It is a case of patient:
An 50 year old female, in 2008 was diagnosed the Coeliac disease. From young ages have got per os iron, but from 3,5 years getting it IV. every 3 month.
For dyspnoea was at cardiologist, who made an ECHO, and saw the large RA, mild Tricuspid insuffitientia and the right apex was thickened and a thrombus was there.
Chest CT made: PE excluded
Cardiac MR: thickened right apes, and septum. Thrombus in right apex. Fibrotic thickening in right ventricle. Good Left and Right systolic function. No more.
Lab parameter: The eosinophilia was only once high (from docs what we have) when she had a maculo-papular exanthema. (39%) After that and before it was normal.
Se Fe, transferin and ferritin now is low.
For neoplasma syndroma was made more exam and show negative result. (Gastro- colonoscopia, gynecology exam Abdominal sonography, consultation with immunologist, etc -)
My question and hypothesis:
Hypothesis: It my be a Löffler endocarditis, because for 2008 she do not know for Coeliac disease and have many times allergic reactions, including itching and rash. Maybe the persistent high level of eosinofils caused the fibrotic changes.
But why other causes where high eosinofil level is persisting long time there is no fibrotic changes in heart or other organs, eg. COPD or Asthma bronchiale?
And how now confirm or reject the Löffler endocarditis or other endocardial fibrosis?
And fibrosis what is the treatment, for your practice?
Thank you for answers!
Very interesting case. I think LGE and T2 should be the right way to confirm the diagnosis because otherwise all other criteria for Löffler endocarditis are met. I would ask the CMR specialist if any areas of heterogeneous areas of hyperintense signal with subepicardial districution are seen, otherwise EMB probably would be the next step (I would suspect fragmentation of muscle bundles by eosinophils, myocyte destruction and coagulation necrosis in case of Löffler endocarditis).
Thank you Asatryan, you think that long eosinofil level witch may be persist (what we dont know) by Coeliac disease may cause itt?
Following administration of contrast material in the circular muscle of the left ventricle late enhancement signal is not detected.
And now what to do? There is no high eosinophil level. Steroids? I think no.
Go for transplant?.... Wait...
I would think more EMB. If EMB would be positive for diagnosis of Loffler's, then steroids. I believe its worth trying steroids before transplant.
She was admitted and went home. Will be an cardiac MRI control in January.
We thought this is, maybe, Löffler endocarditis.
If general condition will go bad, think about EMB or transplant.
No steroid or cytotoxic was prescribed.
Colleague Norbi Gabor!
Your case in a patient of 50 years, i have diagnosed as restrictive cardiomyopathy, complicated with endomyocardial fibrosis.
This disease as well as s “Leffler endocarditis” associated with eosinophilia, but occurs in a mild form. In both diseases the etiological factor is not known. Considered as an option hypereosinophilic syndrome, mainly affecting the heart. According to our observations the development hypereosinophilic syndrome may be induced by activation of Paecilomycosis infection in the blood, where eosinophils serve a protective function, acting as effector cells. Target cells spherules are fungi of the genus Paecilomyces.
I consider it is necessary to examine your patient blood to identify activation of Paecilomycosis infection in the blood. Use the direct quantification of mature spherules fungus of Paecilomyces using a counting chamber (Goryaeva).
This technique is described in my questions to RG. To help you, i will send pictures from a blood sample the patient T., 12 years old, with hypereosinophilic syndrome Paecilomyces etiology and initial signs of restrictive cardiomyopathy. The causative agent - is the fungus genus Paecilomyces species Paecilomyces variotii.
Sincerely Alima Akhunova.
The morphological structure of the tissue forms a parasitic fungus
P. variotii in the blood of the patient T., 12 years old. The content of eosinophils in the peripheral blood of -50.4%, the quantitative content of mature spherules fungus in 1µl of blood - 28,000 at the rate of 1000 - 6000. Attention is drawn to the variety of types of tissue forms, including the presence of yeast cells.
- Badges
- Science topic
- Similar topics
- Chemistry
- Pharmacology
- Medical Pharmacology
- Clinical Pharmacology