based on symptoms

19
Q

what is CML

A

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

20
Q

what is diagnostic of CML

A

philadelphia chromosome t(9;22)

BCR-ABL-1

21
Q

what are the signs and symptoms of CML

A

fatigue, SOB, unexplained weight loss or abdominal discomfort d/t splenomeagly
thrombocytosis >10,000,000
wet purpura, GI bleeding

22
Q

what are the lab findings for CML

A

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

23
Q

what is the tx for CML

A

Imatinib

24
Q

what is myelofibrosis with myeloid metaplasia

A

clonal disorder of the bone marrow characterized by increased marrow reticulum, increased marrow fibrosis, extra-medullary hematopoiesis and splenomegaly

25
Q

how does MMM present

A

fatigue, upper abdominal pain and sometime weight loss
thrombocytosis
splenomegaly and extramedually hematopoiesis

26
Q

what are the labs like for MMM

A

peripheral smear is a leuko-eryhtroblastic
Myelophthisis
Immature RBC, and WBC with teardrop shaped RBC
50% will have JAK2 V617F

27
Q

what is the treatment for MMM?

A 
primarily supportive
anemia is tx'ed with transfusion
Splenectomy or Irradiating the spleen
Hydrea or RUxolitinib 
Allogeneic bone marrow transplantation
28
Q

aplastic anemia/marrow failure and myelodysplastic syndromes

A

dz of marrow failure
characterized by cytopenia’s usually more than one cell line
NO splenomegaly or Extramedually hematopoiesis

29
Q

what causes marrow failure syndromes

A 
toxic bone marrow injury d/t drugs or radiation
congenital
viral infections
thymoma 
graf vs host dz
30
Q

pancytopenias are associated with?

A 
cellular marrow secondary to other non marrow etiologies
B12 or folate
ETOH poisoning
Viral infx
bacterial sepsis
31
Q

what is acquired aplastic anemia

A

pancytopenia associated with bone marrow hypoplasia

32
Q

what are causes for aplastic anemia

A 
phentoins, chloramphenicol
viral infx Hep A,B,C EBV and parvo
thymoma
Paroxysmal noturnal hemoglobinuria
GVH
33
Q

what will the exam for AAA look like

A

petechiae, wet purpura, pallor, fever

34
Q

what will the labs for AAA look like

A 
normocytic or macrocytic
granulocytopenia w/o lymphopenia
thrombocytopenia
retic count low or 0
hypocellular bone marrow with too much fat and little dysplasia
35
Q

what is the tx of AAA

A

pt should be treated supportively till definitive dx can be made
Allogeneic bone marrow transplantation is potentially curative

36
Q

what is myelodysplastic syndromes

A

disease characterized by pancytopenias abnormally formed RBCs and sometimes immature WBC in peripheral smear

37
Q

who is affected by myelodysplastic syndromes

A

older adults > 70 y.o

38
Q

what is the history of MDS look like

A

insidious onset of fatigue, SOB and possibly angina

splenomegaly

39
Q

what is the labs look like for Myelodysplastic syndromes

A

macrocytosis and abnormally formed RBC and WBC
Bone marrow in hypercellular with dysplastic changes
increase in blasts is bad

40
Q

what is the tx for MDS

A

supportive
allogenic bone marrow transplant
azacitidine

Hematology/Oncology (7 decks)

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