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LHT2 > RBC disorders CIS > Flashcards

RBC disorders CIS Flashcards

1
Q

Anemias- pathophysiologic categories

A
  • by Retic count!!
  • dec red cell prod
  • inc red cell destruction or loss
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2
Q

Aplastic Anemia- characterized by?

A

-loss of hematopoietic cells, fatty replacement of marrow, and pancytopenia

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3
Q

Aplastic anemia- causes?

A
  • may be congenital, but most secondary
  • infection- viral hepatitis, EBV, parvovirus, HIV
  • radiation
  • drugs- Chloramphenicol, quinacrine!! (1 in 30,000)
  • chemicals
  • toluene
  • glue
  • immunologic- autoimune, PNH
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4
Q

Chronic kidney dz- causes anemia how?

A
  • shortened RBC survival time
  • dec EPO levels
  • dec RBC prod indep of EPO
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5
Q

Chronic kidney dz- clinical manifestations

A
  • normochromic normocytic anemia
  • acanthocytes on PS (spikey cells)!!!!
  • renal failure
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6
Q

megaloblastic anemia- causes?

A
  • Vit B12 or folic acid def (95%)

- drugs or toxins (arsenic)

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7
Q

megaloblastic anemia- clinical aspects

A
  • neuro sx- B12 def- degeneration of dorsal/lateral columns

- psychosis (severe B12 def)

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8
Q

refractory megaloblastic anemia- caused by?

A

myelodysplasia

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9
Q

Disturbance of Hemoglobin Synthesis

A
  • iron def
  • hemoglobinopathies
  • porphyrias
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10
Q

Iron def anemia- caused by?

A
  • most common cause of anemia in world!!
  • dietary def- infants (bottle milk)
  • pregnant women, children dont get enough in diet
  • GI bleeding- most common cause!!!
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11
Q

Factitious anemia- due to?

A

auto phlebotomy

  • Lasthenia de Ferjol syndrome
  • psychiatric problems
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12
Q

iron def anemia- other causes?

A
  • hemolysis/hemoglobinuria (PNH)
  • post gastrectomy- insuff acid to maintain iron in Fe2+ state
  • intestinal resection
  • acquired- NRAMP2 gene- facilitates Fe2+ transport across brush border
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13
Q

iron def anemia- lab

A
  • dec serum iron and ferritin

- inc TIBC

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14
Q

anemia of chronic dz- etiology

A
  • dec RBC survival time
  • dec erythropoiesis
  • disturbed iron metabolism
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15
Q

anemia of chronic dz- lab

A
  • dec serum iron and TIBC
  • normal/inc serum ferritin
  • normocytic normochromic anemia
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16
Q

anemia of chronic dz- tx

A
  • malignancy

- benign- myelofibrosis, gaucher’s, histiocytosis, sarcoidosis

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17
Q

ice cravings

A

-pagophagia

18
Q

enzyme def

A
  • G6PD
  • PK
  • classic presentation- hereditary nonspherocytic hemolytic anemia!!
19
Q

G6PD def

A
  • food/drug induced
  • FH (x-linked)
  • Heinz bodies (denatured hemoglobin)
  • bite cells
20
Q

G6PD def- lab

A
  • inc retic count

- hemolysis- dec haptoglobin, inc LDH and bilirubin, urine hemosiderin inc

21
Q

G6PD def- tx

A
  • avoid food/drugs that cause hemolysis

- splenectomy help some

22
Q

March hemoglobinuria

A

(sports anemia, footstrike hemolysis)

  • in marathon runners!!
  • feet striking ground- lysis of RBCs in capillaries
23
Q

Cardiac anemia

A

-severe aortic stenosis and prosthetic valves

24
Q

chemical induced anemia

A
  • lead
  • copper
  • oxygen- Astronauts!! (pure oxygen atmospheres)
  • insect venoms- Bee/wasp, spiders
25
Q

lead- anemia

A
  • interferes with cation pump- shortened RBC survival time

- slows prod of RBCs in marrow

26
Q

most common cause of hemolytic anemia in the world??

A

Malaria!!

-urine- “blackwater fever”- contains hemoglobin, very dark color

27
Q

Bartonellosis

A

(Bartonella bacilliformis)

  • adheres to RBC membrane
  • transmitted by sand flea
  • deadly!
28
Q

Babesiosis

A

(Babesia microti)

  • NE US!!
  • parasite in rodents
  • “Maltese Cross” in RBCs with Giemsa-stained peripheral smears!!!!
29
Q

Immune mediated hemolytic anemia- caused by?

A
  • warm reacting ab’s- IgG

- cold reacting ab’s- IgM- complement!

30
Q

Immune mediated hemolytic anemia- clinical features

A
  • positive direct antiglobulin test (DAT, Coombs)- Ig coats RBC surface
  • indirect Coomb’s test- RBC-targeted Ig on serum
  • normochromic normocytic anemia
  • elevated retic count
  • hemolysis evidence on lab tests
31
Q

Tx of warm ab hemolysis

A
  • not in imminent danger
  • Steroids!! (high dose)- mainstay of tx!!
  • immunosuppressives (cyclophosphamide, azathioprine)- considered in refractory cases
  • splenectomy- in pts where chronic steroid use is required
32
Q

cold ab hemolytic anemia- etiology

A
  • lymphoprolif disorders!!
  • cold agglutinin dz (Mycoplasma!!)
  • tertiary syphilis!! (Donath-Landsteiner)
33
Q

cold ab hemolytic anemia- tx

A
  • avoid cold

- tx infections

34
Q

Major porphyrias

A
  • Acute intermittent porphyria

- porphyria cutanea tarda

35
Q

Acute intermittent porphyria- caused by?

A
  • def of PBG (porphobilinogen) deaminase
  • inc incidence in psychiatric pts
  • European descent
  • excretion of inc amts of ALA and PBG in urine
36
Q

Acute intermittent porphyria- clinical features

A
  • sx usually after puberty
  • abd pain- most common sx!!
  • occasional attacks
  • tachycardia, HTN
  • neuro changes- peripheral neuropathy, anxiety, insomnia, depression, hallucinations, seizures
37
Q

Acute intermittent porphyria- precipitating factors?

A
  • hormones- pregnant women
  • drugs- anticonvulsants, barbiturates, sulfonamides, alcohol
  • low caloric intake
  • infection
  • surgery
38
Q

Acute intermittent porphyria- tx

A
  • hydration with carbohydrate solutions
  • Hemin infusion (dec porphyrin prod via neg feedback)
  • beta blockers- for tachycardia and HTN
  • analgesics
39
Q

Porphyria cutanea tarda- caused by? clinical?

A

def of uroporphyrinogen decarboxylase in liver

  • cutaneous photosensitivity- major sx!
  • no neuro effects
  • cutaneous bulla formation on sun-exposed areas
  • “pseudoscleroderma”
40
Q

Polycythemia Vera- clinical presentation

A
  • facial rubor!!
  • hyperviscosity signs- HA, dizziness, blurred vision
  • pruritus with hot shower or bath!!
41
Q

Polycythemia Vera- lab

A
  • inc RBC’s
  • elevated LAP (leukocyte alkaline phosphatase) score
  • inc WBC and/or plt counts in 50%
42
Q

Polycythemia Vera- tx

A
  • phlebotomy- lower RBC mass!

- hydroxyurea

LHT2 (17 decks)

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