B-Cell Chronic Lymphocytic Leukemia (CLL)
Chronic Lymphocytic Leukemia (CLL) is one of four main types of leukemia. CLL starts with a change (mutation) to the DNA of a single cell called a lymphocyte. In 95 percent of people with CLL, the change occurs in a B lymphocyte.
Over time, the CLL cells multiply and replace normal lymphocytes in the marrow and lymph nodes. The high number of CLL cells in the marrow may crowd out normal blood-forming cells, and CLL cells are not able to fight infection like normal lymphocytes do.
CLL is more common in people who are 60 years and older than in younger adults. The number of people with CLL starts to increase after age 50. A small number of people are diagnosed with CLL in their 30s and 40s. Children do not get CLL.
Doctors do not know what causes the cell-change that leads to CLL. There is no way to prevent CLL.
Small Lymphocytic Lymphoma (SLL)
CLL is a stage of Small Lymphocytic Lymphoma (SLL), a type of B-cell Lymphoma, which presents primarily in the lymph nodes. CLL and SLL are considered the same underlying disease, just with different appearances.
In SLL the abnormal lymphocytes mainly affect the lymph nodes. However, in CLL the abnormal cells mainly affect the blood and the bone marrow. The spleen may be affected in both conditions.
SLL affects 1 in 25 of all cases of non-Hodgkin Lymphoma. It can occur at any time from young adulthood to old age, but is rare under the age of 50. It is twice as common in men as in women.
TP53 with 17p deletion (mutated chromosome in my genes)
17p deletions and p53 mutations predict poor survival and chemo-resistance in CLL. A dysfunctional TP53 with 17p Deeltion gene results is an inability of CLL cells to react to DNA damage that is induced by standard chemotherapy.
This mutated gene makes it difficult for my body to respond positively to chemotherapy. – MC
Richter’s Syndrome
Richter’s Syndrome or Transformation is a rare and aggressive type of acute adult lymphoma that results from a transformation of CLL 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.
Coping with a rare cancer like Richter’s can be especially difficult. You are unlikely to meet or hear about anyone else who has been in the same situation.
The prognosis is generally poor. The RS score (Richter syndrome score), which is an estimate of the patient’s prognosis, is based on the patient’s performance status, LDH, platelet count, the size of the lymphoma tumors, and the number of prior therapies already received. Overall, the median survival is between five to eight months. Untreated, RS is invariably fatal.
Treatment with conventional immunochemotherapy is usually indicated; in younger patients, allogenic bone marrow transplantation may be curative.
Note: info taken from Leukemia & Lymphoma Society’s website, Wikipedia & cancerhelp.org.uk, genzymesgenetic.com, answers.com
Update: As of January 2012, 11 months post stem cell/bone marrow transplant, I have had a relapse of Richter’s Syndrome (also known as Richter’s Transformation or Transformed Non-Hodgkin’s Lymphoma). I had a CHOP-R chemo round on February 8, 2012.
As of February 11, 2012, I have been hospitalized with various extreme Acute GVHD issues (Skin, Mouth, Throat, Digestive System, life-threatening elevated Liver enzymes, Lung Infection, Steroid Myopathy). Every time we lower the steroid levels, it seems that an Acute GVHD rears its head. Hopefully this time, we are able to control these issues and I do hope to be walking out of here in a few weeks or month or so. Here’s hoping! I am convinced I will walk out of here on my own two legs.
Once I am discharged from the hospital, I will be going regularly to the Outpatient clininc to be closely monitored and keep on receiving various meds and treatments and begin dealing with the relapse.
Marie-Chantal
