Introduction
Primary bone cell lymphoma is a type of lymphoma that mainly affects the skeletal muscles. But unlike the original type, which involves bone and other structures, primary bone cell lymphoma exclusively involves only the skeletal muscles. Primary bone cell lymphoma comprises almost three to seven percent of the cases of primary bone cell tumors, with a 2 percent predominance in adults. Primary bone cell lymphoma can be a single bone tumor involving only the localized lymph nodes (lymph nodes are small round bodies into which the bodily fluids drain) or multiple bone lesions with localized nodal involvements.
What Is Called a Primary Bone Cell Tumor and Lymphoma?
Primary Bone Cell Tumors: These are cancerous tumors originating from primitive bone cells. Primary bone cell tumors often occur due to an unknown cause and are very aggressive, requiring an early diagnosis and management. Primary bone cell tumors can be of three types: primary bone cell lymphoma, multiple bone tumors, and bone and soft tissue lymphoma.
Lymphoma: It is a type of cancer arising from the white blood cells (WBC) called lymphocytes. Lymphocytes are the defense cells of the body that fight against infection.
How Does a Primary Bone Cell Lymphoma Occur?
The exact cause of primary bone cell lymphoma is unknown, but viral infections and low immunity levels are predisposing factors for primary bone cell lymphoma. Genetic changes and molecular abnormalities in the cell are also associated with primary bone cell tumors.
How Commonly Does a Primary Bone Cell Lymphoma Occur?
Primary bone cell lymphoma is a rare condition from all the other primary cell tumors, with an incidence of less than ten percent globally. It is more common, affecting males than females. Primary bone cell tumor involves people of all age groups but predominantly in patients between the age of 35 to 55. The secondary involvement of bone marrow is seen in around five to fifteen percent of patients with Hodgkin’s lymphoma (Hodgkin's lymphoma is an uncommon type of cancer occurring due to gene-altering in the DNA seen affecting the white blood cells) and 30 to 53 percent in non-Hodgkin's lymphoma (it is a genetically transmitted type of cancer involving the white blood cells).
How Is a Primary Bone Cell Lymphoma Seen Clinically?
The clinical features seen in patients with primary bone cell lymphoma are extreme loss of sensation due to compression in the spine, fever, night sweats, loss of weight, and around 25 percent of pathological fracture due to bone weakening. The commonly involved bones depending on the order of decreasing frequency, are the femur (thigh bone), humerus (bone of the arm), tibia (bone of the leg), spine, hip bone, sternum (chest bone), ribs, and bones of the skull and face. The involvement of small bones of the hand and foot is usually uncommon. Primary bone cell tumor is often associated with patients with immunocompromised disorders like AIDS (acquired immunodeficiency syndrome).
How Is Primary Bone Cell Lymphoma Investigated?
Primary bone cell lymphoma is investigated in different aspects to confirm the diagnosis:
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Physical Examination: Physical examination is done by palpitating all the regional lymph nodes. The lymph nodes appear prominent when observed and palpated.
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Imaging Methods: Images of multiple techniques are taken to study the changes in primary bone cell lymphoma.
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Radiographic Imaging (X-Ray): A radiograph is a two-dimensional imaging done to study a bone cell tumor. The radiographic images are taken to study the structure of the bone. The lesion appears to be a round well-circumscribed white lesion in the X-ray. These tumors appear very subtly in a radiograph.
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Computed Tomography (CT): Computed tomography is a three-dimensional image that gives knowledge about the tumor, its position, and its associated structure. CT images of the chest, hip bone, and abdomen are taken to study.
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Magnetic Resonance Imaging (MRI): Magnetic Resonance Imaging or MRI, is an imaging technique used to study bone and the associated soft tissues. The images of the primary bone cell lymphoma appear very dense and prominent in an MRI. It has extensive tissue involvement compared to bony involvement.
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Positron Emission Tomography (PET Scan): The PET scan is taken in patients by injecting nuclear medicines to find abnormal growth. The PET scan in primary bone cell lymphoma shows increased uptake of nuclear medicine.
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Biopsy: A fine needle aspiration biopsy (FNAC) is done to extract the contents of the tumor to confirm its presence. Other biopsy procedures, like frozen sections, are also taken to study the structure of the cells.
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Blood Culture: Blood tests are done to study the normal blood metabolic levels like alkaline level, lactate level, and blood clotting time, along with blood calcium levels, urine analysis, and liver function tests are taken.
How Is Primary Bone Cell Lymphoma Treated?
Primary bone cell tumors can be treated by two methods, and they are:
Non-Surgical Management: The non-surgical treatment methods involve treating the tumor with surgery or invasive treatments. The treatments commonly used are:
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Chemotherapy: Chemotherapy consists of administering drugs like Cyclophosphamide which suppress the growth of the tumor, along with the antibodies giving immunity.
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Radiotherapy: Radiation therapy is done by giving radiation in a localized area to kill the tumor cells. It is given to control the growth of the tumor by targeting the multiplying cells.
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Combination Therapy: A combination of chemo and radiotherapy shows better results in the treatment outcome. Chemotherapy with a mixed group of drugs is preferred in children showing aggressive tumors, as radiation can affect children's growth. Bone marrow transplantation is also preferred in recurrent manifestations.
Surgical Management: It is not the first line of treatment preferred for managing primary bone cell lymphoma. Patients show an evident result in combination therapy, and no particular surgical management is given, but in severe cases, amputation of the affected site is preferred.
What Are the Post-Operative Complications of Primary Bone Cell Lymphoma?
The complications following the treatment of primary bone cell lymphoma are fractures due to weakness, non-union of the fractured segment, infection of the wound, toxicity due to chemotherapy, inflammation of the blood vessels, bone death, and failure of fracture fixtures.
What Is the Prognosis of Primary Bone Cell Lymphoma?
Primary bone cell lymphoma has a better prognosis in comparison to other tumors of the bone. It shows a favorable outcome of 40 to 50 percent in patients of all age groups, with a highly increased positive outcome in children and younger patients.
Conclusion:
Primary bone cell lymphoma is an uncommon disease of the white blood cells affecting patients of all age groups. Periodic monitoring of the patients is important to decide on the treatment plan. With a proper diagnosis and treatment plan, patients show an excellent prognosis.
