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Vol. 46. Issue S6.
Pages S442-S444 (December 2024)
Vol. 46. Issue S6.
Pages S442-S444 (December 2024)
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Assessing treatment response in thrombotic thrombocytopenic purpura: Beyond the platelet count
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Cilomar Martins de Oliveira Filhoa,
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cmartins5@mgb.org

Corresponding author.
, Ibidunni Bode-Sojobib, Barbara D. Lamb, Stephanie Conradb, Jonathan Berryb, Brian J. Carneyb
a Mass General Brigham, Salem Hospital, Salem, Massachusetts, USA
b Beth Israel Deaconess Medical Center, Boston, USA
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A 50-year-old male with systemic lupus erythematous presented with fever, abdominal pain, and diarrhea. Hemoglobin was 10 g/dL, near patient's baseline, and there was new thrombocytopenia with a platelet count 109 × 109/L. The following day, platelets dropped to 24 × 109/L. Hemolysis parameters were unremarkable. Treatment for immune thrombocytopenic purpura with IV dexamethasone was started but there was no improvement in the platelet count. On hospital Day 7, he developed a seizure, and was intubated. He evolved with shock and renal failure requiring dialysis. Lactate dehydrogenase rose to 1054 U/L and haptoglobin became undetectable. A peripheral blood smear revealed a large population of schistocytes. The PLASMIC score was 6.1 ADAMTS13 assay was done, the patient received fresh frozen plasma (FFP) and was transferred to a tertiary center for daily plasmapheresis with full FFP replacement. The platelet counts initially rose, then remained at around 50 × 109/L on subsequent days. Notably, hemolysis parameters rapidly normalized. The population of schistocytes steadily decreased. Antiphospholipid antibodies and enterohemorrhagic E. coli tests were negative. On Day 5 of plasmapheresis, ADAMTS13 activity was undetectable, confirming a diagnosis of acquired thrombotic thrombocytopenic purpura. By Day 11 on plasmapheresis the patient improved consistently and was extubated (Figure 1).

Figure 1.

Days on plasmapheresis and changes in blood smears (x50), blood counts, and hemolysis parameters.

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Reference
[1]
PK Bendapudi, S Hurwitz, A Fry, et al.
Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study.
Lancet Haematol, 4 (2017), pp. e157-e164
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