Clinical and laboratory characteristics of cyclic thrombocytopenia: an observational study

Steinbrecher, Oskar; Mitrović, Mirjana; Eischer, Lisbeth; Šinkovec, Hana; Eichinger, Sabine; Kyrle, Paul A.

doi:10.3324/haematol.2019.237909

Cited by 12 publications

(47 citation statements)

References 10 publications

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“…Laboratory testing revealed both a T-cell receptor rearrangement as well as antiplatelet antibodies and she had hypothyroidism. 15 Associated with other disorders Eight of our 9 CTP patients, all females, had a disorder of the thyroid gland. 15 Thyrotoxicosis and chronic thyroiditis were also found in one patient with CTP each by Brey and coworkers and by Yujiri and coworkers.…”

Section: Menstrual Cycle-synchronizedmentioning

confidence: 93%

“…15 Associated with other disorders Eight of our 9 CTP patients, all females, had a disorder of the thyroid gland. 15 Thyrotoxicosis and chronic thyroiditis were also found in one patient with CTP each by Brey and coworkers and by Yujiri and coworkers. 2,36 The mechanisms behind this striking association remain obscure.…”

Section: Menstrual Cycle-synchronizedmentioning

confidence: 93%

“…How I manage cyclic thrombocytopenia (Auto)immune mediated platelet destruction Presence of antiplatelet antibodies has been described in a relatively large number of CTP patients. [2][3][4][5][6][7][8][9][10][11][12][13][14][15] In a patient from Japan, for instance, an inverse relationship between platelet count and levels of immunoglobulin (Ig) G antibodies directed against glycoprotein IIIa was detected in the absence of any bone marrow abnormalities. 7 Menitove and coworkers reported 2 patients in whom platelet-associated IgG varied inversely with the platelet count.…”

Section: Pathogenesismentioning

confidence: 99%

“…24 We later identified a clonal T-cell receptor rearrangement in 5 additional CTP patients. 15 Fogarty and coworkers described a patient with T-cell large granular lymphocytic leukemia (T-LGL) who had a Tcell receptor rearrangement and, in addition, antiplatelet antibodies which were highest just prior to the platelet nadir. 14…”

Section: T-cell Receptor Rearrangementmentioning

confidence: 99%

“…In 2 of our patients, platelet counts were inversely correlated with thrombopoietin (TPO) levels suggesting defective regulation of platelet production, an observation also made by several other investigators. 15 In a patient with CTP and inversely fluctuating TPO levels, Zhang and coworkers identified a heterozygous MPL c.1210G>A mutation, which lead to a p.Gly404Arg substitution. The mutant failed to support TPO-stimulated growth of Ba/F3 cells that express wild-type MPL and otherwise exhibit Il-3 independent growth in response to…”

Section: Abnormal Regulation Of Platelet Productionmentioning

confidence: 99%

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How I manage cyclic thrombocytopenia

Kyrle

Eichinger

2021

Blood

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Cyclic thrombocytopenia (CTP) is a rare disease, which is characterized by periodic fluctuation of the platelet count. The pathogenesis of CTP is unknown and most likely heterogeneous. Patients with CTP are almost always misdiagnosed as having primary immune thrombocytopenia (ITP). The interval between ITP and CTP diagnosis can be many years. CTP patients often receive ITP-specific therapies including corticosteroids, thrombopoietin receptor agonists, rituximab and splenectomy which are followed by a transient increase in platelet count that is wrongly attributed to treatment effect with inevitable "relapse". CTP can be diagnosed by frequent platelet count monitoring which reveals a typical pattern of periodic platelet cycling. An early diagnosis of CTP will prevent these patients from being exposed to possibly harmful therapies. The bleeding phenotype is usually mild and consists of mucocutaneous bleeding at the time when the platelet count is at its nadir. Severe bleeding from other sites can occur but is rare. Some patients respond to cyclosporine A or to danazol, but most patients do not respond to any therapy. CTP can be associated with hematological malignancies or disorders of the thyroid gland. Nevertheless, spontaneous remissions can occur, even after many years.

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Section: Menstrual Cycle-synchronizedmentioning

confidence: 93%

Section: Menstrual Cycle-synchronizedmentioning

confidence: 93%

Section: Pathogenesismentioning

confidence: 99%

Section: T-cell Receptor Rearrangementmentioning

confidence: 99%

Section: Abnormal Regulation Of Platelet Productionmentioning

confidence: 99%

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How I manage cyclic thrombocytopenia

Kyrle

Eichinger

2021

Blood

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Disorders of red cells and platelets

2021

Blood Cells

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The highs and lows of cyclic thrombocytopenia

Zhang,

Villar‐Prados,

Bussel

et al. 2023

Br J Haematol

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SummaryCyclic thrombocytopenia (CTP) is characterized by periodic platelet oscillation with substantial amplitude. Most CTP cases have a thrombocytopenic background and are often misdiagnosed as immune thrombocytopenia with erratically effective treatment choices. CTP also occurs during hydroxyurea treatment in patients with myeloproliferative diseases. While the aetiology of CTP remains uncertain, here we evaluate historical, theoretical and clinical findings to provide a framework for understanding CTP pathophysiology. CTP retains the intrinsic oscillatory factors defined by the homeostatic regulation of platelet count, presenting as reciprocal platelet/thrombopoietin oscillations and stable oscillation periodicity. Moreover, CTP patients possess pathogenic factors destabilizing the platelet homeostatic system thereby creating opportunities for external perturbations to initiate and sustain the exaggerated platelet oscillations. Beyond humoral and cell‐mediated autoimmunity, we propose recently uncovered germline and somatic genetic variants, such as those of MPL, STAT3 or DNMT3A, as pathogenic factors in thrombocytopenia‐related CTP. Likewise, the JAK2 V617F or BCR::ABL1 translocation that drives underlying myeloproliferative diseases may also play a pathogenic role in hydroxyurea‐induced CTP, where hydroxyurea treatment can serve as both a trigger and a pathogenic factor of platelet oscillation. Elucidating the pathogenic landscape of CTP provides an opportunity for targeted therapeutic approaches in the future.

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Clinical and laboratory characteristics of cyclic thrombocytopenia: an observational study

Abstract: Cyclic thrombocytopenia (CTP) is a rare disease with less than 70 reported cases. The hallmark of CTP is a periodic fluctuation of the platelet count. The severity of thrombocytopenia and the duration of cycles may vary.

Cited by 12 publications

References 10 publications

How I manage cyclic thrombocytopenia

How I manage cyclic thrombocytopenia

Disorders of red cells and platelets

The highs and lows of cyclic thrombocytopenia

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