LGL Syndrome / LGL Leukemia (Large Granular Lymphocyte (LGL) Syndrome, T-Cell Granular Lymphocytic Leukemia, Large Granular Lymphocyte (LGL) Leukemia).
LGL syndrome is defined as a neoplastic disease (REAL classification) with chronically increased number of large granular monoclonal lymphocytes (white blood cells) (CD3 + cytotoxic NK cells) in blood and bone marrow. The disease was first described in 1985 and is separated from the much more aggressive NK cell leucemia.
- Most have too
- Men and women
- Most commonly aged 12 - 87 years, median 60 years of age
Symptoms and results of medical examination
get symptoms. Often random discovery by medical examination of recurring infections
- The most common are:
- Associated diseases present concurrently with LGL leukemia (40-60%):
Often affected, but to varying degrees.
- Measurement of neutrophil leukocytes (typical discovery: 0,4-2,9 x 109/ L).
- Bone marrow aspirate or biopsy with immunohistochemistry.
Incorrect diagnosis? (Similar diseases / differential diagnoses)
- Viral infections and Autoimmune diseases (Transient or chronically increased polyclonal T-cell or NK-cell lymphocytosis)
- Older persons (oligoclonal and small monoclonal CD3 + LGL cells)
- After transplants (Oligoclonal and small monoclonal CD3 + LGL cells)
- Felty's syndrome (by Rheumatoid arthritis)
Genetically: Clonal changes in T-cell receptors exist. In the gene for the T-cell β chain, more frequent changes than the chain are present.
Not all cases require treatment. If severe or symptomatic neutropenia, anemia or rheumatoid arthritis (RA) a low dose methotrexate or Cyclophosphamide (Sendoxan) as initial treatment are options. Alemtuzumab has been used in refractory cases.
Treatment response can be evaluated after 4 months: Normalization of cell counts (Hemoglobin over 12 g / 100mL, platelets over 150 x 109/ L, neutrophilic leukocytes over 1,5, lymphocytes under 4 and circulating LGL normalized). By PCR examination T-cell clones can be measured (should be gone by complete remission). Steroids (Prednisolone) have a disappointing effect outside of temporary symptom improvement. (Reference: Lamy T, 2011)
No curative treatment, but generally good prognosis. 5 Year Survival 89%