
Bone marrow in LGL syndrome. Prochorec-Sobieszek M, 2008, CC BY 2.0
LGL Syndrome / LGL Leukemia (Large Granular Lymphocyte (LGL) Syndrome, T-Cell Granular Lymphocytic Leukemia, Large Granular Lymphocyte (LGL) Leukemia).
ICD-10: C91.5
Contents
Definition
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.
Disease Cause
Unknown cause.
- Most have too
- Rheumatoid arthritis (The disease picture overlaps in part with Felty's syndrome) (reference: Wlodarski MW, 2006)
- SLE or
- Sjögren's syndrome (Reference: Friedman J, 2006)
Occurrence
- 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:
- Splenomegaly (large spleen) (25-50%)
- Anemia (low hemoglobin in the blood) (erythroid hypoplasia)
- Neutropenia (few neutrophils of white blood cells)
- Recurring infections (at 50%)
- Rarely enlarged lymph nodes
- Associated diseases present concurrently with LGL leukemia (40-60%):
Blood tests
Lymphocytosis (2-20 x 109/ L), neutropenia, anemia, hypergammaglobulinemia, Antibodies, Circulating immune complexes.
Bone marrow
Often affected, but to varying degrees.
Diagnosis
- 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.
Treatment
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)
Medical prognosis
No curative treatment, but generally good prognosis. 5 Year Survival 89%