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Heme and Lymph Final DF > RBC disorders Hubbard > Flashcards

RBC disorders Hubbard Flashcards

1
Q

anemia from increased RBC production intrinsic

A

membrane defects–> HS

enzyme deficiency–> G-6PD deficiency

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2
Q

anemia from increased RBC protduction extrinsci

A

mechanical–> march hemoglobinuria

chemical/physical–> lead, copper

infection–> malaria, babesia, bartonella

antibody acquired–> autoimmune hemolysis

hyperspelnism–> portal HTN, others

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3
Q

classic presentatin of enzyme deficiency anemia

A

hereditary nonspehrocytic hemolytic anemia

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4
Q

what can induce hemolysis with G6PD

A

fava beans, sulfa containing meds

x linked

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5
Q

heinz bodies

  • what disorder
  • another name
  • type of anemia
A

collection of denatured hemoglobin

seen early in hemolytic episode of G6PD deficient pts

also bite cells

normochromic normocytic anemia`

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6
Q

lab features in enzyme deficiency

A

elevated reticulocyte count bc BM is trying to physiologically respond (unless pt is fe, b12, or folate deficient)

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7
Q

lab features of hemolysis

A

decreased haptoglobin
increased LDH and bilirubin
urine hemosiderin may be elevated
plasma hemoglobin may be elevated if hemolysis severe

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8
Q

cardiac anemia

A

pts with severe aortic stenosis (valve gradient >50mmHg) and pts with prosthetic valves

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9
Q

blackwater fever

A

malaria

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10
Q

oroya fever

A

bartonellosis

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11
Q

clinical features of immune mediated hemolytic anemia

A

normochromic normocytic anemia
elevated reticulocyte count
evidence of hemolysis on lab tests

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12
Q

immune mediated hemolytic anemia treatmetn of warm antibogy

A

steroids– use high doses
immunosuppressive like cyclophosphamide and azathiprine
splenectomy for pt in whom chronic steroid use is required

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13
Q

etiology of cold ab hemolytic anemia

A

lymphoproliferative disorders
mycoplasma
tertirary syphillis

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14
Q

clinical features of cold antibody hemolytic anemia

A

chronic anemia in some
some have severe epidosdic anemia following exposure to cold
veno-occlusive phenomena may result in sluding of RBCs in affected caps

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15
Q

treatment of cold Ab hemolytic anemia

A

supportive
avoid cold
treat infections

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16
Q

acute intermittent porphyria
population
deficiency
increased in what pts

A

scandinavians, british, eastern europe
deficiency of porphobilinogen deaminase (PBG)
increased incidence in psychiatric pts

17
Q

Acute intermittent porphyria increased what in urine

A

ALA and PBG

18
Q

most common symptom of AIP

A

abdominal pain (nausea, vomitin, constipation (ileus))

also can have neuro changes, tachycardia, HTN

19
Q

precipitating factors in AIP

A

hormones, more frequent in women and attacks during pregnancy

drugs- anticonvulsants, barbituates, sulfonamides, alcohol

low caloric intake

infection

surgery

20
Q

treatment of acute AIP attack

A

hyrdration with carb solutions (D5 or D10)

hemin infusion– similar to heme, dereases porphyrin production

beta blockers for tachy and HTN

analgesics

21
Q

porphyria cutanea tarda (PCT)
deficiency of what
clinical features

A

most common porphyria
due to deficiency of uroporphyrinogen decarboxylase in liver
cutaenous photosensitivity when exposed to sun
-seems to occur with alcohol consumption
may get skin tightening (pseudoscleroderma)
-hereditary and sporadic form

22
Q

PCT may occur in what (organ problem)

A

end stage renal disease

23
Q

lab features of PCT

A

abnromal liver function tests
increased iron buildup in liver
increased porphyrins in urine– ALA primarily
increased porphyrins in stool

24
Q

treatment for PCT

A

stop exacerbating factors
phlebotomy for iron overload- can help cutan manifest
chloroquin

25
Q

clinical features of PV

A

facial rubor
hyperviscosity (headache, blurred vision)
pruritis with hot shower or bath
splenomegaly in 70%

26
Q

PV lab features

A

increased RBC (may be nucleated ones too)
elevated LAP
increased WBC and or platelet counts in 40-60% pts

27
Q

PV clinical course

A

50% mortality at 18 months without treatment

with treatment pts can liver for years to decades

usual cause of death in treated pts is progressive marrow fibrosis with pancytopenia aka spent phase polycythemia

28
Q

PV DD: secondary cuases of increased RBC count

A 
hemoconcentration
COPD
EPO tumors (RCC, neuroendocrine tumors)
hemoglobinopathy with high affinity hemoglobin
living at high altitude
29
Q

treatment PV

A

phlebotomy every 1-2 weeks

hydroxyurea

30
Q

normal reticulocyte count

A

0.5-1.5%

31
Q

a woman with tachy and mildy hypotensive can get this from which of the following
IDA
aplastic anemia
folate def

A

IDA

32
Q

what is the name for ice cravings

A

pagophagia

33
Q

what is erythrodonita

A

fluorescence of teeth in pts who have veregate porphyria when shine lamp on teeth

Heme and Lymph Final DF (8 decks)

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