jean robert
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A 20 y.o. African American male is evaluated for hematuria. His coagulation studies are within normal limits.His Hb electrophoresis shows the following:
Hb A 60%
Hb S 40%
Which of the following is most likely true about this patient?
a.-Irreversible sickle cells are present on the peripheral smear
b.-Reticulocyte count is elevated
c.-He s protected from P. falciparum
d.-MCHC is decreased
e.-He will probably develop painful crises.
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| new_n_lost
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e.-He will probably develop painful crises.
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| sprint123
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I think HE IS protected from Falciparum goes well....
Sickle cell trait patients rarely go into crisis only under severe hypoxia...BUT,They are definitely protected against falciparum
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| drmohamed811
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C is the answer , the patient is a sickle cell trait
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| rock
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this pt has sickle cell trait...but he has hematuria which points towards a potential crisis...he probably had microinfarctions in the renal papillae which caused hematuria...and his hemoglobin S is also 40% which can be quite significant in conditions of stress, which now he seems to be under considering the history of hematuria...
but then he is also going to be protected against p. falciparum and wont have all the
effects of malaria like a normal person would. it does provide a survival advantage.
i would choose E because of the history of hematuria and that the question tells us hbS is 40%. not sure though.
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| new_n_lost
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his Hb S is 40% based on that fact he has got more tendency to move towards painful crisis( the trigger cna be anything) true the Pt is safe from P.Falciparm but the question here is that he has got atleast 40% of Hb S n 60% of Hb A 60% which is enuf to get u a malarial inf.
this is a UW q the Explanation is going to be somehow realy good. I m sure
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| keepgoing
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mmm very good question...good point rock...i will go for e as well n looking forward to the explanation...
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| treblechar
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HArrison's 16E p 595 table 91-2: therfore even if patient has hematuria he can just have a sickle cell trait Hb S/A: 40/60 sickle cell anemia etc has abnormal Hgb, MCV
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| eva
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if the patient have hematuria there are thrombi formation ,consequently he can have painful crisis.It's what I think.
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| rock
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good question indeed keepgoing. made me think which i rarely do
jean, we are all waiting for the explanation.
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| doc_clotaire
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C is totally and unequivocally the answer
That is Siclke cell trait , painless hematuria in AA is typical symptom , no risk of painfull crisis
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| jole
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C
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| rock
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jean...can yu pls post the answer with the explanation.
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| monam84
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C. Protective against P. falciparum . The answer is not E Because an HbS conc greater that 60% is the most important factor for sickling- HbS concentration is too low in a person with sickle trait to produce sickling in peripheral blood- so there are NO sickled cells in sum1 with the trait. (goljan RR p 217)
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| jean robert
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 C is effectively the correct answer.
Patients who are Heterozygous for the Sickle Cell Trait (Hb AS) have Hb composed of 35-40% Hb; They are generally protected from Sickle cell Crises, aplatic crises and sequestration crises by the presence of > 50% normal hemoglobin (Hb A). Patients with sickle cell TRAIT are usually asymptomatic, although they may develop hematuria and a limited ability to concentrate urine. A high incidence of UTI and splenic Infarction at high altitude have also been reported. In contrast, patients with homozygous SS have severe disease with > 80% HbS.
<>
Although Homozygotes for the sickle cell Trait are often very ill, heterozygotes enjoy relative protection from Plasmodium falciparum(malaria). The exact mechanism is not known; proposed mechanisms include increased sickling of parasitized sickle cell trait red blood cells and accelerated removal of these cells by the splenic monocyte macrophage system.
(Choice E) It s unlikely that this patient will develop painful crises as he s protected by the 60% Hb A(normal Hb).
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| rock
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thanks for the answer and explanation jean. and this was a v. good question
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| drmohamed811
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YES jean robert C IS THE ANSWER.... and your explanation is the PERFECT one....
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