Neutropenia-hyperlymphocytosis with large granular lymphocytes syndrome

Neutropenia-hyperlymphocytosis with large granular lymphocytes syndrome, also known as large granular lymphocyte (LGL) lymphocytosis with neutropenia, is a rare blood disorder. In this condition, the body has too few neutrophils (a type of white blood cell that fights bacterial infections) and too many large granular lymphocytes (another type of white blood cell). Large granular lymphocytes are a special group of immune cells that normally help fight viruses and cancer cells, but in this syndrome they multiply excessively and crowd out or suppress normal neutrophil production. The main concern with this disease is the low neutrophil count (neutropenia), which makes patients more vulnerable to frequent and sometimes serious bacterial infections. Patients may experience recurrent fevers, mouth sores, skin infections, and other infections that are hard to shake. Some patients also develop an enlarged spleen (splenomegaly) and may have associated autoimmune conditions, particularly rheumatoid arthritis. Fatigue is also a common complaint. Treatment typically focuses on managing the neutropenia and preventing infections. Immunosuppressive medications such as methotrexate, cyclophosphamide, or cyclosporine are commonly used to control the abnormal lymphocyte expansion and improve neutrophil counts. Growth factors like granulocyte colony-stimulating factor (G-CSF) may be used to boost neutrophil production. In some cases, the disease follows a chronic, indolent course, but careful monitoring is important because a small number of cases may progress to a more aggressive lymphoproliferative disorder.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Bring these to your next appointment

• Q1.What is my current neutrophil count, and how low is it compared to normal?,Which treatment do you recommend starting with, and what are the possible side effects?,How often will I need blood tests to monitor my condition?,What signs of infection should I watch for, and when should I go to the emergency room?,Is there a risk that my condition could become more aggressive over time?,Should I be tested for associated autoimmune conditions like rheumatoid arthritis?,Are there any clinical trials or newer treatments I should know about?

Common questions about Neutropenia-hyperlymphocytosis with large granular lymphocytes syndrome

Related conditions

Other rare diseases commonly researched alongside Neutropenia-hyperlymphocytosis with large granular lymphocytes syndrome.

• Felty syndrome→