Possible indolent lymphoma? Help please

[Veronicahazel2]

I'm gonna sumerize this as briefly as possible and I'm going to say on front end I have been to several drs including oncologist who are following me aggressively Bc of possible cancer. I'm not one of those ppl who feel a lump are just in panic mode posting. Here goes... I'm 26 female I had a baby 18 months ago I had a rough pregnancy and during second half was discovered I have severe anemia so bad I was hospitalized and nothing seemed to help it. During second half of pregnancy I started to develop horrible drenchung night sweats which I attributed to pregnancy and a couple swollen nodes. I just let it go then after  my daughter was born I felt like I got hit by a bus so tired 24/7 I ached everywhere the night sweats just got worse terrible pain in left side more swollen nodes. My gyn said it was hormones going back to normal. Two months later I go to gp she did cbc severely anemic still said that was it and made me feel nuts. Everything just kept getting worse so I got a second opinion now 4 months post delivery, internalist said my side hurt Bc my speen was swollen she did full work up and I did 7 rounds antibiotics total even though I had no obvious infection. I was tested for every auto disease and infection al neg. I have high blood protein but 6-8 months with several nodes still big she sent for biopsy fna ( I know bad test) said they didn't get enough tissue to be sure but looked like excessive Lambda ultra sound said of the one they took it from (really two were matted there 1.3 sm each in posterior cervical area) hypoechoic rim but hyperchoic helium. Pathology suggested follow up so my dr sent me to hemo onc he said the area they are in is worry some they re did cts and blood did a iron transfusion. The dr said he suspected indolent lymphoma and did bond marrow asp which was good but showed difference in size of rbc. He said my igm protein was high  but not myloma high but def abnormal and I had granulcytopenia in flow test. He said every 4 months he follows and scans me fast forward year later to now still have same swollen nodes night sweats fatigue side pain and now bladder fistula (prob unrelated) I had full gi work up nothing but strange inflamation in ilieum but no gi disease (had geno test). Had my scan last week my spleen has grown 1 cm from last scan now 14.5 (u can see from looking at my stomach) however so stones it gets even bigger (my dr has seen it) but always palpable and swollen. And scan said "again several stable shotty nodes seen in nevk region" and chest said stable but increased lobulated density of anterior superior mediastinum and two 2.1 cm low density ovoid structures in pelvic wall needs ultra sound to rule out necrotic nodes. Spleen more enlarged from last scan. My internalist has ordered full blood work up again Bc she said this is not normal and could be indolent lymphoma. Esp given spleen issues and location of nodes. Anyone with similar experiences or know what to make of this? Please ? Also on my onc paper work under diagnosis I saw term inc. castlemans disease does anyone know anything about castlemans disease?

[Veronicahazel2]

Anyone???

[Ken_PA]

Hi, here is what seems to most tend *against* lymphoma:
you have severe anemia. Red blood cells can be low because of either  
a) abnormally low production of cells
or
b) abnormal destruction of cells

Your bone marrow biopsy showed no cancer cells in the bone marrow. (Lymphoma can cause anemia when the lymphoma cells invade the marrow and crowd out the good cells that produce red blood cells.)  So it seems that something is destroying your red blood cells - possibly some odd immune system condition.

Did you have a 'blood smear' done? They can get clues if any red blood cells are oddly shaped,

Your sonogram seems to be inconclusive.

So it sure seems that you should be having an excisional biopsy. Once they can directly examine cells inside a node, they can rule out lymphoma and also get clues as to what is causing the problems. The biopsy should be able to diagnose Castleman's disease, and the pathologist has to deliberately look for it.

Whatever you have is unusual.

[Ken_PA]

Castleman's Disease centers around the immune system going haywire and secreting lots of the chemicals (called cytokines) that get the immune system overly active. It is *not* cancer.

I can help you look into this, if you like.


You do seem to possibly match up with the 'Multicentric" type of Castleman's.
http://www.castlemans.org/about

which includes autoimmune hemolytic anemia and enlarged spleen.

So I'd put on hold the excisional biopsy just for the moment, and instead demand an immediate blood test for the cytokine called IL-6. If your IL-6 is high, that points to (but doesn't prove) Castleman's.

Have you had the blood tests called ESR and CRP already?

You can possibly also ask for your FNA biopsy sample to be tested for the virus called HHV-8.

[Ken_PA]

"autoimmune hemolytic anemia"

that means a person's own immune system is destroying their red blood cells

[Veronicahazel2]

Thank you for ur input! I have seen from other post you seem to be very knowledgable. I saw a surgeon a year ago Bc my onc suggested excision biopsy but the surgeon (only does oncology of neck head and chest) said he felt we should just watch Bc biggest lymphnode was only 1.6 cm and I would have a visible nasty scar and I'm young. However last week my pcp said it's time for biopsy now so I have apt in 2 weeks to see surgeon again. I believe they did blood smear with flow cytometry test last year and all it showed was granulcytopenia 77% lymphocytes. Which wasn't consistent with cbc, I know my bone marrow aspiration said something about the difference in size of rbc I'm gonna get them to fax me a copy of it. Also I didn't clarify but since iron transfusion I have been anemic per rbc it's always low side of normal range but my mchc is low and rdw high. As far crp it goes up and down last year it was a little high then normal then in oct it was 4.86 (0-.3) then two weeks later down to .6 so idk what that means but I was I'll in oct when it was taken. When I saw pcp last week they took13 tubes blood and she's re ordered every test again. My esr test is back and it's 15(0-20) last time it was 20. The fna showed possible excessive lambda but too little sample to be definite have u heard of this? Also the ultra sound saying hypoechoic rim and hyperchoic helium do you know what this means? Also the ct scan saying stable increased lobulated density of anterior mediastinum under findings it says impression residual disease vs thymic tissue have u heard of this? I just emailed my pcp about testing for castlemans awaiting her reply now. Also so etime when I'm very tired my legs give our and I fall my dr though maybe neurological or maybe just weakness but she's doing lyme test at my request but doesn't seem to think that's it. Again thank u for ur help

[Veronicahazel2]

Since transfusion I haven't **** been anenic

[Veronicahazel2]

Bone marrow asp said normocytic with mild aniso and poikilcytosis under blood smear portion

[Veronicahazel2]

Also they sent me copy of fna report which says lymphocytes 21% : bcells 1-2 % very few detected possible excessive lambda but too few for conclusive light chain assessment and no definite light chain restrictions based on detected events.

Granulocites 13%
Monocytes 2%
Cd45 neg events debris 64% no significant reactivity within markers tested.
Under comments it says the sample cellularity is VERY low. Correlation with clinical, laboratory and mirphologla data is reccomended.

[Ken_PA]

Okay, lots going on here... I am guessing that you have Multicentric (in more than one spot) Castleman's. Not the one-spot kind.

I don't know a lot about Castleman's. It is rare, so not many docs know a lot about it.

There is the subtype that has the HHV-8 virus in the nodes. The virus apparently stirs up a lot of the inflammatory signalling chemical (cytokine) called IL-6. The IL-6 flareups stir up a whole lot of inflammation, but then there can also be calmer periods.

Then there is the subtype that has no HHV-8 virus involved. The cause of that one is a mystery.


The inflammation in the node is like a battle inside - so then there is a lot of resulting 'debris' there. A lot of debris leaves little room for actual live cells - that's 'low cellularity'.

Castleman's also results in 'proliferation' (multiplying) of immune cells called plasma cells. Plasma cells normally make 'globulins' to fight infection. (A globulin looks like a tiny Y-shape think that sticks to bacteria, e.g. and marks them for destruction.) But in your case, things are running amuck and globulins are being produced willy-nilly -- that's all associated with terms like 'lambda' and 'light chain' and "IgM protein".

"mild aniso and poikilcytosis under blood smear portion" Anisocytosis means the sizes of your RBCs varies too much; poikolocytes means odd shaped RBCs; this is all part of your anemia. Castleman's can probably cause that.


I'd tell the surgeon that since Castleman's has some chance of turning into something worse, then you need the biopsy ASAP. They will likely discover that you do not have lymphoma and then diagnose you with Castleman's. That's probably the best result to want here. Then you probably take steroid pills as treatment to suppress the inflammation.

That's a lot of terms to wade through. But if you make the effort to learn them, then your talks with doctors will go much better :)

[Veronicahazel2]

Ok thanks! I'm gonna see a new hematologist too Bc this guy is too non aggressive and my pcp agrees. Do you know anything about  the mediastinum thing from ct scan? My pcp said she was confused why hemo oncologist hasn't scheduled a biopsy but she would look into it, she said sometime thymus is still seen in adults but shrinks until 30 where it's undetectable but mine has increased in density over passed year but not size could that be anything to do with lymphoma or castlemans? Aside from possible castlemans do u think it could just be slow grade lymphoma? My pcp said a spleen that's swollen over a year is a sign of disease Bc there is no infection and according to my onc he said he had never seen such a thorough work up before

[Ken_PA]

"see a new hematologist "
good :)

"Do you know anything about  the mediastinum thing from ct scan?"
I'm guessing that's part of what makes this the Multicenter type of Castleman's - it's not just in one place. The spleen is another place where you have it. Also maybe the "two 2.1 cm low density ovoid structures in pelvic wall".

But one thing is that the type of immune cells in the thymus are a different but similar kind.  The thymus has T-cells, while the lymph nodes have B-cells (and the plasma cells that develop from B-cells). Still, Castleman's, can invole T-cells, too -- while a B-cell lymphoma won't involve T-cells.

"Aside from possible castlemans do u think it could just be slow grade lymphoma?"
Yes, but that's the less likely possibility. Remember that they didn't find any cancer cells in your FNA or in your bone marrow biopsy.

"a spleen that's swollen over a year is a sign of disease"
A doctor only cares about what are called "authoritative sources". Let's find one for a swollen spleen and Castleman's...

Here we go, you can print this and take it to any doctor, it's just the first one I found, from cancer.org:
http://www.cancer.org/acs/groups/cid/documents/webcontent/003093-pdf.pdf
Here is a quote:
"Multicentric CD can also affect lymphoid tissue of internal organs, causing the liver, spleen, or other organs to enlarge."

They also say:
In addition, people with either type of CD can have other symptoms (although these symptoms are much more common in people with multicentric CD:
• Fever
• Night sweats (that soak the sheets)
• Weight loss
• Loss of appetite
• Weakness
• Fatigue (tiredness)
• Shortness of breath
• Nausea and vomiting
• Nerve damage that leads to numbness and weakness (neuropathy)
• Leg swelling (edema)
• Skin rashes
Some of these symptoms might come and go over time.

[Veronicahazel2]

I agree this does seemto fit castlemans more! I'm hoping they will further investigate this! I just can't stand watching and waiting mean while my quality of life is horrible Bc I feel aweful all the time.

[Veronicahazel2]

Also do you know what to make of "again seen are sever stable shotty nodes" under head portion of ct, also I was gonna ask the three biggest nodes I have are all in posterior cervical area and two are matted I guess Bc there connected and long and thin feeling is there any significance to that? And last. Question I have read u say stuff about helium on here do u know what hypoechoic rim and hyperchoic helium mean? Thanks in advance sry for all the questions!

[Veronicahazel2]

Oh and also I read most ppl with castlemans have positive ana I have a negative one but I know every case is individual

[Ken_PA]

"shotty nodes" (a group of small ones like buckshot) can be from various causes

"matted" (attached together) is usually a tendency to lymphoma but in this case I think that the highly inflammatory Castleman's can also create the fibrous tissue ot connect nodes together

I've seen sono reports and journal articles mention the *presence* of the hilum (which is a very good sign). I'm guessing that hyperechoic means the same thing. As for the hypoechoic rim? I don't know. I would guess that once a cancer has grown past the boundary of the nod, then the rim would not be seen anymore.

ANA? I don't know.

Sorry for too many questions? No, not at all  :)  But I hope you make sure to tell how everything works out for you.

[Veronicahazel2]

Oh I most def will I hate when I read these post but then u never get the outcome! Do you mind me asking
What kind of lymphoma you have or had? Is your story posted on here?

[Ken_PA]

My father had Mantle Cell lymphoma. I took care of him for two years and had acquired some knowledge. So one day I stopped here and decided to reply to some questions. That was six years ago - time flew.

Btw, your "strange inflammation in ilieum" is probably also Castleman's. The whole digestive tract has what is called lymphoid tissue -- and the cells we are talking about (plasma cells and B-cells and T-cells) are what make up lymphoid tissue. They are lymphocytes.

If you get the IL-6 blood test (I hope you do ASAP), you can ask that a copy of the results gets mailed directly to you by the lab. That way you don't have to wait to know what it says. The waiting can be terrible, right?

[Veronicahazel2]

Update.. So got all my blood test back negative for any inflammatory markers, negative for lyme, no sign of infection, c reactive was >.1 (very low) ana negative, which pretty much rules out castlemans or anything auto immune my dr says. I have capsule endoscopy today to check small intestines for lymphoma or abnormalities. Spleen is more swollen today (very sore and hard to sit up right) so I guess were back to lymphoma.  My cbc was norm besides low mchc. I'm seeing a surgeon Monday on advice of obgyn they are most likely going to laporscopically operate to see what going on with pelvic masses and find origin of bladder fistula. Do you know any other cancer besides lymphoma to cause enlarged nodes and spleen (spleen been slowing growing for 18 straight months)

[Veronicahazel2]

Also you said shotty nodes could be several causes, what are they? I can't really find anything online about that

[Ken_PA]

"...which pretty much rules out castlemans or anything auto immune my dr says."

I don't know that I'd be satisfied with that. E.g.: "The clinical manifestations of MCD are heterogeneous..."
http://www.jhoonline.org/content/2/1/19

which means that *maybe* a patient with Castleman's can sometimes have a low CRP.  That's just a guess.


(I'll be back later.)  

[Veronicahazel2]

So this may seem off topic but it's not my daughter has been really sickly on and off since birth I stay home with her but she gets sick a lot way more then normal baby her pediatrician says, she gets waves of crazy high fevers too like 104-105 and we were in hospital with her over weekend saw her dr today who wants her tested for cyclic neutropenia. I read up Bc I knew I had read that phrase somewhere and it clicked my flow cytometry test showed sever granulcytopenia but healthy cells then upon retest it was normal but my last three blood draws all exactly three weeks apart showed neutrophil percent low and lymphocyte percent high then my dr just rechecked Bc lym was high (8 days after last test) and it was high end of normal so let it go. But this disease makes sense I read in severe cases can cause spleen and lymphnodes to swell and causes ulcers in gi tract and mouth mimicking crohns. Aso I read it is most common among Jewish ppl and I'm half Jewish (my moms family is like off the boat Jewish). What do you think?

[Ken_PA]

Bingo, excellent thinking! You know, I always ask people with mystery immune conditions about their family history - but with all your testing to discuss, I didn't get to that yet. Now you must also research all your relatives, both sides - not only for Familial Mediterranean Fever.. which it's not even known yet if that's what she has. Could be a virus, because FMF doesn't cause high lymphocytes AFAIK. A virus can cause high lymphocytes and low neuts.

Also, expect to have trouble convincing some docs. Most just match symptoms and don't do much sleuthing.

So, we need to also figure out when to get you tested at a time when your CRP, ight be high. (You might have noticed that I'd said *sometimes* above, in the phrase "can sometimes have a low CRP".)

Current theory: you have the mystery kind of Castleman's - which might even be unrelated to the other kinds of Castleman's.

[Ken_PA]

You still should want a biopsy. I'm at a loss to understand why they did a capsule endoscopy instead of one via tube so they could have snipped some sample tissue of anything that looked odd.

[Veronicahazel2]

Oh they do capsul endoscopy Bc u can't get to small intestines with reg one or colonoscopy both of which I had 6 months ago. I'm wondering of maybe this cyclic neutropenia isn't the cause of my lymphoma like symptoms I mean I did a lot of research today although it is crazy rare if left untreated into adulthood can mimic lymphoma causing swollen spleen fatigue night sweats fever swollen nodes ulcers of mouth and colon. Now I'm not sure but I do know everytime I have had a bad flare up of my problems I have had high lymphocytes low neutrophils and low blood count  which is inconsistent with infection. I got a calendar and took my blood test and the test with neutropenia fall on 24 day intervals and my daughter is being tested for it Bc dr suspected without knowing anything of my medical issues. I'm still having them investigate castlemans though, and as far as capsule goes I haven't gotten results yet however I do know that it showed gastroperesis which makes sense Bc I puke up I digested food from days earlier so etimes. I'm not sure where this fits in however my mom had gastroperesis but hers was so severe she had a feeding tubs and her stomach removed. Hers was thought to be a result of surgery damaging her vega nerve but now I wonder... Unfortunately she died when I was a kid so I have no way to know specifics. In reference to crp I know when it was high last time I had neutropenia then as we'll..