Large granular lymphocytic (LGL) leukemia is an uncommon disease, characterized by a clonal proliferation of mature, post-thymic T-cells, typically CD3+, CD4-, CD8+, CD16+, CD57+ phenotype, representing constitutively active T-cells Less commonly, LGL leukemia is derived from CD3-, CD56+ natural killer (NK) cells. Clonal T-LGLs escape apoptosis by failure to respond to the Fas/Fas ligand (FasL) pathway. Activating mutations in the STAT3gene occur frequently in LGL leukemia, and may play a role in pathogenesis. Autoimmune disorders are frequently associated with LGL leukemia (~1/3 present with rheumatoid arthritis).
The association between LGL leukemia and B-cell lymphoproliferative disorders has been reported, often with low-grade histologies, but is deemed uncommon and the pathogenesis is not well established. We have analyzed a series of patients (pts) diagnosed with both LGL leukemia or expansion and clonal B-cell disorders.
Patients and methods:
Pts with NK or T-LGL leukemia or expansion who were evaluated at Fox Chase Cancer Center or the Cleveland Clinic Taussig Cancer Institute were reviewed, after Institutional Review Board approval. Inclusion criteria were age ≥ 18 yrs and diagnosis of both LGL and B-cell lymphoproliferative disorder.
Results:
One hundred and twenty six pts with a diagnosis of T-LGL leukemia, NK-LGL leukemia or T-LGL expansion were identified. Of these, 44 (34.9%) pts were diagnosed with a clonal B-cell disorder. Twenty-six pts (20.6%) were diagnosed with a clonal B-cell disorder concomitantly with or shortly after the LGL diagnosis, 15 of whom presented with monoclonal gammopathy of unknown significance (MGUS) as their B-cell disorder, 9 with monoclonal B-cell lymphocytosis (MBL), 5 of whom also had monoclonal gammopathy . Eighteen pts (14.2%) had a previous diagnosis of clonal B-cell disorder, including diffuse large B cell lymphoma (DLBCL) (N= 6), CLL (N = 3), mantle cell lymphoma (N=3), multiple myeloma (N = 2), Hodgkin lymphoma (N = 2), Burkitt lymphoma (N = 1) and hairy cell leukemia (N = 1 ). Fifteen pts (11.9%) received treatment prior to the diagnosis of LGL, 10 of them (7.9%) with regimens including rituximab. The median time from completion of last treatment with rituximab to diagnosis of LGL disorder was 33 months. An additional patient with prior DLBCL was diagnosed with LGL shortly after receiving an oral SYK inhibitor.
Two illustrative patients had unexpectedly prolonged remissions of their B cell disorder. A 66 years old man with multiple myeloma who achieved complete remission (CR) after 8 months of bortezomib therapy was then diagnosed with T-LGL, and his myeloma is in ongoing remission now 5 years after T-LGL diagnosis without further therapy.
If B-cell and T- cell are identified under a microscope then it is Non- Hodgkin lymphoma. If Reed - Sternberg cell is present then it is Hodgkin lymphoma. There are different tools to diagnose these cancers and different options of treatments. One treatment that was approved by the FDA (food and drug administration ) seems to have greater success in the fight against Non Hodgkin lymphoma. Most Patients treated with only Chemotherapy will reach remission at some point but eventually will die. However the development of new treatment where chemotherapy or radiotherapy are given in addition to Monoclonal Antibodies have a higher rate of success in defeating the cancer all together. Monoclonal Antibodies are laboratory engineered molecules that are engineered to attach to a specific protein that is found only in B-cell. The immune system task is to fight invaders, however it does not always recognize the cancer as a harmful invader and does not attack it. The Monoclonal Antibody are directed to attach to a specific part of the cancer, marking the cancer cell as harmful and making it easy for the immune system to identify it and destroy it. Scientists are looking to to find out the reason lymphocytes turn into cancer, why they grow fast or live longer then they should. Once this is understood the focus will be on finding a cure for the disease. There are new drugs being tested on patients and studies are being done on how to improve steam cell transplant, and new drugs are being tested. A cure may be out of reach for time being but treatments and research are advancing every
This group accounts for approximately 5% of all cases. It is associated with the worse prognosis. It is most commonly seen in elderly males. Patients typically present with advanced staged disease and B-type symptoms. Lymphocyte depleted Hodgkin’s disease closely resembles non-Hodgkin’s lymphoma.
Causes of Hodgkin’s lymphoma are not known but it is most common in young people age 15 to 35 and in older patents of 50 to 70 years old.
What is Leukemia? Leukemia is a cancer in blood forming cells that are in early stages of development. Most of the time the white blood cells, but some types of leukemia start in other types of blood cells. Blood cells are form in bone marrow. Any of these cells can turn into a leukemia cell, once this happens the cell does not mature like it should. The cell may start to produce rapidly and the mutated cells probably won’t go through apoptosis like they should. These cells build up in the bone marrow and crowd out the healthy cells. Typically, leukemia cells get into the blood stream rather quickly. From the blood stream they can spread to places like lymph nodes, spleen, liver, central nervous system or other organs where the leukemia cells can cause those other cells to function irregularly.
Lymphomas are a group of neoplastic diseases that come from the lymphoid and hematopoietic systems and are divided into Hodgkin disease and NHL. In Hodgkin disease the cancer starts in the lymphoid system and mostly involves the lymph nodes. It can spread to the spleen, liver, bone marrow, lung and the mediastinum. The outlook is excellent in children with localized disease. Overall survival rates for patients with Hodgkin disease is as high as 95%; however, the survival rate is dependent on histology and staging. The primary clinical symptom are painless enlargement of lymph nodes. Other signs and symptoms depend on the extent and location of involvement. Diagnostics consist of multiple tests to confirm the presence of Hodgkin's disease and to evaluate the extent of involvement for acute staging. The tests include CBC, uric acid levels, liver function test, erythrocyte sedimentation rate or C-reactive protein, alkaline phosphatase, and urine analysis. Radiographic tests include CT scans of the neck, chest, abdomen and hip, pet scans, chest x-ray, and if indicated a bone scan to detect
30 percent of the cases are Lymphoblastic non-Hodgkin lymphoma and they usually involves the T-cells, and typically presents with a mass in the chest, swollen lymph node, with or without bone marrow and central nervous system involvement. Burkitt's and non-Burkitt's lymphoma are fast-growing lymphomas in which the cells are undifferentiated and diffuse. This has also been referred to as small noncleaved cells. Burkitt's and non-Burkitt's lymphoma accounts for about 40 percent of the cases. They are usually characterized by a large abdominal tumor and may have bone marrow and central nervous system involvement. Large cell or diffuse histiocytic non-Hodgkin lymphoma. Large cell or diffuse histiocytic non-Hodgkin involves the B-cells or T-cells and accounts for about 25 percent of the cases. Children with this type of non-Hodgkin lymphoma usually have lymphatic system involvement, as well as a non-lymph structure involvement. Anaplastic large cell lymphoma is a type of large cell lymphoma in children. Large cell lymphomas usually do not grow as quickly as other lymphomas in
The University of Texas MD Anderson Cancer Center states the disease is divided into two major types namely acute and chronic. The acute types of the disease are those that progress quickly and involve an overgrowth of very immature blood cells. This becomes life threatening because very few mature cells mean that the body loses its ability to prevent infection, anemia and bleeding disorders. A diagnosis of the acute type is given when the immature cells found account for 20% or more of the blood cells produced. The chronic type progress slowly and involves an overgrowth of mature blood cells. In contract to the acute type people affect by this type usually h...
While lymphoma is a complex cancer of the lymph system that can have devastating effects, it can be overcome. Over 75% of those with intermediate-risk lymphoma have at least a five year survival rate. And that rate is likely to increase as detection and treatment options improve with new genetic research and lymphoma vaccine developments. Maybe one day there will even be a cure.
95% of the genetic mutations resulting in CLL are in B-cells. The other 5% are in T- or NK-cells. These mutations result in abnormal lymphocytes (leukemic cells). As the cancer progresses from the blood to the marrow, the increased number of cancerous cells push healthy white blood cells, red blood cells and platelets out of the bone marrow. This “takeover” of the marrow can result in anemia, reduced ability to fight infection and easy bleeding. The increased rates of secondary infections are the result of decreased infection-fighting capacity of white blood cells.
Therefore, being an older Caucasian male, having an autoimmune disease, eating a diet high in meat and fat and exposure to certain pesticides increase the risk of the disease. Lymphoma can be cured using several method of treatment such as: chemotherapy, radiation therapy, and immunotherapy using monoclonal antibodies, immune checkpoint inhibitors, and CAR T-cells. Targeted therapy is another treatment method with newer drugs that block certain functions within the lymphoma cell. Lymphoma cannot be prevented because the cause remains unknown, however, it might be triggered by an infection or exposure to the radiation. Chills, fevers, unexplained weight loss, loss of appetite, persistent coughing, breathlessness, Persistent itch all over the body without an apparent cause or rash and general fatigue are the symptoms associated to the
Diagnosis of CLL is usually straight forward. Most cases are usually suspected by the presence of peripheral blood lymphocytosis of small mature lymphocytes with scant cytoplasm. Smear cells are typically feature of the leukaemic cells due to increased cellular fragility. International Workshop on Chronic Lymphocytic Leukaemia criteria for diagnosis require >5x10^9/L of clonal B lymphocytes. The clonality of the circulating b cells must be confirmed with Immunophenotyping by flow cytometry(1). When a clonal population of B
Acute lymphocytic leukemia (ALL) is a form of cancer that occurs when the lymphoid stem cell is affected thereby causing abnormal white blood cells to build up in the bone marrow. This cancer rapidly produces and replaces healthy cells with immature lymphoblast. The leukemia cells moves into the bloodstream to other organs and tissues where further growth and division occurs. The proliferation of cancer cells to other area produces a variety of symptoms. ALL typically occurs in children under the age of 15, however it can happen at any age. ALL is
“The word 'leukemia' is a very frightening word. In many instances, it's a killer and it's something that you have to deal with in a very serious and determined way if you're going to beat it” - Kareem Abdul-Jabbar. Many people, including tons of children, fight leukemia every day trying to beat this vicious cancer. Without knowing how leukemia is exactly caused, it puts a damper on how to avoid it.
Lymphomas are malignancies of the lymphoid tissue, which includes the lymphocytes, lymph nodes, the spleen and bone marrow (Pearson, 2015). The two types of lymphomas are Hodgkin’s lymphoma and Non-Hodgkin’s lymphoma (NHL). Unlike Hodgkin’s lymphoma, the cells of NHL do not contain the Reed-Sternberg cell. Instead, this disease involves lymphoctyes (B, T, or Natural Killer cells) in various stages of development and may mimic a leukemia (Lewis et al, 2016 ). Nearly 70,000 people are diagnosed with Non-Hodgkin’s lymphoma in the United States each year (Sommers & Fannin, 2015). Although the definite cause of NHL is unknown, chromosomal abnormalities and exposures to viruses and infections are suspected as some.
Kanwar, V.S. (2013, Sep 16). Diseases & Conditions - Medscape Reference. Pediatric Acute Lymphoblastic Leukemia. Retrieved January 13, 2014, from http://emedicine.medscape.com/article/990113-overview#a0156