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Chris' $0.02

Posted Feb 21 2009 11:36pm
D +139

Today's blood work...

WBC 4.6 K/uL
RBC 2.74 M/uL
HGB 8.8 G/DL
PLT 437 K/uL
ANC 2.88 K/uL

No blasts or otherwise weird cells show up in the blood work. Now that that is out of the way let me give everyone the the details of what is happening right now.

MDA is 95% sure that Ann has something called Post-transplantation Lymphoproliferative Disorder or PTLD for short. It is a pre-cancerous condition that develops often because of infection by the Epstein Barr Virus. PTLD presents as a spectrum of problems ranging from Lymphadenopathy (swollen lymph nodes often painless and hard), diarrhea, and cramps. It is primarily detected by swollen masses in the head and neck of unexplained origin. It can cause lymphoid "tumors" in virtually every organ and if it is allowed to persist long enough it can progress into non-Hodgkin's Lymphoma.

So here is what Ann is dealing with right now. She has several swollen lymph nodes on the left hand side of her face. The most predominant is her superior deep jugular lymph node on her left side, which is swollen to about 2 cm and is hard and painless (this is what got biopsied yesterday. In addition there is a mass approximately 4 cm x 5 cm that is occupying her nasopharynx and abutting the base of her skull. The mass appears to be mostly made up of necrotic (dead) lymphoid tissue. These "dead" lymph glands have swollen and squeezed together enough to seal off her left nasal passage completely. In an around this mass is a thick yellow viscous mucus material that is made of up dead cells. Probably dead white blood cells, and the mucus has become impacted because the blocking lymphoid mass is not allowing it to drain properly. In addition to all this there are several Supraclavicular lymph nodes on her left hand side (near the collar bone) which appear to be necrotic as well.

So from all this I have composed a working theory as to what has happened. First let me lay out the time line of events:

On 9/21 Ann started running a fever and also complained of a little tickle in the back of her throat on the right hand side. Ann started Biaxin to combat what ever infection was occurring.

On 9/23 she had a single lymph node present on the left side of her jaw (near the suprajugular area). It was swollen and tender with a diameter of approximately 1 cm.

On 9/26 the lymph node had nearly completely resolved and her fever broke.

On 10/1 Ann reported a sore throat on the left side along with an increasing ear ache on the left hand side of her head.

On 10/3 A follow up with and ENT in Baton Rouge revealed an 1 cm ulcer on the left hand inside of Ann's throat. Additionally her throat was observed to be red. Ann began taking 1000mg of Valtrex.

On 10/4 Ann visited her local hematologist for a routine appointment. Her CBC came back with an elevated WBC count of 9.6 K/uL. Her HGB was 12.2 G/DL and her PLTS were elevated to 569 K/uL. LDH is 204 IU/L BR hematologist theorizes that this a lagging indicator of the new immune system fighting infection. He except it to clear up shortly. Ann has a single incident of sudden vomiting that night.

On 10/6 Ann notes that her appetite is not quite what has been. She has a incident of nausea after eating. She claims it is because of sinus drainage.

On 10/7 Ann starts to run another low grade fever which resolves itself. She again has another episode of nausea and vomiting after eating.

On 10/8 Ann is uninterested in eating and has several episodes of diarrhea along with persistent nausea. Late night she begins to run a fever which exceeds 100.5 F.

On 10/9 I returned her to MDA suspecting GVHD of the gut. At MDA her 1st CBC comes back with WBC of 4.4 K/uL , HGB 8.9 G/DL and PLTs of 342 K/uL. LDH was 431 IU/L.

OK so there is the time line. I suspect ( and remember kids, I'm a Mechanical Engineer and not a Doctor) that Ann picked up one, perhaps two infections around 9/20. The first may have been bacterial which was cleared with antibiotics. The second was an opportunistic viral infection. In theory that would explain the soreness of her throat first on the right hand and then on the left hand sides.

The viral infection attacked the left hand back of her throat and eventually entered her lymphatic system. The virus was probably EBV (although the tests are still not back on it yet), and in the process of it trying to replicate it mutated a white blood cell (B-Cell) in one of Ann's lymph nodes in her nasopharynx. One node drained into another, the malignancy spread and the nodes began to swell.

Eventually this put pressure on her ear canal and caused the ear ache she felt on 10/1. That persisted until the nodes drained releasing some internal pressure but spreading the contamination to a lower level of lymph nodes in her head and neck. The nasopharynx lymph nodes continued to swell and eventually blocked off the left hand sinus passage around this time.

Her new immune system may have mobilized to fight the viral infection as evidenced by the elevated WBC on 10/4. The WBC's produced at this time probably make up some of the very impacted mucosal material that is built up on top of the nasopharynx mass blockage in her left sinus. However, the critical damage was probably already inflicted by this point. A PTLD B-cell mutant had been cloning itself inside these inflamed impacted lymph nodes for approximately 14 days.

While I have no proof that the PTLD has not spread past Ann's lymph nodes to a more distant region of her body, I am hopeful. Principally because of the LDH levels that were recorded at Baton Rouge and the current ones at MDA. Neither level is elevated beyond what is the normal acceptable range. So I hope that the nausea and diarrhea is the product of necrotic drainage from the swollen lymph nodes and not tumor growths on her gut. Although I will admit that I have learned that PTLD can and sometime does manifest like that. We have a CT scan of Ann's abdomen tomorrow which should clear this up.

The hemoglobin and RBC drop in Ann's blood counts could be from the constant diarrhea she has been having. It is also a possible, but not very likely side effect of EBV running free in her blood stream. I am encouraged that her platelets and WBCs remain stable because I believe it tends to indicate that the problem has not affected her new baby bone marrow. If the PTLD was given time to advance to full lymphoma it undoubtedly would.

So in conclusion I think we are looking at a EBV viral infection that caused PTLD. Which in turn is limited to the head and neck. Low LDH numbers and Ann's marrow not being effected tend to support the assumption that this is an recent development and has not had more than 25 days to develop. Translation - I think we caught it very early and can treat it quickly.

So that's my theory...just remember you go to an ME to design turbines not for medical advice.

So what is MDA going to do about it? They answered that this morning. Rituximab (aka Rituxan) is the weapon of choice in fighting PTLD and it they are pretty confident that it will work. I understand the experience of getting it is similar to the Rabbit ATG that Ann got just before her transplant.

If thats the case then we are in for fever, chills, nausea, vomiting, rashes...fun stuff. They start the first infusion just as soon as the pharmacy delivers it to the BMT floor.

Wish us luck.


**Update**

Dr. Kebriaei just stopped by and has pretty roughly critiqued my theory. Turns out MDA is not 95% sure but more like 60%.

She still believes that the necrotic tissue and "abnormal cells" in Ann's lymph nodes could still be just a common infection. MDA's pathologists are working to get slides made and stained so that they can work out exactly what those necrotic cells are. Which as it turns out could be anything from simple dead cells (puss) to a rapidly dividing lymphoma. She still thinks that Ann's tummy troubles could be GVHD and will not rule out the possibility until the endoscope samples are back (still not yet). They are still going ahead with the Rituximab however, just to be on the safe side and because its not too risky.

Serves me right for trying to get ahead of the experts.

**Update II**

The Rituximab has arrived on the floor. Here goes nothing.
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