RBC disorders Hubbard Flashcards
anemia from increased RBC production intrinsic
membrane defects–> HS
enzyme deficiency–> G-6PD deficiency
anemia from increased RBC protduction extrinsci
mechanical–> march hemoglobinuria
chemical/physical–> lead, copper
infection–> malaria, babesia, bartonella
antibody acquired–> autoimmune hemolysis
hyperspelnism–> portal HTN, others
classic presentatin of enzyme deficiency anemia
hereditary nonspehrocytic hemolytic anemia
what can induce hemolysis with G6PD
fava beans, sulfa containing meds
x linked
heinz bodies
- what disorder
- another name
- type of anemia
collection of denatured hemoglobin
seen early in hemolytic episode of G6PD deficient pts
also bite cells
normochromic normocytic anemia`
lab features in enzyme deficiency
elevated reticulocyte count bc BM is trying to physiologically respond (unless pt is fe, b12, or folate deficient)
lab features of hemolysis
decreased haptoglobin
increased LDH and bilirubin
urine hemosiderin may be elevated
plasma hemoglobin may be elevated if hemolysis severe
cardiac anemia
pts with severe aortic stenosis (valve gradient >50mmHg) and pts with prosthetic valves
blackwater fever
malaria
oroya fever
bartonellosis
clinical features of immune mediated hemolytic anemia
normochromic normocytic anemia
elevated reticulocyte count
evidence of hemolysis on lab tests
immune mediated hemolytic anemia treatmetn of warm antibogy
steroids– use high doses
immunosuppressive like cyclophosphamide and azathiprine
splenectomy for pt in whom chronic steroid use is required
etiology of cold ab hemolytic anemia
lymphoproliferative disorders
mycoplasma
tertirary syphillis
clinical features of cold antibody hemolytic anemia
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
treatment of cold Ab hemolytic anemia
supportive
avoid cold
treat infections
acute intermittent porphyria
population
deficiency
increased in what pts
scandinavians, british, eastern europe
deficiency of porphobilinogen deaminase (PBG)
increased incidence in psychiatric pts
Acute intermittent porphyria increased what in urine
ALA and PBG
most common symptom of AIP
abdominal pain (nausea, vomitin, constipation (ileus))
also can have neuro changes, tachycardia, HTN
precipitating factors in AIP
hormones, more frequent in women and attacks during pregnancy
drugs- anticonvulsants, barbituates, sulfonamides, alcohol
low caloric intake
infection
surgery
treatment of acute AIP attack
hyrdration with carb solutions (D5 or D10)
hemin infusion– similar to heme, dereases porphyrin production
beta blockers for tachy and HTN
analgesics
porphyria cutanea tarda (PCT)
deficiency of what
clinical features
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
PCT may occur in what (organ problem)
end stage renal disease
lab features of PCT
abnromal liver function tests
increased iron buildup in liver
increased porphyrins in urine– ALA primarily
increased porphyrins in stool
treatment for PCT
stop exacerbating factors
phlebotomy for iron overload- can help cutan manifest
chloroquin
clinical features of PV
facial rubor
hyperviscosity (headache, blurred vision)
pruritis with hot shower or bath
splenomegaly in 70%
PV lab features
increased RBC (may be nucleated ones too)
elevated LAP
increased WBC and or platelet counts in 40-60% pts
PV clinical course
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
PV DD: secondary cuases of increased RBC count
hemoconcentration COPD EPO tumors (RCC, neuroendocrine tumors) hemoglobinopathy with high affinity hemoglobin living at high altitude
treatment PV
phlebotomy every 1-2 weeks
hydroxyurea
normal reticulocyte count
0.5-1.5%
a woman with tachy and mildy hypotensive can get this from which of the following
IDA
aplastic anemia
folate def
IDA
what is the name for ice cravings
pagophagia
what is erythrodonita
fluorescence of teeth in pts who have veregate porphyria when shine lamp on teeth
