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Acquired amegakaryocytic thrombocytopenia associated with proliferation of γ/δ TCR + T‐lymphocytes and a BCR‐ABL (p210) fusion transcript
Author(s) -
Colovic Milica,
Pavlovic Sonja,
Kraguljac Nada,
Colovic Natasa,
Jankovic Gradimir,
Sefer Dijana,
Tosic Natasa
Publication year - 2004
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/j.1600-0609.2004.00316.x
Subject(s) - immunophenotyping , t cell receptor , bone marrow , cd8 , biology , immunology , megakaryocyte , gene rearrangement , t lymphocyte , monoclonal , microbiology and biotechnology , t cell , monoclonal antibody , progenitor cell , antigen , immune system , gene , stem cell , antibody , genetics
Acquired amegakaryocytic thrombocytopenia (AATP) in adults is a rare disorder characterized by severe thrombocytopenia and decreased or absent megakaryocytes in an otherwise normal bone marrow. We present a 44‐yr‐old man in whom the diagnosis of AATP was established in January 2001. Immunophenotyping of the peripheral blood lymphocytes showed a relative increase in the subpopulation of gamma/delta T‐cell receptor (TCR) positive ( γ / δ TCR + ) and (CD4, CD8) negative T lymphocytes, and PCR suggested a monoclonal pattern of TCR gamma chain gene rearrangement. Cytogenetic examination of his bone marrow cells showed a normal male karyotype but RT‐PCR analysis revealed a BCR‐ABL (p210) fusion transcript. The inhibition of CFU‐Mk growth mediated by the patient's T lymphocytes indicated that the pathogenic mechanism for AATP could be an immunological attack on megakaryocyte progenitors where the γ / δ TCR‐positive T lymphocytes are directly involved. The case emphasizes the complex association of T‐lymphocyte monoclonal proliferation and AATP.