T-gamma large granular lymphocyte leukemia associated with amegakaryocytic thrombocytopenic purpura, Sjögren's syndrome, and polyglandular autoimmune syndrome type II, with subsequent development of pure red cell aplasia.
We present a female patient with T-gamma LGL leukemia, who was followed for the last 20 years. Over these years she developed several autoimmune disorders, including Sjögren's syndrome, Hashimoto's thyroiditis, premature ovarian failure (compatible with type II autoimmune polyglandular syndrome), amegakaryocytic thrombocytopenic purpura, and finally pure red cell aplasia. PCR analysis confirmed rearrangement for TCR gamma. This case emphasizes the complex association of LGL leukemia with autoimmune disorders.
Ergas
David
D
Hematology Institute Kaplan Medical Center, Rehovot, Israel.
Tsimanis
Alexander
A
Shtalrid
Mordechai
M
Duskin
Chen
C
Berrebi
Alain
A
D000328:Adult; D001803:Blood Transfusion; D005260:Female; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / Q000235:genetics*; D006801:Humans; D015458:Leukemia, T-Cell / Q000097:blood / Q000276:immunology* / Q000628:therapy; D016884:Polyendocrinopathies, Autoimmune / Q000097:blood / Q000276:immunology* / Q000628:therapy; D016133:Polymerase Chain Reaction; D011247:Pregnancy; D011248:Pregnancy Complications; D016649:Primary Ovarian Insufficiency / Q000276:immunology; D011696:Purpura, Thrombocytopenic / Q000097:blood / Q000276:immunology* / Q000628:therapy; D012010:Red-Cell Aplasia, Pure / Q000097:blood / Q000276:immunology* / Q000628:therapy; D012859:Sjogren's Syndrome / Q000097:blood / Q000276:immunology* / Q000628:therapy; D013967:Thyroiditis, Autoimmune / Q000276:immunology