Nodular Histiocytic Lymphoma, also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare form of lymphoma, a type of cancer that affects the lymphatic system. It is classified as an indolent lymphoma because its progression tends to be slow.
This condition primarily affects the lymph nodes, particularly those found in the neck and mediastinum (the area between the lungs). It is characterized by the presence of large nodules or masses within the lymph nodes, composed of abnormal histiocytes (a type of white blood cell). These histiocytes, which are part of the immune system, lose their normal function and undergo abnormal growth, forming these nodules.
The exact cause of nodular histiocytic lymphoma is unknown, but it is believed to be associated with an abnormal immune response or genetic factors. Symptoms may vary but typically include painless swelling of the affected lymph nodes, fever, night sweats, fatigue, and weight loss.
Diagnosis is usually confirmed through a combination of physical examination, imaging tests such as CT scans or MRIs, and a biopsy of the affected lymph nodes. Treatment options for nodular histiocytic lymphoma depend on the extent and severity of the disease, and may include watchful waiting, radiation therapy, chemotherapy, or targeted therapy.
Prognosis for nodular histiocytic lymphoma is generally favorable, with a good long-term outlook. However, regular follow-up appointments and monitoring are necessary to detect any potential recurrence or progression of the disease.
