Medical Update on Joe's CLL

So much has happened the last two days that I am going to break it down into two different blogs. This one will concentrate on the medical aspects.

I had e-mailed Dr V and his clinical nurse on Monday as to the INCYTE update. Cathy e-mailed me back that once she heard from INCYTE that she would contact us and that she had sent another e-mail to INCYTE on Monday afternoon which I am sure was from my follow up. Lesson: KEEP BUGGING THEM!

Last evening I went back on mymdanderson.com to see if anything new was written up and there was for the two days we were at MDACC last week.

Dr Michael Keating, CLL expert at MDACC who we saw first,  wrote on Joe's Patient Reports the following:

" I personally conducted the history and physical examination of Mr. Evans, together with Dr Xavier Badoux, on 09/21/10. The patient has evidence of an autoimmune myeloproliferative neoplasm, but also chronic lymphocytic leukemia. He has a past history of polycythemia and has increased megakaryocyte mass and collagen fibrosis. The rapid rising count is a cause for concerns. We will evaluate and discuss with his primary physician, Dr Bistodek (sic), what the most appropriate approach might be."

Later on that same day we met with Dr Verstovsek (V) which was dictated by the nonchalant Dr Eric Sung Yung Kim. It is a full page but I will write the most important parts of that report:

DIAGNOSIS: Chronic lymphocyctic leukemia and myelofibrosis.

IMPRESSION: This is a 67-year old gentleman with post-polycythemia vera myelofibrosis and chronic lymphocyctic leukemia, currently on treatment with INCB 50 mg b.i.d , doing well. (Please note that the 50 mg is wrong and Joe only takes 30 mg daily and I e-mailed them the error).

PLAN: The patient is doing very well in the myelofibrosis standpoint. The patient has seen Dr. Keating to address his chronic lymphocytic leukemia and he will go back to see him tomorrow to discuss treatment.
All their questions answered to satisfaction and we are in agreement.

Dr V adds a small subscript:

HISTORY OF PRESENT ILLNESS: This is a patient with myelobifrosis, on INCB. This is a JAK2 inhibitor. He is here for follow up. He is seen by my fellow. Please see his note for details.
PLAN: We will continue therapy. The spleen is 19cm and also 12 cm from the umbillicus to the right.

On that same day, 9/21, Dr Keating and Dr Badoux wrote more detail on the Patient Report but was more thorough and more accurate than Dr V and Dr Kim. I found this report more interesting perhaps you will too:

REASON for APPOINTMENT: Consultation for chronic lynmphocytic leukemia in the setting of a myeloproliferative disorder.

BRIEF HISTORY: This is a 67 year old male from Stone Mountain, Georgia who has a 13-year (really 11 years) history of polycythemia rubra vera. He was initially treated with phlebotomy and interferon alpha but because of the flu-like symptoms, the patient continued on Hydrea for the next 10 years. In 8/2008 (really June 2008), the patient developed significant constitutional symptoms including night sweats, fatigue, loss of weight, and poor energy. It was also noted that his white blood cell count was elevated and he was investigated with a bone marrow biopsy, which confirmed the diagnosis of pos-polycythemia myelofibrosis. The patient was referred to MD Anderson Cancer Center for further management of his myelofibrosis and he was placed on JAK-2 inhibitor Incyte drug INCB and he is currently on 15 mg p o twice daily. Since 5/21/2010, the patient has had a progressive rapidly and his current account above 100,000. At about that time, he was noticed to have also a lymphocytic infiltrate in the marrow with a bone marrow biopsy (this is wrong, the BMB was in February 2010 not May 2010), his immunohistochemistry consistent with CD5 positive B cells. Interestingly, his bone marrow prior to that period of time did not identify any lymphocytic abnormalities. In the last year or so, the patient has had recurrent respiratory tract infections that have been prolonged. He has had  a couple of episodes of shingles above the left eye and recurrent herpes simplex viral infection of the lip. The patient has some mild night sweats (not mild in our opinion), although most of his systemic symptoms have improved significantly on JAK2 inhibitor. He has not noted any significant lympadenopathy, although he did have a prominent left inguinal lesion that flared up a number of months ago. He does have some easy bruising and has large ecchymosis over his legs, but no active bleeding or petechiae. He does have progressive splenomegaly and abdominal distention.

REVIEW OF SYSTEMS: There are no other abnormalities on 12-point systems review.

PHYSICAL EXAMINATION:
GENERAL: This is a middle aged gentleman (THANK YOU). He is in no acute distress. He is alert and oriented x3, euvolemic and well nourished.
VITAL SIGNS: Temperature 35.8C, pulse rate 92, respiratory rate 20, blood pressure 119/65, weight 84.2 kg
HEENT: The neck is supple. Mucous membranes are most. There are no mucosal lesions.
LYMPHATICS: There is so significant lymphodenopathy in the cervical, supraclavicular, axillary, or inguinal areas. The patient does have some some palpable lymph nodes in the neck and inguinal areas. However. these are less than 1 cm in maximum diameter.
RESPIRATORY: The chest is clear to auscultation.

ABDOMEN: The abdomen is moderately distended. This is massive splenomegaly palpable 20 cm below the coastal margin and 10 cm across from the midline. The liver edge is just palpable below the coastal margin. There are no obvious masses palpable otherwise.

LABORATORY DATA: CBC, white blood count 137,3, hemoglobin 9.6 g/dL, platelet count 77,000, ANC 8.2, ALC 123.6, Chemistry, BUN 31 mg/dLm creatinine 1.2 mg/dL, LDH 849 International Units per liter, GGT 119.

ASSESSMENT AND PLAN:
1. POst-polysythemia myelofibrosis. The patient is currently on Incyte JAK2 inhibitor 15 mg p o twice daily. He appears to have had good symptomatic response to this medication, although his spleen remains enlarged. It is difficult to know whether whether this is predominately due to the chronic lymphocytic leukemia or the myelofibrosis. However, his disease appeared to be reasonably well controlled until the recent progression of his B-cell lymphoproliferative disorder.
2. B-cell lymphoproliferative disorder. This appears to be chronic lymphocytic leukemia. We well confirm this further with flow cytometry. We will also perform some prognostic markers for further information and planning of treatment. The patient may have a predominately splenic version of chronic lymphocytic leukemia, whichg may partly explain his splenomegaly and this may respond to therapy. The patient will return to see us to discuss results of investigations and treatment planning. We will communicate further with Dr. Verstovsek once we have further information.
3. Moderate anemia. The patient is mildly symptomatic with fatigue and some shortness of breath. We will preform hemolytic screen to exclude that possibility and look for alternative cause of anemia including vitamin B12 and folate in view of the high MCV.
4. Thrombocytopenia. This is likely related to hypersplenism. The patient has no indication of therapy at this point. We will continue to monitor.

Dr Michael Keating has personally interviewed and examined the  patient and formulated the above plan of care.

Michael Keating MD dictated by Dr Xaviwe Badoux

SECOND DAY VISIT with Dr KEATING and DR BADOUX at MDACC on 9/22/2010

LABORATORY DATA: CBC (I am only listing the additional data)  gamma CT 119 units per liter, Beta 2-microglobulin 7.8 mg per liter. Vitamin B12, folate and iron are normal.

ASSESSMENT AND PLAN:
1. Chronic Lymphocytic Leukemia. The patient requires therapy. He has massive splenomegaly, which may partly related to his CLL as well as progressive lymphocytosis and a recent drop in hemoglobin. We suggest he should start FCR chemotherapy with some dose reduction. We will discuss this further with the patient. We recommend to use fludarabine 15-20 mg/m2 and cyclophosphamide 150-200 mg/m2 with rituximab as usual dose. We will discuss further with Dr. Verstovek for coordination of therapy. The patient will return to see us for therapy in early October.
2. Myelobibrosis. The patient is on JAK2 inhibitor INCYTE. We will need to communicate with the trial coordinator to determine when and how the patient should come off therapy or whether they will have a dispensation to continue.
3. Seizure disorder. The patient will need to continue following up with his neurologist for management of the seizure disorder.
4. Skin Cancers. In view of the chemotherapy and known history of CLL, it is important for the patient to have continued surveillance for skin cancers

____________________________________________________________________________
My viewpoint:
Dr. V has kept the myelofibrosis under control and Joe has responded well to INCYTE. I still feel that Dr V should have personally informed us on what the BMB in February 2010 stated and I believe that there should had been a closer look at the new BMB diagnosis stating:

CD5+CD20+ Aberrant B Cell Infiltrate, consistent with involvement by Lymphoma
.
CD5+ B-Cell Lymphoma/Leukemia in Peripheral Blood by Flow Cytometric Analysis

When I questioned Dr V personally and my alarm, he told me not to worry about it and those words just scare people and continue what we were doing. I am sure that he felt like many physicians that CLL is slow growing and it was not a concern and that Joe was doing well on the INCYTE is controlling his MF symptoms. I still find it strange that I am aware of the fast growing wbc and lymphocytes results on his regular local CBC.  I also find it strange that I was not notified of his last BMB on July 2010 that now his disease is diagnosed specifically as MF3, CLL and SLL and Joe's local CBC counts were skyrocketing. My dissatisfaction is that I had to find out this on my own AND that Joe should had been referred to the CLL expert who were right there in the same department. It does not change Joe's situation and I really feel that I caught it in time. I am so grateful for on line records that a patient can review  and see for themselves. You should use every tool that is available to  patient.

We have been waiting since last Wednesday a response back from Dr V after his team gets instruction directly from INCYTE Corporation about coming off of INCTE, any adverse concerns with INCYTE with the FCR therapy as well will Joe be able to go back on the INCYTE drug to keep his Quality of  Life good. As of  Tuesday night, we still have no word. I have contacted Dr V and his clincal nurse, Cathy, several times and they stated that they had e-mailed INCYTE but they had not responded and that Cathy sent another e-mail to INCYTE on Monday afternoon. Still no word by late Tuesday afternoon, so I called INCYTE Corporation twice before I was able to get a real person on the phone. I spoke with Pamela, VP of Investor Relations, who said that it would be unlikely that INCYTE would not had responded to Dr V's staff at MDACC. Pam stated that the INCYTE physician on staff in charge is Dr Rick Levy who happened to be in their offices today. She agreed that it was important for MDACC to know about any drug interactions and the recommendation on how to go off the INCYTE. Pam stated that she would have Dr Levy call me but of course he never did. I thought perhaps she had him check his e-mails and/or call Dr V about why is Mrs. Evans contacting him directly. Earlier this evening I e-mailed Dr V and he responded that they he does not know why the delay in getting word from INCYTE and he would have Cathy call INCYTE on Wednesday morning. We shall see but now I have two names at INCYTE Corporation whom I will stay on top of.

Joe continues to stay busy around the yard and vehicles. It is amazing to me how much he does. Today he did even more. He loved the cooler weather that has invigorated him. I still feel that he is blocking all of this out but he is aware of the seriousness of it. I tell him everything I find out since I do not want him to feel that it is out of control. He is eating well and enjoying our dining out.

I am surprised that my little blog has created so much discussion on the two support sites that I am enrolled. It is healthy to have the discussions and I find that they reinforce that what I am doing is right. I love the comments on the blog as well as the e-mails directly to me as well as on the support lists. It is uplifting and I feel less stress and anxiety because of you. Little did I know that I would be helping others when I was just trying to cope. The blog for me has been a success since I am coping so much better. Thank you all.

I will have to do the personal event blog perhaps tomorrow since this one has taken me hours to do. You will surely get a few chuckles when I write the more " life goes on" stuff.

Till then,
Bonnie