AA again after 30 years!

[Sara J]

Hi everyone,
My husband Rich has now been diagnosed with AA for the second time. Here’s the story so far…

In April 1979, Rich (then aged 13) started to develop bruises and petechiae. He was diagnosed with AA in June 1979. His blood levels were Hb 6.7, WBC 3.0, plt 10, neutrophils 27%. At the time, the outlook was not good as there was no match for bone marrow in the family.

Fortunately, by August 1979, he started making a spontaneous recovery with no treatment other than a blood transfusion. He continued making regular visits to the oncology clinic for blood tests and his results continued to improve. By January 1984, his results were Hb 16.2, WBC 4.9, plt 114, neutrophils 22%. In 1985, Rich moved away to university and stopped visiting the clinic. He assumed he was ‘cured’ and thought no more about it.

In January 2011, Rich visited his GP, complaining of feeling run down after various cold and flu like symptoms. Fortunately, his GP looked up his previous records and spotted the AA diagnosis. After referrals to various hospital departments for ultrasound, gastroscopy, CT scan and finally a bone marrow biopsy, he was diagnosed with AA again, in April 2011. Blood results this time were Hb 13.1, WBC 3.9, neutrophils 0.8 and plt 81. He was due to have ATG treatment in July. Again, however, his blood levels started to get better on their own.

At the moment, he is having blood tests in the hospital haematology department every 3 months. His blood levels are good enough not to need treatment (thank goodness ) but his fatigue has meant a long period off work.

After lots of research, I now feel I know a lot more about AA. I’m so pleased to have found the AAMDSIF website and marrowforum. Thanks to everyone who contributes. It’s good to feel you are not alone trying to understand such a rare condition.

It would be great to hear from anyone else who is in the ‘watch and wait’ group.

Thanks for listening,

Sara

[Hopeful]

Hi Sara,

I am sorry to read that your husband has to deal with this again. However, I'd be extremely cautious with his doctor. Many people with AA would dream of having your husband's counts month/years after ATG treatment. It is not standard protocol to consider ATG with counts as you listed.

AA is diagnosed by exclusion. So, it is important to do a full workup on your husband to see what else could be causing his bone marrow suppression. This should include tests for vitamin and mineral deficiencies (folate, B12, iron, etc), as well as viruses (CMV, EBV, HIV, parvovirus, hepatitis, etc.) , lupus, and heavy metal toxicities, among other things. If these tests have not been done on your husband, than his doctor probably isn't an expert in AA, and you should seek out someone who is while your husband is stable.

I wish you both the best of luck!

[Sara J]

Hi Hopeful,

Rich was tested for viruses and vitamin/mineral deficiencies before being given his AA diagnosis. in fact, I remember his haematologist saying exactly what you said, that it was a disease of exclusion. He hasn't been tested for lupus though, definitely worth investigating further.

Thanks for the good advice,

Sara

[evansmom]

I totally agree with the idea of ruling out Lupus. That is a disease that can come and go, and sometimes the only measurable symptom is pancytopenia.

[mscrzy1]

Yes, to getting tested for lupus. I had SAA over 15 years ago. They had done the ATG treatment of which I didn't have a response. I was transfusion dependant while we figured out our next form of treatment when I started to recover on my own. Now, I'm having a decline in counts again. They are not near the severity of decline I had seen originally and I have a rheumatologist on my team of hematologists now. You can read my story to get the full story, but they "think" I'm actually dealing with lupus. Lupus is difficult to diagnose. You can test negative on the ANA testing and still have lupus. I'm currently taking Plaquinil and seeing my counts gradually come back up. My hematologist is starting to agree with my rhuematologist especially after seeing my counts on the rise and finding out that the ATG did nothing for me, but I started to turn around on my own. He had said that is typical for autoimmune diseases. Said he has had patients with ITP in the hospital where they have done everything they thought they could for them with no improvement then suddenly out of nowhere the patients start to improve on their own with no treatment. After reading that your husband had recovered on his own with no treatment, I would seriously look into lupus. Good luck!

PS: I would be sure to get a rhuematologist to test for lupus. My hematologist tested me for it and I was negative. My rhuematologist said that the tests are very generic through regular labs and aren't as accurate. My rhuematologist tested me and I was ANA positive, but the rest of the blood indicators were negative. She was still unwilling to rule lupus out. I have no other indicators, except raynaud's. As I said...very difficult to diagnose, but good to have a rhuematologist check it out. They are more versed in lupus and what to look for to diagnose it.

[Sara J]

Hi Nicole,

I read Evan's story. I'm so glad he made a full recovery. His experience was so much better than my husband's first time round. He was the same age as Evan and it was really frightening because nobody really knew much about AA then. An unmatched BMT was considered too dangerous and steroids were the only option available. Fortunately, so much more can be done now and the outlook is so much better. Meeting Evan's donor must have been an incredible experience.

All the best for the future

Sara J

[Sara J]

Hi Angie,

There certainly seem to be similarities with Rich's history, especially the spontaneous recovery. I've been doing some research on Lupus and the vast majority of cases are seen in women. It's definitely something to ask about at his next appointment in March. If test results come back positive, that would make him even more of a rare case than he is already Will let you know how he gets on.

I'm really glad to hear your results are improving.

Sara x

[Sara J]

Just a quick update. Rich went for a check up 3 weeks ago. Apparently, he had already been checked for Lupus and thyroid deficiency but as it was 12 months ago, he's been tested again. He is now on prednisolone as a thereputic trial to see if it raises his blood counts. Although he felt nauseous and a bit wobbly to start with, it's gradually getting better. He has another visit to the consultant next week to see how it's going.

Also good news, he is going back to work, initially 2 days a week. Things are looking up

[mscrzy1]

Sara,

I'm so glad to hear that he is gradually getting better and going back to work! That's fantastic news! I'm also glad to hear that they are retesting some of his bloodwork so they can determine exactly why he saw this drop in counts again. Keep us updated!

[Sara J]

More good news. The latest blood results show neutrophil count of 2.0 and WBC 4.0. The prednisolone seems to be working Lupus and thyroid tests both came back negative. The diagnosis is most likely to be mild AA that is steroid responsive.

The next step is to taper off during the next 4 weeks and see what happens.

Hope you are responding well to treatment too, Angie.

[glitterandlace]

Originally Posted by Sara J

Hi everyone,
My husband Rich has now been diagnosed with AA for the second time. Here’s the story so far…

In April 1979, Rich (then aged 13) started to develop bruises and petechiae. He was diagnosed with AA in June 1979. His blood levels were Hb 6.7, WBC 3.0, plt 10, neutrophils 27%. At the time, the outlook was not good as there was no match for bone marrow in the family.

Fortunately, by August 1979, he started making a spontaneous recovery with no treatment other than a blood transfusion. He continued making regular visits to the oncology clinic for blood tests and his results continued to improve. By January 1984, his results were Hb 16.2, WBC 4.9, plt 114, neutrophils 22%. In 1985, Rich moved away to university and stopped visiting the clinic. He assumed he was ‘cured’ and thought no more about it.

In January 2011, Rich visited his GP, complaining of feeling run down after various cold and flu like symptoms. Fortunately, his GP looked up his previous records and spotted the AA diagnosis. After referrals to various hospital departments for ultrasound, gastroscopy, CT scan and finally a bone marrow biopsy, he was diagnosed with AA again, in April 2011. Blood results this time were Hb 13.1, WBC 3.9, neutrophils 0.8 and plt 81. He was due to have ATG treatment in July. Again, however, his blood levels started to get better on their own.

At the moment, he is having blood tests in the hospital haematology department every 3 months. His blood levels are good enough not to need treatment (thank goodness ) but his fatigue has meant a long period off work.

After lots of research, I now feel I know a lot more about AA. I’m so pleased to have found the AAMDSIF website and marrowforum. Thanks to everyone who contributes. It’s good to feel you are not alone trying to understand such a rare condition.

It would be great to hear from anyone else who is in the ‘watch and wait’ group.

Thanks for listening,

Sara

His levels the second time weren't that bad honestly..... My opinion not low enough to take the chemo. Hemoglobin normal, WBC just below normal, and platelets yes they were low,, but not super low,,,, Glad he is recovering and best of luck to you.