Hemonc Test I Review

Front 1

what is the hallmark of polycythemia vera?

Back 1

a Hct above normal, at times greater than 60%

Front 2

what is the most common complication of polycythemia ver and the major cause of morbidity and death?

Back 2

thrombosis

Front 3

how do most patients present (initially) with PV?

Back 3

expanded blood volume and increased blood viscosity

Front 4

what are some of the signs/symptoms of p with polycythemia vera?

Back 4

HA, dizziness, tinnitis, blurred vision, pruritis (after hot bath due to histamines released by baso's) plethora, splenomegaly

Front 5

what would be a textbook CBC for a p with PV?

Back 5

microcitic MCV, Increased HcT, Increased WBC, PLT, presence of Eosinophils, and basophils

Front 6

an increase in the number of abnormally shaped RBC. Pearshape/tear drop morphology?

Back 6

poikilocytosis

Front 7

what are the two main causes of iron deficiency in PV?

Back 7

1.iron stores are usually absent from the hypo-cellular bone marrow
2. chronic GI bleed= blood loss

Front 8

what is the bcr/abl fusion gene also known as?

Back 8

the philadelphia chromosome

Front 9

what is the hallmark of essential thrombocytosis?

Back 9

an elevated platelet count, may be over 2,000,000/mcL

Front 10

an uncommon myeloproliferative disorder of unknown cause in chich marked proliferation of the megakaryocytes in the bone marrow leads to elevation of the PLT count?

Back 10

essential thrombocytosis

Front 11

what is the median age presentation for ET?

Back 11

50-60 y/o
MC in women

Front 12

what is the most common clinical problem of thrombocytosis?

Back 12

thrombosis

Front 13

what would be the typical lab findings of ET?

Back 13

elevated PLT, WBC (mildly), normal HCT, macrocytic Rbc. prolonged bleeding time

Front 14

what are some examples of other inflammatory disorders that cause elevated PLT's?

Back 14

RA, UC

Front 15

what distinguishes ET from PV?

Back 15

the lack of elevated HCT and RBC mass

Front 16

what distinguishes ET from myelofibrosis?

Back 16

RBC mass, absence of nucleated RBC's, lack of giant degranulated plt's

Front 17

this disorder is characterized by fibrosis of the bone marrow, splenomegaly, leukoerythroblastic peripheral bllod w/ teardrop poikilocytosis?

Back 17

myelofibrosis

Front 18

what is believed to be the cause of fibrosis in myelofibrosis?

Back 18

in response to increased secretion of platelet derived growth factor (PDGF)

Front 19

what are some common presentations in myelofibrosis?

Back 19

Fatigue: due to anemia
Abdominal fullness: due to splenomegaly
Hepatomegaly in >50%

Front 20

dramatic peripheral blood smear: significant poikilocytosis + numerous teardrop RBC, Nucleated RBC, giant PLT is indicitive of?

Back 20

myelofibrosis

Front 21

the triad of teardrop poikilocytosis, leukoerythroblastic blood and giant abnormal PLT's is highly suggestive of ?

Back 21

myelofibrosis

Front 22

the presence of erythroblasts and myelocytes (which are precursors of mature cells) in the blood. Often indicates marrow infiltration eg by secondary cancer, or fibrosis

Back 22

leukoerythroblastic blood

Front 23

a vegan is most likely to have deficiencies in what dietary vitamin?

Back 23

B12. since it is present in all foods of animal origin.

Front 24

the most common cause of Vitamin B12 deficiency is associated with which hereditary disease?

Back 24

pernicious anemia

Front 25

what is the hallmark of symptomatic vitamin B12 deficiency?

Back 25

megaloblastic anemia

Front 26

a physical exam that reveals a pale and mildly icteric skin tone, mild paresthesias, difficulty with balance, decreased vibration and position sense might be strongly indicitive of which disorder?

Back 26

megaloblastic state in vitamin B12 deficiency

Front 27

red blood cells are of unequal size.

Back 27

anisocytosis

Front 28

a medical investigation used in patients with vitamin B12 deficiency. The purpose of the test is to determine if the patient has pernicious anemia

Back 28

the schilling test

Front 29

a type of autoimmune anemia. Antibodies are directed against intrinsic factor or parietal cells which produce intrinsic factor. Intrinsic factor is required for vitamin B12 absorption, so impaired absorption of vitamin B12 can result. An anemia is a deficiency of the blood cells, but in addition to blood cells, many other cells in the body need vitamin B12, including nerve cells.

Back 29

pernicious anemia

Front 30

what are two common causes of megaloblastic anemia/

Back 30

1. vitamin B12 deficiency
2. folic acid deficiency

Front 31

microcytosis (out of proportion to the degree of anemia) + FHx of microcytic anemia, microcytes, acanthocytes and target cells on RBC morphology are all essential to which type of anemia?

Back 31

the thalassemias

Front 32

p presents with low reticulocyte count, and microcytic anemia. what is a possible list of differentials?

Back 32

iron deficiency anemia, Thallasemia minor, sideroblastic anemia, lead poisoning

Front 33

P presents with low reticulocyte count + signs of a macrocytic anemia. what diff dx do you consider?

Back 33

megaloblastic anemias, folate deficiency, Vit B12 def,

Front 34

what is the leading cause of anemia world wide?

Back 34

iron deficiency

Front 35

what are the two types of macrocytic anemias?

Back 35

1.megaloblastic: arises froma failure of DNA synthesis
2. Non-megaloblastic: reflect membrane abnormalities resulting from abnormalities in cholesterol metab. (advanced liver dz, hypothyroid)

Front 36

The common feature of this anemia is a failure to completely form heme - whose biosynthesis takes place partly in the mitochondrion. This leads to deposits of iron in the mitochondria that form a ring around the nucleus of the developling red blood cell.

Back 36

sideroblastic anemia