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

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 distinguished from the much more aggressive NK cells leucemia.

Disease Cause

Unknown cause.

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

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)

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 metotrekste 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%

Literature


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