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PCM Exam 3 > Red Cell Disorders 4 > Flashcards

Red Cell Disorders 4 Flashcards

1
Q

once iron is absorbed in the _______, it can follow two pathways:

A

duodenum

  1. transport to the blood
  2. storage as mucosal iron
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2
Q

hepcidin is synthesized in response to high levels of ______ iron stores and inhibits _______

A

liver iron stores;

iron uptake from duodenal mucosal cells into the bloodstream

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3
Q

normally _____% of the transferrin in saturated with iron

A

33%

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4
Q

patient is easily tired and has a constant desire to eat ice. What is the most likely diagnosis

A

iron deficiency anemia;

ice craving is specific for iron deficiency anemia

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5
Q

Plummer Vinson Sydrome has a triad of: _____, _____, and iron deficiency anemia

A
  • dysphagia

- esophageal webs

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6
Q

low/high RDW is seen with iron deficiency anemia

A

high

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7
Q

low/high reticulocyte count is seen with iron deficiency anemia

A

low

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8
Q

what type of chronic diseases is associated with anemia of chronic disease

A
  • chronic bacterial infections: lung abscesses, osteomyelitis and endocarditis
  • chronic immune disorders: RA
  • malignant tumors: lymphomas, lung or breast cancer
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9
Q

what is the pathogenesis of the anemia of chronic disease?

A
  • impairment of iron utilization and reduction in RBC production:
  • inflation will inc. production of hepcidin and thus causes dec. production of erythropoietin
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10
Q

discuss the levels of ferritin, iron, TIBC and transferrin saturation in anemia of chronic disease

A
  • ferritin: high (functional IDA; due iron not being utilized and decreased RBC production)
  • iron: low (sequestration of iron from the circulation)
  • TIBC (low)
  • transferrin saturation (normal)
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11
Q

transferrin saturation is a ratio of ____ to ______

A

serum iron: TIBC

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12
Q

list the clinical features of aplastic anemia

A
  • symptoms of anemia
  • recurrent infections (do to neutropenia)
  • mucosa hemorrhage or menorrhagia (due to thrombocytopenia)
  • NO ORGANOMEGALY
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13
Q

“dry tap” of bone marrow aspiration is seen in _____

A

aplastic anemia because the bone marrow is filled with fat

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14
Q

what is myelophthisic anemia?

A

bone marrow replacement by abnormal infiltrates that disturb the normal architecture (often with fibrosis)

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15
Q

causes of bone marrow failure:

A
  • aplastic anemia
  • myelophthisic anemia
  • diffuse liver disease
  • chronic renal failure
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16
Q

leucoerythoblastic picture is seen with ________ anemia

A

myelophthisic anemia

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17
Q

how would a patient with polycythemia present?

A
  • hyper-viscosity symptoms: headache, dizziness, visual disturbances
  • pruritus
  • early satiety due to splenomegaly
  • thrombosis/bleeding
18
Q

relative polycythemia is secondary to _____

A

dehydration;

relative polycythemia is due to reduced plasma volume

19
Q

_______ absolute polycythemia is associated with LOW EPO levels and ______

A

primary (aka polycythemia vera); primary absolue polycythemia id due to a chronic myeloproliferative neoplasms
SPLENOMEGALY

20
Q

what are some secondary causes of polycythemia with appropriately high EPO levels

A
  • lung dise ase
  • cyanotic heart disease
  • living at high altitude
21
Q

what tumors are associated with EPO secreting tumors

A
  • renal
  • cerebellar
  • hepatic
22
Q

______ mutation is found in 98% of people with polycythemia vera

A

JAK2 mutation

23
Q

causes of bleeding disorders (3)

A
  1. increased small blood vessel fragility
  2. platelet dysfunction or deficiency
  3. coagulation abnormality
24
Q

thrombin time tests _____

A

fibrinogen

25
Q

what are 4 categories for the causes of thrombocytopenia

A
  • decreased platelet production
  • decreased platelet survival
  • splenic sequestration of platelets
  • dilutional thrombocytopenia
26
Q

immune thrombocytopenic purpura is due to antibodies directed against ________

A

platelet membrane glycoproteins IIb/IIIA or Ib/IX complexes

27
Q

describe the role of the spleen in immune thrombocytopenic purpura

A
  • site of anti platelet antibody production

- site of destruction of IgG coated platelets

28
Q

________ bone marrow is seen in immune thrombocytopenic purpura

A

megakaryocytic hyperplasia due to the excessive peripheral destruction of platelets

29
Q

________ can induce a remission fo chronic ITP

A

splenectomy

30
Q

what would you see on bone marrow biopsy of someone with immune thrombocytopenic purpura?

A
  • inc. megakaryocytes
31
Q

describe the mechanism of thrombocytopenia due to non immune thrombocytopenia: TTP/HUS

A
  • there is widespread formation of platelet rich thrombi in small blood vessels → platelet consumption → thrombocytopenia
32
Q

give examples fo hereditary clotting factors

A
  • Factor VIII
  • Von Willebrand Factor
  • Factor IX
33
Q

what are some acquired clotting disorders

A
  • vitamin K deficiency
  • severe liver disease
  • DIC
34
Q

hemophilia A is due an abnormality in )____

A

factor VIII

Factor VIII is a cofactor for Factor IX to activate factor X in the intrinsic pathway
8+9 = 10

35
Q

what are the functions of VWF?

A
  • promotes adhesion of platelets to the subendothelial layer after damage
  • prolongs the half life of Factor VIII
36
Q

type _____ of von willebrand disease is associated with reduced quantity of VWF

A

types 1 and 3

37
Q

Type _____ of von willebrand disease is associated with qualitative/ functional abnormality of VWF

A

type 2

38
Q

VWF prolongs the half life of _____

A

Factor VIII

39
Q

the most severe type of VWD (von willebrand disease) is type _____

A

type 3 because it has a severe deficiency or lack of VWF → presents very similar to Hemophilia A

40
Q

what the lab findings for someone with VWD:
bleeding time
PT:
PTT

A
  • bleeding time: normal
  • PT: normal
  • PTT: normal or ↑
41
Q

describe the clinical presentation of someone with hemophilia A

A
  • massive bleeding after trauma or surgery
  • “spontaneous” hemorrhages usually in joints or muscles
  • NO PETECHIAL HEMORRHAGES because no problem with platelets

PCM Exam 3 (32 decks)

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