LGL syndrome and rheumatic disease 4/5 (2)

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LGL syndrome

Bone marrow in LGL syndrome. Prochorec-Sobieszek M, 2008CC 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


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.


  • 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

Blood tests

Lymphocytosis (2-20 x 109/ L), neutropenia, anemia, hypergammaglobulinemia, Antibodies, Circulating immune complexes.

Bone marrow

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)

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)

Medical prognosis

No curative treatment, but generally good prognosis. 5 Year Survival 89%


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