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36 Cards in this Set

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  • Back
what is the hallmark of polycythemia vera?
a Hct above normal, at times greater than 60%
what is the most common complication of polycythemia ver and the major cause of morbidity and death?
how do most patients present (initially) with PV?
expanded blood volume and increased blood viscosity
what are some of the signs/symptoms of p with polycythemia vera?
HA, dizziness, tinnitis, blurred vision, pruritis (after hot bath due to histamines released by baso's) plethora, splenomegaly
what would be a textbook CBC for a p with PV?
microcitic MCV, Increased HcT, Increased WBC, PLT, presence of Eosinophils, and basophils
an increase in the number of abnormally shaped RBC. Pearshape/tear drop morphology?
what are the two main causes of iron deficiency in PV?
1.iron stores are usually absent from the hypo-cellular bone marrow
2. chronic GI bleed= blood loss
what is the bcr/abl fusion gene also known as?
the philadelphia chromosome
what is the hallmark of essential thrombocytosis?
an elevated platelet count, may be over 2,000,000/mcL
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?
essential thrombocytosis
what is the median age presentation for ET?
50-60 y/o
MC in women
what is the most common clinical problem of thrombocytosis?
what would be the typical lab findings of ET?
elevated PLT, WBC (mildly), normal HCT, macrocytic Rbc. prolonged bleeding time
what are some examples of other inflammatory disorders that cause elevated PLT's?
what distinguishes ET from PV?
the lack of elevated HCT and RBC mass
what distinguishes ET from myelofibrosis?
RBC mass, absence of nucleated RBC's, lack of giant degranulated plt's
this disorder is characterized by fibrosis of the bone marrow, splenomegaly, leukoerythroblastic peripheral bllod w/ teardrop poikilocytosis?
what is believed to be the cause of fibrosis in myelofibrosis?
in response to increased secretion of platelet derived growth factor (PDGF)
what are some common presentations in myelofibrosis?
Fatigue: due to anemia
Abdominal fullness: due to splenomegaly
Hepatomegaly in >50%
dramatic peripheral blood smear: significant poikilocytosis + numerous teardrop RBC, Nucleated RBC, giant PLT is indicitive of?
the triad of teardrop poikilocytosis, leukoerythroblastic blood and giant abnormal PLT's is highly suggestive of ?
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
leukoerythroblastic blood
a vegan is most likely to have deficiencies in what dietary vitamin?
B12. since it is present in all foods of animal origin.
the most common cause of Vitamin B12 deficiency is associated with which hereditary disease?
pernicious anemia
what is the hallmark of symptomatic vitamin B12 deficiency?
megaloblastic anemia
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?
megaloblastic state in vitamin B12 deficiency
red blood cells are of unequal size.
a medical investigation used in patients with vitamin B12 deficiency. The purpose of the test is to determine if the patient has pernicious anemia
the schilling test
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.
pernicious anemia
what are two common causes of megaloblastic anemia/
1. vitamin B12 deficiency
2. folic acid deficiency
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?
the thalassemias
p presents with low reticulocyte count, and microcytic anemia. what is a possible list of differentials?
iron deficiency anemia, Thallasemia minor, sideroblastic anemia, lead poisoning
P presents with low reticulocyte count + signs of a macrocytic anemia. what diff dx do you consider?
megaloblastic anemias, folate deficiency, Vit B12 def,
what is the leading cause of anemia world wide?
iron deficiency
what are the two types of macrocytic anemias?
1.megaloblastic: arises froma failure of DNA synthesis
2. Non-megaloblastic: reflect membrane abnormalities resulting from abnormalities in cholesterol metab. (advanced liver dz, hypothyroid)
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.
sideroblastic anemia