Cyclic Idiopathic Pure Acquired Amegakaryocytic Thrombocytopenic Purpura: A Patient Treated With Cyclosporin A

Telek, B; Kiss, Andrea; Pecze, K; Ujhelyi, P; Rák, K.

doi:10.1111/j.1365-2141.1989.tb00232.x

Cited by 22 publications

(19 citation statements)

References 7 publications

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“…CsA, which is used to treat LGL lymphoproliferative disorders [6] and has decreased the severity of platelet count nadir in some patients with cyclic thrombocytopenia [1,5,8], was ineffective. Because a dramatic reduction in LGLs in the peripheral blood was observed in this case following use of CsA, is not known what degree of interruption of the lymphoproliferative process (if any) would be required to achieve clinical remission of platelet count fluctuations.…”

Section: Discussionmentioning

confidence: 99%

Large granular lymphocytic proliferation-associated cyclic thrombocytopenia

et al. 2005

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Cyclic thrombocytopenia is a rare condition characterized by regular fluctuations in the platelet count, resulting in bleeding at the time of platelet count nadir. We evaluated a male patient whose platelet count cycled between <10 · 10 9 /L and a maximum of >1300 · 10 9 /L over a median of every 42 days (range, 28-57 days). Antiplatelet antibodies were present at highest titer just prior to platelet nadirs. A pathologic expansion of CD3 + CD57 + large granular lymphocytes (LGLs) along with a clonal rearrangement of the T-cell receptor (TCR) gamma gene in circulating T cells was detected.LGLs decreased in response to treatment with cyclosporine-A (CsA), but the cycling of the platelet count continued. This is the first report of cyclic thrombocytopenia associated with a T-LGL lymphoproliferative disorder. Am.

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Section: Discussionmentioning

confidence: 99%

Large granular lymphocytic proliferation-associated cyclic thrombocytopenia

et al. 2005

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“…In cyclic AATP the bone marrow shows fluctuations in the megakaryocyte counts, which is in synchrony with the changes in the platelet counts. Antiplatelet antibodies are usually absent and the platelet life-span is normal [1][2][3][4][5][6]9]. However, only Kimura et al [7] have observed fluctuating levels of platelet-associated IgG (PA-IgG) in synchrony with the variations in platelet count in their male patient with cyclic AATP.…”

Section: Discussionmentioning

confidence: 99%

“…The fluctuations in the platelet counts can be secondary to defective platelet production (amegakaryocytic type) or increased destruction of the platelets by the reticulo-endothelial system (the megakaryocytic variety). Amegakaryocytic cyclic thrombocytopenia (cyclic AATP) is rare; seven cases have been reported in males and only three cases in females [1][2][3][4][5][6][7][8][9][10].…”

Section: Introductionmentioning

confidence: 99%

Danazol therapy in cyclic acquired amegakaryocytic thrombocytopenic purpura: A case report

Kashyap¹,

Choudhry²,

Pati³

1999

Am. J. Hematol.

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Cyclic acquired amegakaryocytic thrombocytopenic purpura (AATP) is a rare disorder characterized by periodic fluctuations in the platelet counts due to a defect in the platelet production. We describe a 42-year-old female with cyclic AATP, in whom the cyclic fluctuations in the platelet counts ceased with danazol therapy. The pathogenesis of the disease and the possible mechanisms of danazol action have been reviewed. Am. J. Hematol. 60:225-228, 1999.

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“…However, the PAIgG level was not related to the platelet count in most cases [6,12,22,23]. An autoantibody directed against either GPIIb/IIIa complex or GPIIb has been reported and was related to the cyclic fluctuations in the platelet count [13].…”

Section: Discussionmentioning

confidence: 99%

Periodic Production of Antiplatelet Autoantibody Directed against GPIIIa in Cyclic Thrombocytopenia

et al. 1993

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We report here a female patient with cyclic thrombocytopenia associated with antiplatelet autoantibodies. There was an inverse relationship between the level of platelet-associated IgG and platelet count. Bone marrow megakaryocytes were normal in number even during the thrombocytopenia. The binding of monoclonal antibodies (mAbs) against glycoprotein (GP) Ilb/IIIa to patient platelets was significantly inhibited in the thrombocytopenic phase, while these mAbs normally bound to patient platelets obtained during the normal platelet count. Western blotting and mAb-specific immobilization of platelet antigens showed that both plasma autoantibody and the eluted IgG from the patient platelets bound to GPIIIa. These results suggest that the periodic production of antiplatelet autoantibody against GPIIIa caused cyclic destruction of platelets in this patient.

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Cyclic Idiopathic Pure Acquired Amegakaryocytic Thrombocytopenic Purpura: A Patient Treated With Cyclosporin A

Cited by 22 publications

References 7 publications

Large granular lymphocytic proliferation-associated cyclic thrombocytopenia

Large granular lymphocytic proliferation-associated cyclic thrombocytopenia

Danazol therapy in cyclic acquired amegakaryocytic thrombocytopenic purpura: A case report

Periodic Production of Antiplatelet Autoantibody Directed against GPIIIa in Cyclic Thrombocytopenia

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