Adult‐onset cyclic bicytopenia: A case report and review of treatment of cyclic hematopoiesis

Tefferi, Ayalew; Solberg, Lawrence A.; Petitt, Robert M.; Willis, Larry

doi:10.1002/ajh.2830300311

Cited by 15 publications

(5 citation statements)

References 44 publications

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“…Some authors suggested that periodic failure of effective platelet production might be important in the pathogenesis of the disorder (Lewis, 1974;Cohen & Cooney. 1974: Tefferi et al, 1989.…”

Section: Discussionmentioning

confidence: 99%

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Cell‐mediated cyclic thrombocytopenia treated with azathioprine

Dan

Inokuchi

et al. 1991

Br J Haematol

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We studied a male patient with cyclic thrombocytopenia whose bone marrow megakaryocyte count showed cyclic fluctuations in synchrony with cyclic changes of platelet count. He failed to respond to either prednisolone or bolus methylprednisolone therapy, but subsequently he was successfully treated with azathioprine. To investigate the underlying pathogenesis of the cyclic fluctuations in the platelet count, we studied the kinetics of megakaryocyte progenitor cells (CFU-Meg) and the effects of the patient's peripheral blood mononuclear cells on CFU-Meg. In one cycle of the platelet fluctuation, the increase in the CFU-Meg number preceded an increase in the bone marrow megakaryocyte count and then the platelet count. In the latter half of the cycle, CFU-Meg, bone marrow megakaryocytes and platelets began to decrease in that order. Peripheral blood mononuclear cells obtained from the patient in the thrombocytopenic phase suppressed megakaryocyte colony formation from normal bone marrow cells in a dose-dependent manner. In contrast, these cells obtained in the phase of a normal platelet count did not suppress megakaryocyte colony formation at all. These findings indicate that the cause of platelet fluctuation is periodic failure of megakaryocytopoiesis at the stage of CFU-Meg and that the patient's peripheral mononuclear cells are responsible for periodically suppressing the CFU-Meg.

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Section: Discussionmentioning

confidence: 99%

“…1966: Wilkinson & Firkin, 1966Bernard & Caen, 1962: Wasastjerna. 1967: Brey et al, 1969Lewis, 1974;Cohen & Cooney, 1974;Aranda & Dorantes, 1977;Wahlberg et al 1977;Menitove et al, 1989: Telek et al, 1989: Tefferi et al 1989Tomer et al, 1989: AI-Hashid, 1989. but the aetiology of these cyclic fluctuations in the platelet count has remained elusive.…”

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confidence: 99%

Cell‐mediated cyclic thrombocytopenia treated with azathioprine

Dan

Inokuchi

et al. 1991

Br J Haematol

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“…Unprovoked periodic oscillations of the platelet count have been reported in both the absence 7 and the presence of chronic myeloid disorders, including PV. 8,9 The periods of cycling in these patients varied from 27 to 66 days.…”

Section: Resultsmentioning

confidence: 99%

Hydroxyurea-induced marked oscillations of platelet counts in patients with polycythemia vera

Tefferi

Elliott

Kao

et al. 2000

Blood

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Two prospectively studied patients with polycythemia vera (PV) whose platelet counts showed marked periodic fluctuation during treatment with hydroxyurea (HU) are reported. Cycle lengths in both were approximately 28 to 30 days. In one patient, the cyclic process was no longer evident when treatment with HU was withheld, and it reappeared on treatment rechallenge. Circulating thrombopoietin (TPO) levels fluctuated out of phase with the platelet count despite markedly reduced TPO-receptor (c-Mpl) expression in bone marrow megakaryocytes. These observations suggest that the cyclic phenomenon may be related to both a transient state of HU-induced depletion of megakaryocytes and a concentration-dependent mitigation by TPO of the HU effect on megakaryocytes and their precursors. It is conceivable that the affected patients harbor a megakaryocyte progenitor pool whose apoptotic activity is differently modulated by either HU or high concentrations of TPO.

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“…Hematopoietic stimulators include corticosteroids (Rodgers and Shuman 1982), androgen (Roozendaal et al 1981) and colony stimulating factor (Hammond et al 1989), and immunomudulators include corticosteroid (Rodgers and Shuman 1982) lithium (Verma et al 1982) and cyclosporin A (Selleri et al 1988). The effects of these treatments, however, are not consistent and several serious adverse reactions have been described (Lange 1983;Tefferi et al 1989).…”

Section: Discussionmentioning

confidence: 99%