What is Lymphoplasmacytic Lymphoma?
Lymphoplasmacytic Lymphoma is a type of white blood cell cancer in which abnormal excessive growth of B lymphocytes occurs characterized by the presence of small lymphocytes and IgM (immunoglobulin/antibody M) monoclonal gammopathy. Gammaopathy is the presence of abnormal proteins (antibodies) in blood. Lymphoplasmacytic lymphoma is a low-grade lymphoma (cancer of white blood cells). Low-grade cancer is cancer which grows and spread slowly than high-grade tumour and nearly similar in appearance to the normal cells.
Disease Etiology (Cause)
There is no exact well-known etiology, however, an association with hepatitis C and autoimmune disorders was observed. In 20% of cases, there is always a genetic susceptibility of developing lymphoplasmacytic lymphoma. Patients who have a genetic tendency to develop lymphoplasmacytic lymphoma present with signs and symptoms at an earlier stage of life and show a greater degree of bone marrow involvement.
It is a rare disease with a frequency of occurrence of the disease is 3-4 cases per million populations. It takes only 2% of all the blood cancers. It is slightly more common in males as compared to females. It is a disease of old age that mostly develops in the 7th to 8th decade of life.
Signs and Symptoms
The patient may present with the following signs and symptoms. These are;
Symptoms of Bone Marrow Involvement:
- Due to the frequent involvement of bone marrow patients may present with the symptoms of anemia (decreased RBCs count or decreased oxygen-carrying capacity of the blood), lethargy, and weakness.
- Neutropenia: decreased neutrophils (cells fight against the infectious agents) resulted in recurrent infections.
- Thrombocytopenia: decreased platelet leading to a tendency to bleeding and bruising.
Symptoms of Monoclonal IgM (Accumulation of Abnormal Proteins/Antibodies in the Blood):
- Bleeding from nose
- Blurred vision
- Difficulty in breathing( Shortness of breath)
- Headache and dizziness
- Tinnitus ( ringing sensation in ears)
- Lack of concentration or poor concentration
- Swollen and enlarged lymph nodes
- Some patients may present with the B or systemic symptoms where lymphoplasmacytic lymphoma spread to the other distant parts of the body like fever, night sweats, and weight loss.
- In a few cases, the involvement of spleen, liver, stomach, skin, intestines, and other extranodal sites are also observed.
Diagnosis and Treatment
Serological, radiological, and biopsy investigations are useful to diagnose. These are:
- Complete blood count (CBC) with peripheral smear and blood culture
- Blood coagulation profile
- LFT’s(Liver function tests) and RFT’s( Renal function tests)
- Bone marrow aspiration and biopsy
- Chest X-ray, CT chest
- MUGA and Echocardiography
- Lumbar Puncture
Asymptomatic patients are managed by close observation. Treatment of the symptomatic patient depends upon the extent of the disease and extent of the end-organ involvement. Chemotherapy, immunotherapy, and autologous stem cell transplant for the bone marrow are used to treat symptomatic patients. Chemotherapeutic agents effective in treating macroglobulinemia in Waldenstrom include dexamethasone, fludarabine, bortezomib, and cyclophosphamide.
What Support can we Give for Lymphoplasmacytic Lymphoma?
Lymphoplasmacytic Lymphoma is a rare cancer, meaning it is not as well known as other forms of cancer. Without a Ribbon is an Australian organisation that provides support for individuals who suffer from rare cancers. So, we provide a designated platform for Warriors to obtain information specific to their Rare Cancer. We also provide annual opportunities for our Warriors to meet and learn from each other. If you suffer from rare cancer such as Lymphoplasmacytic Lymphoma, we can help and support you through your journey thanks to the generous donations we receive. Click the link below to sign up and become a Warrior today!
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