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Thrombotic thrombocytopenic purpura: Description and analysis of 23 cases treated in Chile between 2017 and 2022

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Proposed clinical outcomes in PTT.
Clinical responsePlatelet count ≥ 150 000 and LDH < 1.5 times the LSN, sustained (e.g., at least 2 consecutive days) and without evidence of new or progressive ischemic organ damage.
Referral (whichever occurs first)
Clinical remissionSustained clinical response after discontinuation of PEX for at least 30 days. It is usually associated at least with a stabilization of the organic damage produced by the disease.
LaboratoryADAMTS13 demonstration ≥ 20%. May be considered partial (≥ 20% but less than LSN) or total (> LSN) remission.
ExacerbationPlatelet count below 150,000 (excluding other causes of thrombocytopenia), within 30 days of clinical response (i.e. before achieving remission criteria), with or without evidence of new organ damage.
Recurrence or relapse
ClinicalThere is a drop in platelet count below 150,000, regardless of whether organ damage is present, after achieving remission, whether clinical or laboratory.
LaboratoryDrop in ADAMTS13 activity below 20%, after having achieved remission, either clinical or laboratory.
Refractory diseasePersistent thrombocytopenia < 50 000 or lack of sustained increase in platelet count, with LDH > 1.5 times the LSN, despite 5 PEX and corticosteroid treatment. In addition, it is defined as severe if platelets are < 30 000.

LDH, lactate dehydrogenase; PEX, plasma exchange. TTP, thrombotic thrombocytopenic purpura.UNL, upper normal limit.

Links currently accepted in the literature.

Source: adapted from Scully et. al [10].