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Large granular lymphocytic proliferation-associated cyclic thrombocytopenia
Patrick F Fogarty1, Maryalice Stetler-Stevenson, Aloysius Pereira
1Department of Laboratory Medicine, Clinical Center, National Institutes of Health, Bethesda, Maryland, USA. pfogarty@medicine.ucsf.edu
American Journal of Hematology
|July 27, 2005
Summary
Cyclic thrombocytopenia, a rare bleeding disorder, involves regular platelet count fluctuations. This study links it for the first time to a T-large granular lymphocyte (LGL) lymphoproliferative disorder.
Area of Science:
- Hematology
- Immunology
- Oncology
Background:
- Cyclic thrombocytopenia is a rare disorder with cyclical drops in platelet count, leading to bleeding.
- The underlying cause of most cyclic thrombocytopenia cases remains unknown.
Observation:
- A patient presented with platelet count cycling between <10x10(9)/L and >1300x10(9)/L every 28-57 days.
- Antiplatelet antibodies peaked before platelet nadirs.
- Pathologic expansion of CD3+CD57+ large granular lymphocytes (LGLs) with T-cell receptor (TCR) gamma gene rearrangement was detected.
Findings:
- This is the first reported case of cyclic thrombocytopenia associated with a T-large granular lymphocyte (LGL) lymphoproliferative disorder.
- Cyclosporine-A (CsA) treatment reduced LGLs but did not alter the platelet count cycling pattern.
Implications:
- This association suggests a potential link between T-LGL lymphoproliferative disorders and cyclic thrombocytopenia.
- Further research is needed to elucidate the mechanisms connecting T-LGL disorders and platelet count dysregulation.
- Understanding this link may offer new diagnostic or therapeutic avenues for patients with unexplained cyclic thrombocytopenia.