Nodular sclerosing Hodgkin lymphoma
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Nodular sclerosing Hodgkin lymphoma | |
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Synonyms | N/A |
Pronounce | N/A |
Specialty | N/A |
Symptoms | Lymphadenopathy, fever, night sweats, weight loss |
Complications | Secondary malignancies, cardiovascular disease |
Onset | Typically young adults |
Duration | Variable |
Types | Classical Hodgkin lymphoma |
Causes | Unknown, potential Epstein-Barr virus association |
Risks | Family history, immunosuppression |
Diagnosis | Lymph node biopsy, immunohistochemistry |
Differential diagnosis | Non-Hodgkin lymphoma, infectious mononucleosis |
Prevention | None specific |
Treatment | Chemotherapy, radiation therapy |
Medication | N/A |
Prognosis | Generally good with treatment |
Frequency | Most common subtype of Hodgkin lymphoma |
Deaths | N/A |
Nodular sclerosing Hodgkin lymphoma (NSHL) is a subtype of Hodgkin lymphoma, which is a type of lymphoma, a cancer originating from lymphocytes, a type of white blood cell. NSHL is characterized by the presence of large, abnormal cells known as Reed-Sternberg cells in a background of fibrous tissue and nodular architecture.
Pathophysiology
Nodular sclerosing Hodgkin lymphoma is distinguished by its unique histological features. The lymph nodes affected by NSHL typically show a nodular pattern due to bands of fibrosis that divide the lymphoid tissue into nodules. The presence of Reed-Sternberg cells, which are large, abnormal, multinucleated cells, is a hallmark of the disease. These cells are often surrounded by a mixed inflammatory infiltrate, including eosinophils, plasma cells, and histiocytes.
Clinical Presentation
Patients with nodular sclerosing Hodgkin lymphoma often present with painless lymphadenopathy, most commonly in the cervical or mediastinal regions. The disease may also present with B symptoms, which include fever, night sweats, and weight loss. These symptoms are indicative of systemic involvement and are important for staging the disease.
Diagnosis
The diagnosis of NSHL is typically made through a combination of clinical evaluation, imaging studies, and histopathological examination of a lymph node biopsy. The biopsy will reveal the characteristic nodular sclerosis and the presence of Reed-Sternberg cells. Immunohistochemistry is often used to confirm the diagnosis, with Reed-Sternberg cells typically expressing CD15 and CD30 markers.
Treatment
The treatment of nodular sclerosing Hodgkin lymphoma usually involves a combination of chemotherapy and radiation therapy. The most common chemotherapy regimen used is ABVD, which includes doxorubicin, bleomycin, vinblastine, and dacarbazine. The choice of treatment depends on the stage of the disease, the presence of B symptoms, and the overall health of the patient.
Prognosis
The prognosis for patients with nodular sclerosing Hodgkin lymphoma is generally favorable, especially when diagnosed at an early stage. The overall survival rate is high, with many patients achieving long-term remission. However, the prognosis can vary based on factors such as the stage of the disease at diagnosis and the patient's response to treatment.
See also
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Contributors: Prab R. Tumpati, MD