At the point when to associate a conclusion with intrinsic acquired thrombocytopenia CTP Or when low platelet level doe.

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When to suspect a diagnosis of congenital inherited thrombocytopenia (CTP) ? Or when low platelet level does not mean immune thrombocytopenic purpura (ITP). Pr JF Viallard Hôpital Haut-Lévêque, CHU BORDEAUX FRANCE. Chronic thrombocytopenia in adult diagnostic procedure.
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At the point when to associate a finding with intrinsic acquired thrombocytopenia (CTP) ? Then again when low platelet level does not mean invulnerable thrombocytopenic purpura (ITP). Pr JF Viallard Hôpital Haut-Lévêque, CHU BORDEAUX FRANCE

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Chronic thrombocytopenia in grown-up demonstrative system Pseudothrombocytopenia Bone marrow examination HIV, B-or C-hepatitis Antinuclear antibodies, antiphospholipid antibodies,… Coagulation tests Schistocytes hypogammaglobulinemia Spleen echography

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ITP or CTP? ITP: determination of avoidance various CTP had been misdiagnosed as ITP and the patients subjected to spleenectomy or potentially cyclophosphamide, among other unappropriate treatments Which patients must be investigated?

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FOUR POINTS OF TRIAGE TO SUSPECT CONGENITAL THROMBOCYTOPENIA (CTP) A family history of "ITP" Absence of an expansion in the platelet tally because of ITP medications (recalcitrant ITP) Presence of certain "associated features" proposing particular judgments, for instance thrombocytopenia with truant radii Platelet measure assessed on spread (blood smears are generally accessible)

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Reasons to presume CTP: family history Especially > 2 relatives Especially parent-kid or maternal uncle-nephew Non-particular criteria Cases of familial ITP (immune system illnesses)

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Reasons to speculate CTP: non-reaction to ITP treatment Lack of platelet reaction to established immune system thrombocytopenia treatments including IVIG, steroids, and spleenectomy Non-particular criteria: obstinate ITP No correct meaning of absence of reaction; no very much characterized reaction limits Arbitrarily: > 30,000/µl increment from standard: ITP < 10,000/µl increment is good with CTP, however "refractory" ITP is conceivable.

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Reasons to speculate CTP: non-reaction to ITP treatment Possible immunological segment to the CTP Impeding the freedom instrument for deviant platelets balances disabled platelet creation. Since "spontaneous" change in the platelet tally may happen (for instance as a consequence of a viral disease), the evaluation of two treatment reactions is most likely more supportive as an indicative model.

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Reasons to presume CTP: related elements In the patient or in a relative Absence of radii ( ± other orthopedic distortions) is suggestive of a TAR disorder, Severe thrombocytopenia in the principal year of existence with decreased megakaryocytes Severe bleedings their platelet numbers increment with time the platelets may fall again amid adulthood Signaling by means of the TPO receptor is irregular

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Reasons to associate CTP: related components Features with velocardiofacial (VCF) disorder and DiGeorge disorder : T-cell imperfection (yet factor clinical immunodeficiency) Rightsided coronary illness Neonatal hypocalcemia Cleft sense of taste or bifid uvula Neuro-psychologic issues Acronym "CATCH22" ( c ardiac variation from the norm, T - cell shortfall, c left sense of taste, h ypocalcemia because of Chr 22 erasure).

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Reasons to speculate CTP: related components Patients with either Wiskott-Aldrich disorder (WAS) or the XLT type of WAS: Marked or serious thrombocytopenia and littler than ordinary platelets. Extreme diseases (preference to pneumococcal sepsis) Eczema is normal Very youthful babies: drain hypersensitivity, and hematochezia Frequent contaminations may cover with safe thrombocytopenias auxiliary to hypogammaglobulinemia!

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Thrombocytopenia + substantial/goliath platelets bleeding +/ - Fechtner Syndrome (1985) : +leuko. Considerations, nephritis, deafness, waterfall Epstein Syndrome (1972): + nephritis, deafness Sebastian Syndrome (1990) : + leuko. incorporations May-Hegglin Anomaly : (1909; 1945) + leuko. incorporations Alport-like disorder (1986): + nephritis, deafness, waterfall Molecular premise : MYH9 transformations (Kelley 2000, Heath 2001, Seri 2002)

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Reasons to associate CTP: audit with the spread Visual investigation of the spread remains the "gold standard" for platelet estimate in clinical practice Large platelets: Bernard-Soulier disorder or MYH9 surrenders. Small platelets: reliable with Wiskott-Aldrich Platelet bunching may recommend von Willebrand sort IIb

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Mutations MYH9 Döhle-like bodies (light-blue leukocytes considerations) MHA FTNS MGG IC

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Other motivations to associate innate thrombocytopenia Bleeding out with extent to the platelet tally Onset during childbirth Persistence of a steady level of thrombocytopenia for quite a long time Normal life expectancy of platelets dictated by isotopic platelet life traverse contemplate notwithstanding low platelet level

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PERSPECTIVES Certain patients don\'t fit into any of the known issue Refer to a pro in acquired platelet issue: Number and morphology of megakaryocytes MK settlements examines MK societies Platelet capacity and conglomeration thinks about with high platelet fixations Plasma TPO, glycocalicin levels New treatments: TPO

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Acknowledgments A Nurden and P Nurden, Center de Référence des pathologies plaquettaires rares, Hôpital Haut-Lévêque, BORDEAUX

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