Sinus histiocytosis

Description of Ligos

This is a disease characterized by proliferation, infiltration, and accumulation of histiocytes in various tissues of the body. It is histiocytosis driven by macrophages, the etiology of which is unclear. It is believed that the disease develops due to the accumulation of histiocytes and lymphocytes in the lymph nodes, disrupting their function. Immune regulation may be disrupted due to an abnormal antibody response to the measles virus.

Symptoms

Individuals up to 20 years old suffer from SHML. The disease is characterized by chronic, painless enlargement of the cervical lymph nodes, fever, often accompanied by infiltration of eyelids, eye sockets, salivary glands, liver, spleen, bones, testicles, and brain.

Many experience nonspecific symptoms – when the retropharyngeal lymphoid tissue enlarges, patients may snore, sometimes even develop sleep apnea (breathing stops for a while during sleep). Less commonly, spinal cord compression occurs due to epidural infiltration.

Diagnosis

A blood test reveals anemia, increased leukocyte count, decreased neutrophils, ESR up to 50mm/h, leukophagocytosis, and increased globulin levels.

Treatment

There is no specific treatment, in severe cases prednisolone is prescribed, if it does not help, 6-mercaptopurine and methotrexate are given, sometimes alpha-immunoglobulin is effective. About 20% recover within 3-9 months, while for others, the disease persists for several years.

Source | Author Doctor Nikas Samuolis, reviewed by Prof. Virginijus Šapoka | Vilnius University | Faculty of Medicine | Head of the Department of Internal Medicine, Family Medicine, and Oncology