Table 1.

Principal diseases associated with LGL leukemia

Autoimmune disordersPrevalence
Joint disorders RA 10%-18% 
 Rhizomelic pseudopolyarthritis Sporadic 
 Unclassified inflammatory arthritis Sporadic 
Endocrinopathies Hashimoto's disease Sporadic 
 Cushing's syndrome Sporadic 
 Autoimmune polyglandular syndrome Sporadic 
Connective tissue disorders Sjögren's syndrome 13% 
 Uveitis Sporadic 
 Systemic lupus erythematosus Sporadic 
Muscular and neurological disorders Polymyositis Sporadic 
 Inclusion body myositis Sporadic 
 Peripheral neuropathy Sporadic 
 Lambert-Eaton syndrome Sporadic 
 Myasthenia gravis Sporadic 
Skin disorders Psoriasis Sporadic 
 Livedoid vasculopathy Sporadic 
 Cutaneous vasculitis Sporadic 
 Cutaneous papules and nodules Sporadic 
Vasculitis Small-vessel vasculitis Sporadic 
 Type 1/2 cryoglobulinemia vasculitis Sporadic 
 Glomerulonephritis Sporadic 
 Pulmonary artery hypertension Sporadic 
Bowel disorders Celiac disease Sporadic 
 Inflammatory bowel disease Sporadic 
Immunodeficiencies Good syndrome Sporadic 
 Adenosine deaminase deficiency Sporadic 
Immune cytopenia PRCA 5%-7%a 
 Autoimmune hemolytic anemia Sporadic 
 Immune thrombocytopenia 1%-2% 
 Evans syndrome Sporadic 
Autoimmune disordersPrevalence
Joint disorders RA 10%-18% 
 Rhizomelic pseudopolyarthritis Sporadic 
 Unclassified inflammatory arthritis Sporadic 
Endocrinopathies Hashimoto's disease Sporadic 
 Cushing's syndrome Sporadic 
 Autoimmune polyglandular syndrome Sporadic 
Connective tissue disorders Sjögren's syndrome 13% 
 Uveitis Sporadic 
 Systemic lupus erythematosus Sporadic 
Muscular and neurological disorders Polymyositis Sporadic 
 Inclusion body myositis Sporadic 
 Peripheral neuropathy Sporadic 
 Lambert-Eaton syndrome Sporadic 
 Myasthenia gravis Sporadic 
Skin disorders Psoriasis Sporadic 
 Livedoid vasculopathy Sporadic 
 Cutaneous vasculitis Sporadic 
 Cutaneous papules and nodules Sporadic 
Vasculitis Small-vessel vasculitis Sporadic 
 Type 1/2 cryoglobulinemia vasculitis Sporadic 
 Glomerulonephritis Sporadic 
 Pulmonary artery hypertension Sporadic 
Bowel disorders Celiac disease Sporadic 
 Inflammatory bowel disease Sporadic 
Immunodeficiencies Good syndrome Sporadic 
 Adenosine deaminase deficiency Sporadic 
Immune cytopenia PRCA 5%-7%a 
 Autoimmune hemolytic anemia Sporadic 
 Immune thrombocytopenia 1%-2% 
 Evans syndrome Sporadic 
a

In the West and 29%-48% in Asia.

AIHA, autoimmune hemolytic anemia; ITP, immune thrombocytopenia; T-CUS, T-cell clones of uncertain significance.

or Create an Account

Close Modal
Close Modal