Differential Diagnosis Problem

[nickolas]

I don't know if this is the place to post it but here it goes.

40 year old male presents with jaundice, peripheral edema, NYHA IV to hospital. No previous history of heart disease or jaundice. U/S indicates low EF, global hypokinesia. Labs show Tbil 12mg/dl ~50% dbil, mildly elevated lft's, nothing else unremarkable.
After administration of diouretics and inotropes patient is asymptomatic but Tbil continues to be elevated, ranging between 7mg/dl and 12mg/dl always 50% dbil, 2 months since patients initial visit.
In the mean time there has been a slow decline in Hb (from 13grams to 11grams). On patients last visit there is a 2gram drop in Hb from a week a go (from 11grams to 9grams). Patient denies any sign of hemorrhage, Coombs upon first visit was negative.
What is your next step?

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[jdh71]

I don't know if this is the place to post it but here it goes.

40 year old male presents with jaundice, peripheral edema, NYHA IV to hospital. No previous history of heart disease or jaundice. U/S indicates low EF, global hypokinesia. Labs show Tbil 12mg/dl ~50% dbil, mildly elevated lft's, nothing else unremarkable.
After administration of diouretics and inotropes patient is asymptomatic but Tbil continues to be elevated, ranging between 7mg/dl and 12mg/dl always 50% dbil, 2 months since patients initial visit.
In the mean time there has been a slow decline in Hb (from 13grams to 11grams). On patients last visit there is a 2gram drop in Hb from a week a go (from 11grams to 9grams). Patient denies any sign of hemorrhage, Coombs upon first visit was negative.
What is your next step?

Is this homework?

 

[nickolas]

No this is not homework...

 

[jdh71]

No this is not homework...

Does his heart still suck 2 month later?

 

[nickolas]

Yeap! It does...
We performed right heart catheterization and filling pressures on both ventricles were very high ~20mmHg Right Atrium and ~30mmHg Left Atrium.
(patient asymptotic, not sounding wet, no peripheral edemas having lost 6kg-10% of his weight- since initial visit)
This is good reason for elevated Tbil but not for 50%dbil.
His liver is palpable not tender, not soft but evenly surfaced.

The critical question is why this sudden drop of Hb and how to figure out if there is a liver disease as well.

 

[SimonSez]

I would consider doing PT/INR/PTT, Iron Studies (serum Ferritin, TIBC, Transferrin Saturation) and Hepatitis B/C serologies.

Hemochromatosis can account for both the deranged LFT's and low EF (since it is associated with cardiomyopathy).

Are his glucometer readings normal? Does he have recent onset Diabetes Mellitus? Any skin discoloration?

You said that the Hb is low. We need more information, in particular : what is the MCV? Are the Platelet count and WBC normal?

 

[nickolas]

I would consider doing PT/INR/PTT, Iron Studies (serum Ferritin, TIBC, Transferrin Saturation) and Hepatitis B/C serologies.

Hemochromatosis can account for both the deranged LFT's and low EF (since it is associated with cardiomyopathy).

Are his glucometer readings normal? Does he have recent onset Diabetes Mellitus? Any skin discoloration?

You said that the Hb is low. We need more information, in particular : what is the MCV? Are the Platelet count and WBC normal?

MCV is normal, though upon first visit was low. PLT and WBC counts are normal. There is a great variation on the size of red blood cells. No diabetes/skin discoloration.
Hemochromatosis... ferritin is elevated but this is normal with advanced heart failure, TIBC though sounds a good idea!

edit: Pt is negative for Hepatitis, no history of alcohol abuse

 

[gutonc]

What does the peripheral smear look like?
Imaging?
FOBT/Colo? (Almost nobody has an idea whether their poop is "black" or not. I don't assume patients are lying, just that they have no idea what they're talking about and whatever they tell me is wrong.)
Just marrow him already...be sure to get the Congo Red stain.

 

[jdh71]

What does the peripheral smear look like?
Imaging?
FOBT/Colo? (Almost nobody has an idea whether their poop is "black" or not. I don't assume patients are lying, just that they have no idea what they're talking about and whatever they tell me is wrong.)
Just marrow him already...be sure to get the Congo Red stain.

lolhemeonc

 

[jdh71]

Yeap! It does...
We performed right heart catheterization and filling pressures on both ventricles were very high ~20mmHg Right Atrium and ~30mmHg Left Atrium.
(patient asymptotic, not sounding wet, no peripheral edemas having lost 6kg-10% of his weight- since initial visit)
This is good reason for elevated Tbil but not for 50%dbil.
His liver is palpable not tender, not soft but evenly surfaced.

The critical question is why this sudden drop of Hb and how to figure out if there is a liver disease as well.

Any diastolic dysfunction? Is the heart big? He's got a low EF and can't walk to the kitchen without getting SOB, but is it dilated. Walls thickened?

I was trying to lead to you hemochromotosis, but it's already been brought up. Let me also add, amyloidosis is also a consideration. Also strange rheum (It's lupus! NO!! It's not lupus!!), some of your mixed connective tissues disorders can crap on the liver and the heart. Hell, he could have gotten a virus and everything can be explained by passive congestion. We don't know how long the pressures have been backing up into his liver . . . passive congestion can given you biliary stasis and portal htn backing everything up into the spleen.

EDIT: Oops WBCs and Plts are ok, maybe not splenic sequesteration. I'd probably EGD this guy too.

 

[Mr.Bip]

Agree with jdh71 about the passive congestion. It's hard to explain concurrent such dramatic heart failure + biliary stasis from hemochromatosis, amyloidosis, or rheum issue if it presented relatively acutely as it sounds in your little vignette (though who knows). BUT, a myocarditis could have led to acute heart failure --> biliary stasis. I bet the heart is the primary organ dysfunction here.

 

[Gastrapathy]

RUQ US with doppler, serologic eval for chronic liver disease, physical exam for stigmata of CLD...all will be unremarkable.

Liver no like sick heart.

 

[Polish Farmer]

You forgot to mention the results of his left hearth cath, which I am sure was done. Did he have ischemic coronary disease as explanation for his heart failure? Were his valves ok on echo? What was his TSH? Did he have any further workup for his CHF? Also, were the repeat LFTs done while the patient was stable as an outpatient and euvolemic? What is his renal function?

 

[J-Rad]

You forgot to mention the results of his left hearth cath, which I am sure was done. Did he have ischemic coronary disease as explanation for his heart failure? Were his valves ok on echo?

And if a left heart was accomplished, was an aortic pullback done?

 

[gutonc]

And if a left heart was accomplished, was an aortic pullback done?

And...if this isn't really a homework help thread (as jdh thought at the outset and I continue to think), please post an update and answers to some of these questions.

Otherwise I'll close it as a presumed homework help thread.

 

[brc12]

I've had a few patients with similar liver abnormalities purely from their severe cardiomyopathy.