postmidterm 2 Flashcards

1
Q

what are reticulocytes?

A

early RBCs that still have nucleus

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2
Q

PT (prothrombin time) / INR (international normalized ratio) tests what and what pathway? what med would make you test this pathway?

A
Tests extrinsic coagulation pathway (VII); (common pathway V, X, II)
**Time (seconds) for clot to form
Measures:
Production of coagulation proteins
-Liver function
-Vitamin K dependent 
monitor in warfarin or coumadin therapy
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3
Q

PTT (partial thromboplastin time) tests what and what pathway? what med would make you test this pathway?

A

Tests intrinsic pathway (XI, IX, VIII); (common pathway V, X, II)
Measures:
**Production of coagulation proteins
Antibody interference with coagulation (lupus “anticoagulant”)
**heparin therapy

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4
Q

give an example of how physical injury can cause accelerated blood cell destruction

A

mechanical heart valve can shear RBCs

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5
Q

Erythropoietin produced by kidney in response to:

A
  • Anemia (low red blood cell mass)
  • Hypoxemia (low oxygen delivery)
  • -High altitude
  • -Lung disease: smokers
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6
Q

T/F: mild anemia is usually asymptomatic

A

true

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7
Q

T/F: anemia can trigger angina

A

yes with underlying heart disease

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8
Q

4 main anemia categories:

A
  • iron deficiency: microcytic
  • vit B12 def: macrocytic
  • sickle-cell: normocytic
  • beta-thalassemia: microcytic
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9
Q

what are some systemic complications of anemia?

A
  • Stomatitis: inflamm of mouth, tongue atrophy
  • Dysphagia, esophageal web
  • Nail changes (koilonychia - flat, spoon-shaped)
  • Pica: dirt eating
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10
Q

what are some ocular complications of anemia?

A
  • Conjunctival pallor
    • Conjunctival/retinal flame hemorrhages
    • Blue sclerae
    • Retinal venous distension
    • Retinal edema, exudates
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11
Q

what are some lab findings in iron def anemia?

A

Microcytic, hypochromic anemia

  • Low serum iron level (< 50 ug/dl)
  • High serum iron-binding capacity (> 400 ug/dl)
  • Low serum ferritin (< 20 ng/ml)
  • Decreased iron stores on marrow biopsy
  • *reticulocytes should be elevated to compensate
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12
Q

pernicious anemia:

A

destroyed gastric parietal cells

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13
Q

what role does vit B12 play in RBC formation?

A

critical in conversion of homocysteine to methionine for RBC creation

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14
Q

how is vit B12 absorbed?

A

B12 binds to intrinsic Factor (IF: secreted by gastric parietal cells) in the stomach

IF + B12 complex is absorbed in the distal ileum

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15
Q

what are some possible causes of vit B12 def?

A
veganism
alcoholism
Pernicious anemia (autoimmune destruction of parietal cells)
Gastrectomy
Inability to absorb IF + B12
Ileal resction, ileitis, celiac disease
Digestive enzymes absent
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16
Q

what are some neurologic side effects of vit B12 def

A
  • Numbness, other sensory abnormalities
    • Weakness, ataxia
    • Depression, memory loss, dementia
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17
Q

what are some ocular side effects of pern anemia def

A
  • Retinal, choroidal hemorrhages
  • Optic neuropathy
  • Ophthalmoplegia (rare)
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18
Q

lab findings for pern anemia def?

A
  • Macrocytic, megaloblastic anemia
    • Pancytopenia
      • Low serum B12 level
      • Elevated methylmalonoic acid and homocysteine
      • (Abnormal Schilling test)
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19
Q

what else should you check for with pernicious anemia?

A

autoimmune or gastric carcinoma

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20
Q

what is erythroid aplasia? what are some causes?

A

many causes, but RBCs not made properly
causes:
**Parvovirus B19 (Fifth disease / erythema infectiosum)
Infectious
Hepatitis viruses
HIV
-Iatrogenic (caused by meds)-usually reversible

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21
Q

what is Parvovirus B19 (Fifth disease / erythema infectiosum)?

A
  • Slapped cheek appearance with reticular peripheral rash in children
  • Arthralgias in adults-not actually arthritis
  • can lose baby
  • Aplastic crisis in sickle cell disease?
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22
Q

what causes sickle cell?

A

Mutation in hemoglobin beta chain (Glu6 -> Val6)

-Combination with normal a-chains produces Hemoglobin S

23
Q

what are some systemic assoc with anemia?

A
  • Splenic infarction
  • Recurrent bacterial infections (lung, bone, etc.)
  • Cerebrovascular events
  • Growth retardation
  • Liver, renal disease
  • Osteonecrosis
  • Recurrent aplastic marrow crises
24
Q

what are some ocular assoc with anemia?

A
  • Scleral icterus
  • Comma-shaped conjunctival veins
  • Retinal vascular occlusions, edema, atrophy
  • Neovascularization, retinal detachment
  • Hemorrhagic glaucoma, rubeosis iridis
  • Retinal, vitreous hemorrhages
  • Angioid streaks
  • “Sunburst” chorioretinal scars
25
what is sickle cell "disease"
Milder disease Higher blood viscosity Retinopathy may be more severe (than SS)
26
what is thalassemia?
- Unbalanced production of a- and b-chains | - Accelerated destruction of erythrocytes within marrow
27
what is Relative polycythemia?
Volume depletion (e.g. dehydration)
28
what are 2 drug induced causes of polycythemia?
``` EPO doping (sports) androgen overuse ```
29
what are some systemic effects of polycythemia?
Chronic tissue hypoxia: - Chronic lung diseases -Smoking / chronic CO exposure - Arteriovenous shunts -Abnormal Hgb (high O2 affinity) - High altitudes
30
what is Polycythemia Vera?
A clonal proliferation of early hematopoietic stem cells generally seen in older patients (60-80 yo) - Inappropriate overproduction of RBCs, WBCs & platelets - Increased blood viscosity - Despite increased platelets, function often ineffective (bleeding)
31
what clinical side effects do you see with Polycythemia Vera?
Facial plethora=red face Headache, dizziness, malaise Pruritis (especially feet) Cerebrovascular events Thrombophlebitis=clots in veins Epistaxis, easy bruising Hepatosplenomegaly Extramedullary hematopoiesis
32
what are some ocular features of polycythemia vera
- Conjunctival, retinal venous engorgement - Congested (“dusky red”) fundus - Retinal arterial, venous occlusions - Retinal hemorrhage, neovascularization - Ischemic optic neuropathy - Papilledema
33
what's one major cause of luekopenia?
drug-induced (just know there are many) - Antibiotics - NSAID’s - Anti-thyroid drugs - Antihypertensives - Thiazide diuretics - Anticonvulsants - Alkylating agents
34
what are some causes of leukemia?
* Mutations of cellular oncogenes – genetic (Down’s etc.), somatic, viral (HTLV), radiation, chemotherapy - Progressive infiltration/replacement of marrow, disruption of normal hematopoiesis -> bleeding, infection, anemia
35
what are the systemic side effects of leukemia?
- Anemia: Fatigue, headache, anorexia, weight loss - Abnormal bleeding (epistaxis, bleeding gums, ecchymosis) - Infection (often bacterial) - Veno-occlusive events, bone pain - Infiltration of liver, spleen, lymph nodes, skin (rash)
36
what are the ocular side effects of leukemia?
- Conjunctival, retinal hemorrhages - Retinal vascular occlusions - Orbital, conjunctival, retinal infiltration - Orbital chloroma (AML)??
37
what are some ocular side effects of lymphoma?
- Lid, conjunctival infiltrates - Orbital, lacrimal gland infiltration - Proptosis, diplopia. optic nerve compression
38
Hodgkins vs non-hodgkins lymphoma?
Hodgkin’s lymphoma diagnosed with Reed-Sternberg cell on pathology
39
Waldenstrom’s macroglobulinemia:
excess IgM secretion -Hyperviscosity syndrome (IgM)
40
what are some systemic side effects of multiple myeloma?
- Fatigue, weight loss, back pain, headache - Anemia - Abnormal bleeding, recurrent infection - Hypercalcemia, bone erosion - Renal failure
41
what are some ocular side effects of multiple myeloma?
- Crystalline deposits in cornea, conjunctiva - Band keratopathy, pars plana cysts - Orbital infiltration, papilledema, ophthalmoplegia - Retinal engorgement, edema, exudates, hemorrhages - Retinal vascular occlusions
42
how does aspirin vs NSAIDs affect platelet function?
Aspirin (irreversible inhibition), NSAIDS (reversible)
43
when do you see thrombocytopenia vs thrombocytosis?
Thrombocytopenia=low platelet - Idiopathic Thrombocytopenic Purpura (ITP)=kids - Thrombotic Thrombocytopenic Purpura (TTP) Thrombocytosis=high platelet - Infection, inflammation - Trauma, blood loss (reactive) - Post-splenectomy (spleen stores platelets)*
44
what are some ocular side effects of thrombocytopenia?
- Conjunctival petechiae - Orbital, retinal hemorrhages - Ophthalmoplegia (orbital or CNS hemorrhage) - Stroke syndromes (CNS hemorrhages) - Ischemic optic neuropathy (systemic hypotension)
45
what is von willebrand's disease?
Inherited bleeding disorder -VW factor functions in: Platelet plug formation Platelet adhesion to site of vascular injury *so basically these people can't form platelet plug, although platelet count is normal
46
what is hemochromatosis?
hereditary disorder with excessive absorption of dietary iron /abnormal accumulation of iron in tissues
47
what are some clinical features of hemochromatosis?
- Skin pigmentation - Arthritis * Hepatomegaly *splenomegaly * Cardiomyopathy -Diabetes, other endocrinopathy - Conjunctival, lid-margin hyperpigmentation
48
T/F: genetics is not involved in hemochromatosis
false, it is involved; common in caucasians and north american ancestry
49
what should you rule out in the context of DM with liver disease?
hemochromatosis
50
Excesses of procoagulant proteins is assoc with what factor?
Factor V Leiden
51
Deficiencies of natural anticoagulant proteins is assoc with what proteins?
protein S and C
52
Protein C does what? | what are these patients at risk for?
Inactivates Factors Va and VIIIa, which are trying to clot * protein C is a vitamin K dependent protein - at risk for thrombosis, especially: - Deep vein thrombosis (DVT) in 65% - Pulmonary embolism (PE) in 40%
53
what is the function of Factor V leiden? what happens when it is mutated?
Factor V binds to platelets, serves as receptor for Xa Inactivated by activated Protein C -in mutation, protein C can't bind and deactivate it, creates hypercoagulable state ***Most common genetic thrombophilias BCP risk is additive, also at risk for spontaneous abortion