Acquired platelet dysfunction and overproduction of platelet cyclic AMP in two patients with myeloid malignancies
The pathophysiology of impaired platelet function in acquired disorders is often poorly understood. We report two unrelated patients with hematologic malignancies associated with acquired severe bleeding diathesis, and complex platelet function abnormalities, including overproduction of the physiolo...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Taylor & Francis Group
2019-11-01
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| Series: | Platelets |
| Subjects: | |
| Online Access: | http://dx.doi.org/10.1080/09537104.2019.1636020 |
| _version_ | 1797684192866205696 |
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| author | Anna Lecchi Eti A. Femia Silvia La Marca Francesco Onida Andrea Artoni |
| author_facet | Anna Lecchi Eti A. Femia Silvia La Marca Francesco Onida Andrea Artoni |
| author_sort | Anna Lecchi |
| collection | DOAJ |
| description | The pathophysiology of impaired platelet function in acquired disorders is often poorly understood. We report two unrelated patients with hematologic malignancies associated with acquired severe bleeding diathesis, and complex platelet function abnormalities, including overproduction of the physiological inhibitor cyclic-AMP (cAMP). Patient 1, with mild macrocytic anemia and thrombocytopenia (100 x 109/L), was diagnosed with chronic myelomonocytic leukemia a few months after the onset of her bleeding diathesis and our analysis of platelet function. Patient 2, with bleeding diathesis of recent onset, was studied when his myelodysplastic syndrome with excess blasts had already progressed to acute myeloid leukemia. In both patients, platelet aggregation/ATP secretion, serum thromboxane B2, intraplatelet content of ADP, ATP, serotonin, and fibrinogen were severely impaired. Baseline platelet cAMP levels were mildly elevated and markedly increased after stimulation by prostaglandin E1. In conclusion, these are the first patients with myeloid malignancies associated with acquired severe platelet dysfunction and overproduction of cAMP. |
| first_indexed | 2024-03-12T00:26:56Z |
| format | Article |
| id | doaj.art-3c3f4dbdb75e485896618f6e56c3db90 |
| institution | Directory Open Access Journal |
| issn | 0953-7104 1369-1635 |
| language | English |
| last_indexed | 2024-03-12T00:26:56Z |
| publishDate | 2019-11-01 |
| publisher | Taylor & Francis Group |
| record_format | Article |
| series | Platelets |
| spelling | doaj.art-3c3f4dbdb75e485896618f6e56c3db902023-09-15T10:32:01ZengTaylor & Francis GroupPlatelets0953-71041369-16352019-11-013081053105610.1080/09537104.2019.16360201636020Acquired platelet dysfunction and overproduction of platelet cyclic AMP in two patients with myeloid malignanciesAnna Lecchi0Eti A. Femia1Silvia La Marca2Francesco Onida3Andrea Artoni4Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis CenterFondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis CenterFondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis CenterFondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Università degli Studi di Milano, Department of Oncology and Hemato-Oncology, BMT CenterFondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis CenterThe pathophysiology of impaired platelet function in acquired disorders is often poorly understood. We report two unrelated patients with hematologic malignancies associated with acquired severe bleeding diathesis, and complex platelet function abnormalities, including overproduction of the physiological inhibitor cyclic-AMP (cAMP). Patient 1, with mild macrocytic anemia and thrombocytopenia (100 x 109/L), was diagnosed with chronic myelomonocytic leukemia a few months after the onset of her bleeding diathesis and our analysis of platelet function. Patient 2, with bleeding diathesis of recent onset, was studied when his myelodysplastic syndrome with excess blasts had already progressed to acute myeloid leukemia. In both patients, platelet aggregation/ATP secretion, serum thromboxane B2, intraplatelet content of ADP, ATP, serotonin, and fibrinogen were severely impaired. Baseline platelet cAMP levels were mildly elevated and markedly increased after stimulation by prostaglandin E1. In conclusion, these are the first patients with myeloid malignancies associated with acquired severe platelet dysfunction and overproduction of cAMP.http://dx.doi.org/10.1080/09537104.2019.1636020blood platelet disorderscyclic ampmyelodysplastic syndrome (mds)myeloid leukemiaplatelet function tests |
| spellingShingle | Anna Lecchi Eti A. Femia Silvia La Marca Francesco Onida Andrea Artoni Acquired platelet dysfunction and overproduction of platelet cyclic AMP in two patients with myeloid malignancies Platelets blood platelet disorders cyclic amp myelodysplastic syndrome (mds) myeloid leukemia platelet function tests |
| title | Acquired platelet dysfunction and overproduction of platelet cyclic AMP in two patients with myeloid malignancies |
| title_full | Acquired platelet dysfunction and overproduction of platelet cyclic AMP in two patients with myeloid malignancies |
| title_fullStr | Acquired platelet dysfunction and overproduction of platelet cyclic AMP in two patients with myeloid malignancies |
| title_full_unstemmed | Acquired platelet dysfunction and overproduction of platelet cyclic AMP in two patients with myeloid malignancies |
| title_short | Acquired platelet dysfunction and overproduction of platelet cyclic AMP in two patients with myeloid malignancies |
| title_sort | acquired platelet dysfunction and overproduction of platelet cyclic amp in two patients with myeloid malignancies |
| topic | blood platelet disorders cyclic amp myelodysplastic syndrome (mds) myeloid leukemia platelet function tests |
| url | http://dx.doi.org/10.1080/09537104.2019.1636020 |
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