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Author Topic: Hello! New here - we suspect Evans Syndrome  (Read 46 times)

KatColM_O

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Hello! New here - we suspect Evans Syndrome
« on: December 07, 2017, 12:10:51 AM »

Hi Everyone! I was referred to this forum by a member in the BoxerWorld forum as my dog Millie (9 year old boxer) was recently diagnosed (loosely anyway) with an autoimmune disorder. From what I have learned, she has the symptoms of Evans Syndrome in that she has essentially no platelets and significantly decreased red blood cells and it's getting worse. We haven't ruled out lymphoma, but the vet wants to see if she responds to the immunosuppressive therapy.

To give a little history - about a year and a half, almost 2 years ago she first presented with low platelets. They suspected erlichea and have a long course of doxycycline. She seemed to do ok and her platelets went back up, not entirely, but improvement nonetheless.

Fast forward to this past July, she had a check up and her platelets were still low (78K) and we did another course of doxycycline. In August she got really sick and wound up back at the vet and hospitalized for a day and a half. Her platelets had dropped again into the 40s. I can't exactly keep straight the course of events from there, but she seemed to recover well enough and we went back for follow-up blood work in Sept. Her platelets had gone down again and were in the 20s. We started an aggressive course of predinose which she did not tolerate well at all. After a few days she began vomiting what looked like blood and her stool was really dark. We managed to avoid the e-Vet that weekend, but saw our vet immediately and she was taken off the prednisone. Her platelets were now 5K and her red blood cells has dropped. The vet suspected it was due to some kind of stomach ulcer caused by the prednisone and so we waited to see if they would bounce back up at all.

Well..they haven't. She still has essentially no platelets and her RBCs are continuing to drop.

This past weekend she had a collapse, but it was so brief I thought I'd imagined it. When she collapsed again on Monday we went right back to the vet. RBCs down even more (26%), still no platelets.

The vet did an abdominal ultrasound and said she saw no signs of abdominal tumors or unusual growths, although one lymph node was somewhat enlarged. She said we needed to start an immunosuppressive right away and cyclosporine has been called in to a local compounding pharmacy to alleviate some cost. I will pick that up tomorrow.

She is currently on Imuran 50mg (1 tablet once a day for 14 days), Famotidine 10mg (1.5 tabs 2x/day) and sucralfate 1g (2x/day given 1 hr after other meds).

She has also been on Proin for incontinence for quite some time since she has had incontinence issues since she was a baby, but it's much worse with the steroids and she requires a diaper. She has always had elevated kidney values which we believe to be the culprit of the incontinence, but this has been since before she was even 6 months old. I had originally been told that she possibly had Juvenile Renal Disease and we would be lucky to hit 2 years...well...here we are and she just turned 9 a month ago.

She has in the last few months also had a little bit of fecal incontinence where I have found little poop nuggets (no better way to describe it) where she had been laying. It's not a regular occurrence, but I thought worth mentioning.

She seems to be losing some weight, but still seems to have an appetite. She only weighed 50lbs to begin with and I'm guessing she is closer to 40lbs now.

I am here to hopefully find some information that could help our vet with treatment and get her on the road to recovery.

Of all things we could be facing, this is a bit like salt in a very fresh wound. My father passed away this past June after a long battle with myelodysplastic syndrome (MDS) in which he initially presented with low platelets which the doctors suspected was auto-immune related. It's all a bit fresh, but at least I had a crash course in understanding blood disorders and functions.

I also have a male 9 year old boxer named Ozzy. He had a mast cell tumor removed last week as well. Biopsy results came back today and we got the best possible results (SubQ and low grade with excellent margins). I would like to end 2017 on a happy note and want my Millie feeling better.

« Last Edit: December 07, 2017, 12:20:06 AM by KatColM_O »
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Jo CIMDA

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Re: Hello! New here - we suspect Evans Syndrome
« Reply #1 on: December 07, 2017, 09:47:27 AM »

Hi and welcome

I am sorry Millie has this problem and it is still such a worrying time for you after all you have been through this year.

I have known other dogs from time to time to have low blood platelets that have resolved spontaneously without therapy, immunosuppressive or otherwise.  These dogs have ultimately had to be treated with immunosuppressive therapy for an AI disease, but that still doesn't explain how or why platelets can become very low and increase without treatment.  Here are my considerations:

Has your vet checked Millie's spleen?  The spleen is a site of red cell and platelet destruction and even with immunosuppressive treatment the symptoms do not resolve without removal of the spleen, so perhaps this should be investigated.

In Evan's syndrome, or most other inflammatory AI diseases,  prednisolone is the mainstay if treatment and it acts very fast - within hours.  It takes 5+ days for the new red blood cells and/or platelets to migrate from the bone marrow to the circulation of the blood once immunosuppressive treatment has started.  Cyclosporin is widely used and can be an effective immunosuppressant but, unfortunately doesn't have the same immediate action as prednisolone so it is wise to start with immunosuppressive doses of prednisolone alongside Cyclosporin and when the Cyclosporin kicks in then you can start to reduce the pred.

I know Millie had a bad reaction to prednisolone but what dose was she prescribed?  The recommended dose for immunosuppressive therapy with prednisolone is 1-2mg/kg/12hours but treatment should always be started at the lowest immunosuppressive dose of 1mg/kg/12hours and this is most important in a larger dog because their metabolism is much slower than smaller dogs and therefore the side effects of the drugs are greater.   Check out the dose Millie was given and if it was higher than the lowest immunosuppressive dose then this could be why she reacted so badly and it might be worth starting her on the correct dose again (especially as she is now on a gastroprotectant) even if it is for a short time until the Cyclosporin takes effect (about 10 days I think). There are some side effects of prednisolone treatment that have to be accepted but these reduce as the preds are lowered.

The other option is to give Millie a blood transfusion or an IGIV transfusion.  This will buy time for the drugs to start to work and hopefully take her out of the immediate danger of not having any blood platelets or very low red blood cells.

One dog I knew who had a similar problem to Millie was eventually diagnosed with primary Addison's disease.  This should be considered in Millie's case especially as she has kidney involvement. When a case is not 'classic' like Millie's then you have to consider that there may an underlying cause such as Addison's disease or spleen destruction of the red blood cells and platelets. The important thing is to prevent her going into a crisis by giving support in the form of blood transfusion etc, until a firm diagnosis and primary cause of the problem is made.

I hope you can stabilise her very soon.

Jo


PS.  This is the best immunosuppressive drug protocol I have come across and it can be confidently used as a guide.

Example: Reduction Protocol for prednisolone:

Clinical Immunology of the Dog & Cat , 2nd Edition,  by Michael J Day
Professor Michael DayBSc, BVMS(Hons), PhD, DSc, DiplECVP, FASM, FRCPath, FRCVS 

Professor of Veterinary Pathology, University of Bristol, UK and WSAVA - Chairman of Scientific Advisory Committee.
This example is based on a dog receiving an induction dose of 1.0mg/kg/q 12hrs (q = every)
Dose                           Duration (based on clinical effect)
1.0mg/kg/q 12h                          10-28 days
0.75mg/kg/q 12h                       10-28 days
0.5mg/kg/q 12h                         10-28 days
0.25mg/kg/q 12h                        10-28 days
0.25mg/kg/q 24h                        10-28 days
0.25-0.5mg/kg EOD                    at least 21 days
0.25-0.5 mg/kg every third day      at least 21 days

Every reduction is made after consideration to improvement of clinical signs, blood results and side effects of the drugs.

 
"Doses above 2.2mg/kg/day do not give more immunosuppression but do cause more side effects. Many internists believe that prednisolone doses should not exceed 80mg per day, regardless of the dog's weight."  Plumb's Veterinary Drug Handbook Eight Edition.








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KatColM_O

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Re: Hello! New here - we suspect Evans Syndrome
« Reply #2 on: December 08, 2017, 04:21:12 AM »

Hi Jo, Thank you for the information. I will discuss the prednisone dosing schedule below. She had been given 20mg 2x/day - she hovers between 45-50lbs so that's right on for the lowest dosing of 1mg/kg every 12 hrs. She threw up in the middle of the night of day 2 on the med (normal looking throw up for her), but another day after and she was vomiting was looked at first like diarrhea (I came home to a messy house and didn't know what had happened...then I read about vomit from ulcers looking like coffee grounds and it made sense).

She had blood work done again today and started her first dose of the cyclosporine this evening. We have a follow-up for blood work scheduled for Monday. I got copies of all of her records while I was at the vets office today so I am going to review and can post more specifics on the different blood counts.

I haven't considered Addison's disease, but will read up on it and see if anything stands out in the records I have. The vet tech also sent me a link to the ultrasounds and other diagnostic imaging that has been done in the last year. I am not sure I'll be able to tell much from that, but I will be curious to take a look. My sister's fiance is a radiologist so I may ask for his expert opinion...even though I know humans and dogs are very different.

I was able to get Millie to eagerly eat her breakfast this morning by adding a bit of olive oil to it and she seemed to be in better spirits when I got home from work. I'll try to continue to encourage eating and hopefully we will get some answers soon.
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Jo CIMDA

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Re: Hello! New here - we suspect Evans Syndrome
« Reply #3 on: December 08, 2017, 03:37:37 PM »

Hi

Omeprazole might be  a better gastroprotectant and it will heal an ulcer. You are very lucky to have a radiologist in the family.

Cyclosporin can sometimes upset the tummy at first and if this happens  lower the dose for a few days to customise her to the new drug.

Good luck
Jo
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