A 30 year old physician presents to the physician because his morning urine is dark red brown in color. He describes several months of increasing weakness and dyspnea on exertion, several episodes of cramping abdominal pain and recently developed a cold. Physical examination reveals pallor and mild jaundice. A urine sample is taken in the morming which appears yellow ans clear. Which of the folowing additional tests will detect abnormal results.
1.Urine complete cell count
2. Urine free hemoglobin
3. Urine free myoglobin
4.Urine hemosiderin
5. Urine red cell count
Ans. is 4. unlike its name Paroxsymal nocturnal hemoglobinuria...it's mostly a chronic hemolytic codition (suggested by increasing weakness n dyspnea)..as the urine apeears yellow n clear not coke colored..less likely that he is having acute hemolysis..so hemoglobinuria is less likely in this case...hemosiderinuria is a marker of chronic intravascular hemolysis......hope im correct....!!
perfect!!thats what i thought too,though my mind was going towards option B first...
what is the Dx here and how you pick this lab. please, upaudel and drabhijit?
hi 99 maniac
first id like to tell u that ur Gr8 on this forum i saw some of ur postsn and they are awsome
well the condition above is paroxysmal nocturnal hemoglobinuria...In the above patient at first look i was tempted to mark..Urine hemoglobin ,,however this is seen only in acute conditions..the above patient seems to be have a chronic hemolytic anemia due to the condition,this results in accumulation of hemosiderin within the renal tubules..over a period of time these lining cells of the tubule exfoliate and lead to increse in urine hemosiderin...The cause is due to a defect in a gene known as PIG-A...it is diagnosed by Hams test which demonstrates in vitro complement induced lysis in acidified serum or can be diagnosed by flow cytometry demonstrating absense of cd 59 negative erythrocytes..However in an acute hemolysis in this patient the urine wud be coke colored and wud have free hemoglobin in large amounts
hope u got it!!
regards
abhijit
thank u for the complement drabhijit, you guys are all excellent. it looks that this dz (PNH, i think that is the dx if I am not mistaken) is genetic dz, my question why a physician get that dx late at 30 years of age, why it take so long to reach this dx. Is this the classic way to explain this dz. And in the stem it did not say that change in urine color happens in the morning.
Hey guys,
99Maniac,abhijit ,victor u guys are great.
Pl somebody answer my q what type of hy reactions does hapten cause.
I think it is mostly type 2 .
But there r some drugs cause type 3???
Please answer.
Thanks
Dear 99 maniac
U must understand that this is a form of ACQUIRED hemolytic anemia and generally presents after 20 years of life so the late diagnosis is justified...
and this is not the classical presentation of this disease,...Since u generally wud have coke colored urine in classic PNH...but we must keep unusual presentations also since they are heavily tested
abhijit
you are right thank for very useful explanation.