Monday, August 19, 2013

UPDATED: Mary Jo Update #48: NED, RS/RT: The Good, The Bad and The Ugly

I have not been posting lately. Mainly, because nearly all of the news since we arrived home since Mary Jo's transplant has been good news. I figured that most people would think that "no news is good news."

Mary Jo celebrated her Day+100 last Wednesdy, August 14th. We are at Vanderbilt today for a PET scan, bone marrow biopsy, pulmonary function test, and meeting with the transplant coordinator, Dr. Nashitha Reddy, to evaluate the success of her transplant.

The results are in for everything, except for the bone marrow biopsy, and the report is fantastic - NED - No Evidence of Disease. The initials and words that every cancer patient want to hear. It will take a couple of days for bone marrow results, but there is no reason to expect the result will be any different from the others. Dr. Reddy has released Mary Jo back to the care of her oncologists in Louisville.

We owe a debt of gratitude to all of the doctors, nurses, nurse practitioners, aides, and social workers here at Vanderbilt. Needless to say Vanderbilt and the dedicated people who are performing miracles everyday here will always hold a special place in our hearts. Go Vandy!

Mary Jo has had a couple of mornings where she has had some unexplained dizziness, and it appears that the BCNU (carmustine) chemotherapy that she received just prior to her stem cell transplant on May 6th has caused some diastolic dysfunction with her heart.

Her blood labs since we returned home, all indicated that Mary Jo's MCL was in remission and the transplant was successful. Other than having some periods of fatigue, and the things that I mentiones, things have been pretty much back to normal with us.

Unfortunately, all the news that I have for you is not good. We received some disturbing news about Mary Jo's nephew, Billy, at the end of last week. Billy was diagnosed with Chronic Lymphocytic Leukemia (CLL) about the time that Mary Jo was diagnosed with MCL in October, 2009. Billy had completed a clinical trial here at Vanderbilt for refractory CLL patients using the BK inhibiting drug Ibrutinib.

Billy had been taking Ibrutinib for several weeks after the clinical trial ended back in June. His lymph nodes had shrunk. Everything was looking great. there was the possibility of an allo stem cell transplant somewhere far out in the future. Billy was feeling great both physically and mentally. he was full of hope for the future, and getting his life back to normal.

Billy had a CT scan a few weeks ago to evaluate the great progress that had been made with the Ibrutinib. Without any adverse symptoms, the CT indicated that lymph nodes in his back and in his chest area were enlarged. One near his heart measured 5cm.

When Billy's doctor at Vanderbilt gave Billy the bad news, he scheduled a CT guided biopsy of one of the affected lymph nodes last week. The pathology report indicates that Billy's CLL has transfomed into an aggressive blood cancer called diffuse large cell lymphoma. When this occurs the lymphoma is called Richter Syndrome RS, or Richter Transformation RT. I have included some information about RS/RT below

Last week while Billy was here at Vanderbilt, the lymph nodes in his back had grown to the point that they were causing pressure on his kidneys causing excruciating pain. The pain became so intense Thursday night that he went to Vanderbilt's emergency room. He is now on a pain killer to manage the pain.

We were able to see Billy, and his friend, Rita, yesterday over at Hope Lodge before they went home, and I talked to him again this evening after we got home from Nashville. Billy will be going back to Nashville on Wednesday to begin chemotherapy treatments to reduce the size of his lymph nodes.

Dr. Olalekan O. Oluwole, his doctor at Vanderbilt told Billy that the game plan is for him to have three-five day treatments with the R-EPOCH chemo regimen with 21 days off after each of the three-five day treatments. Then they will begin the allo stem cell transplant process. The R-EPOCH chemo is much stronger than any chemo that Mary Jo has had which says alot for the powerfully, agressive nature of the Richter Syndrome.

Dr. Oluwole told Billy that he is the first CLL patient at Vanderbilt who has had CLL with the P17 deletion that has transformed into Richter Syndrome. It appears that Billy's transplant could happen sometime in November. He will have an allo stem cell transplant, using his sister's stem cells, as soon.

Needless to say, this development has bummed us all out. RS/RT is so aggressive and can cause the patient's health to deteriorate so rapidly and severely that time is of the essence.

Please pray for Billy in the coming weeks and months, and thanks again for all of your wonderful thoughts and prayers for Mary Jo and I over the last several months. I will continue to keep you posted on Billy's journey. Hopefully, Mary Jo's "no news is good news" status will last for a long time. Amen



Richter's syndrome is a rare and aggressive type of acute adult leukemia that results from a transformation of chronic lymphocytic leukemia into diffuse large cell lymphoma.


Leukemia is a group of cancers of the white blood cells. In adults, white blood cells are made in the bone marrow of the flat bones (skull, shoulder blades, ribs, hip bones). There are three main types of white blood cells: granulocytes, monocytes, and lymphocytes. Richter's syndrome concerns only the lymphocytes.

Lymphocytic leukemia develops from lymphocytes in the bone marrow. Unlike many other cancers in which a tumor starts growing in one particular location, lymphocytic leukemia is a disease of blood cells that travel throughout the body. In chronic (long-term) lymphocytic leukemia (CLL), lymphocytes do not follow a normal life cycle, and eventually, too many will exist in the blood. They are abnormal and do not fight infections well.

In a small percentage of people, CLL, even when it is treated, transforms into a new kind of aggressive blood cancer called diffuse large cell lymphoma. When this transformation occurs, it is called Richter's syndrome. The disease is named for the American pathologist Maurice Nathaniel Richter, who practiced medicine early in the twentieth century.


Richter's syndrome is a disease of older adults. It is an extremely rare disease. In a recent year, the American Cancer Society estimates that, there were over 8,000 new cases of chronic lymphocytic leukemia, and that 98% of these were in adults. Of these new cases, 5% or less, will develop into Richter's syndrome.

In general, people who are more likely to get CLL are those who smoke, have been exposed to high doses of radiation, or who have had long-term exposure to herbicides, pesticides, or other chemicals. People who have close relatives (parent, siblings or children) with CLL are also more likely to develop the disease. However, none of these risk factors predict whether CLL will develop into Richter's syndrome.

Causes and symptoms

Scientists have yet to understand why some people develop Richter's syndrome and others do not. So far, no firm genetic or environmental links have been found.

When the transformation from CLL to Richter's syndrome occurs, a change occurs in the way the lymphocytes look under the microscope. In addition, lymph nodes swell, tumors grow rapidly in the lymph system, and the patient may experience fever, night sweats, and weight loss. The patient's health deteriorates rapidly and severely.

No comments:

Post a Comment