Scientific letter
Caplacizumab in acquired thrombotic thrombocytopenic thrombocytopenic purpura: dose adjustment based on von Willebrand factor level
Caplacizumab en la púrpura trombocitopénica trombótica adquirida: ajuste de la dosis basado en el factor de von Willebrand
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La línea en horizontal indica la duración del tratamiento en días. E. Evolución de la actividad de ADAMTS13 (%). A. Evolución del recuento plaquetario. F. Evolución del factor de von Willebrand (FvW). B. Evolución de la lactato deshidrogenasa (LDH). C. Evolución de la hemoglobina. D. Evolución de la bilirrubina total. G. Aspectos clave y resultados clínicos del paciente. La línea en horizontal indica la duración del tratamiento en días.</p> <p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">ADAMTS13: una desintegrina y metaloproteinasa con motivos de tromboespondina-1, 13° miembro; FvW: factor de von Willebrand; IgG: inmunoglobulina G; LDH: lactato deshidrogenasa; RP: recambios plasmáticos.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Marta Albanell-Fernández, Inés Monge-Escartín, Joan Cid" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Marta" "apellidos" => "Albanell-Fernández" ] 1 => array:2 [ "nombre" => "Inés" "apellidos" => "Monge-Escartín" ] 2 => array:2 [ "nombre" => "Joan" "apellidos" => "Cid" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S2387020623003455" "doi" => "10.1016/j.medcle.2023.04.028" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020623003455?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S002577532300235X?idApp=UINPBA00004N" "url" => "/00257753/0000016100000006/v1_202309190535/S002577532300235X/v1_202309190535/es/main.assets" ] ] "itemSiguiente" => array:19 [ "pii" => "S2387020623003480" "issn" => "23870206" "doi" => "10.1016/j.medcle.2023.09.002" "estado" => "S300" "fechaPublicacion" => "2023-09-29" "aid" => "6284" "copyright" => "Elsevier España, S.L.U." 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The horizontal line indicates treatment duration in days. (E) Change in ADAMTS13 activity (%). (A) Change in platelet count. (F) Change in von Willebrand factor (vWF). (B) Changes in lactate dehydrogenase (LDH). (C) Changes in haemoglobin. (D) Trends in total bilirubin. (G) Key aspects and clinical outcome of the patient. The horizontal line indicates the duration of treatment in days.</p> <p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">ADAMTS13, a disintegrin and metalloproteinase with thrombospondin type 1 motif, 13; vWF, von Willebrand factor; IgG, immunoglobulin G; LDH,: lactate dehydrogenase; PE, plasma exchange.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare but potentially fatal disease caused by deficiency of the ADAMTS13 protease.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> Immune-mediated aTTP is caused by autoantibodies that inhibit ADAMTS13 activity, leading to platelet consumption due to the formation of von Willebrand factor (vWF)-platelet aggregates and microvascular thrombosis.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Caplacizumab was approved in conjunction with plasma exchange (PE) and immunosuppression for adults with aTTP. This humanised bivalent immunoglobulin prevents platelet-vWF interaction. The usual dose is 10 mg subcutaneously after PE, although current studies suggest adjusting the dose according to vWF.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,5</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">We report the case of a patient in whom vWF-based caplacizumab adjustment led to aTTP resolution.</p><p id="par0020" class="elsevierStylePara elsevierViewall">A 40-year-old male presented to the emergency department with choluria for 7 days, with petechiae in the limbs and no neurological involvement or signs of haemorrhage. Laboratory tests showed a platelet count of 13 × 10<span class="elsevierStyleSup">9</span>/l, elevated cardiac troponins and signs of haemolysis: undetectable haptoglobin and elevated bilirubin and lactate dehydrogenase. ADAMTS13 activity was 1% (normal >10%) and anti-ADAMTS13 antibodies: 48.16 U/mL (abnormal >12 U/mL). The patient was diagnosed with aTTP and admitted to the intensive care unit (ICU) to start caplacizumab in combination with PE and immunosuppressive therapy (methylprednisolone 1 mg/kg/24 h). In the presence of positive anti-ADAMTS13, rituximab was added. Initial vWF was 171 U/dl (normal 60−160 U/dl).</p><p id="par0025" class="elsevierStylePara elsevierViewall">The patient received caplacizumab 10 mg/24 h for 5 days; platelet count and laboratory parameters rapidly returned to normal levels (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A–D). The patient reported feeling better, however ADAMTS13 activity remained low (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>E). Based on vWF determinations (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>F) and platelet count (302 × 10<span class="elsevierStyleSup">9</span>/l) the caplacizumab dose was adjusted to 10 mg/48 h <span class="elsevierStyleItalic">(off-label)</span> on day 6 of treatment. PEs were spaced out every other day, intravenous immunoglobulins were added and methylprednisolone was reduced. After 10 days of treatment the vWF was 84.65 U/dl (in normal range), indicating a decrease in vWF activity and that caplacizumab was correctly blocking vWF from binding to platelets, but ADAMTS13 activity continued with no increase, even decreasing to 0%. However, it was decided to maintain the regimen every 48 h, as the platelet count remained in an adequate range (257 × 10<span class="elsevierStyleSup">9</span>/l). After 8 PE procedures and 14 days in the ICU, the patient was transferred to the conventional ward. The caplacizumab dose was missed one day by mistake (forgetfulness) and vWF levels increased to 189 U/dl. After 21 days of treatment ADAMTS13 activity increased to 26%, and anti-ADAMTS13 antibodies were negative, so rituximab was discontinued. The dose of methylprednisolone was changed to the equivalent of prednisone and progressively reduced. The patient had one episode of self-limiting epistaxis, an adverse effect commonly described with caplacizumab, but without requiring additional support.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">After 24 days of admission and 10 PEs (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>G) the patient was discharged. One-week later ADAMTS13 activity was 65% and platelet count 169 × 10<span class="elsevierStyleSup">9</span>/l with negative anti-ADAMTS13 antibodies, so caplacizumab was discontinued. After more than 6 months the patient remains asymptomatic and has recovered 100% of ADAMTS13 activity.</p><p id="par0035" class="elsevierStylePara elsevierViewall">The clinical trials that led to the approval of caplacizumab did not include returning to normal ADAMTS13 activity as one of the objectives to be achieved. Platelet counts normalise rapidly (median 3–5 days).<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–3</span></a> However, measurement of vWF may be useful to target treatment and achieve earlier normal ADAMTS13 activity, preventing early recurrences.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,5</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The dose of 10 mg/48 h is not included in the SmPC but has been shown by authors such as Kühne et al. to be safe and feasible in selected patients.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> In our patient, the dose was adjusted when the vWF was ±20% outside the limits of normality (60−160 U/dl). This regimen is a suitable option to maintain the beneficial effect of the drug without excess drug functionality, reducing the potential adverse effects and exacerbations caused by abrupt drug discontinuation.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,5</span></a> In addition, the costs of a drug with a high economic impact are reduced.</p><p id="par0045" class="elsevierStylePara elsevierViewall">VWF monitoring is effective in adjusting caplacizumab and restoring ADAMTS 13 activity earlier.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Ethical considerations</span><p id="par0050" class="elsevierStylePara elsevierViewall">The ethical considerations of our centre with regard to the protection of patient data have been observed.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Funding</span><p id="par0055" class="elsevierStylePara elsevierViewall">The study has not received any funding.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflict of interest</span><p id="par0060" class="elsevierStylePara elsevierViewall">Joan Cid has received research funding from Cerus, Kawasumi Laboratories and Sanofi. He has also received speaker/consultant fees from Cerus, Fresenius Kabi, Grifols, MacoPharma, Sanofi and TerumoBCT.</p><p id="par0065" class="elsevierStylePara elsevierViewall">The other authors declare that they have no conflict of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:4 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Ethical considerations" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Funding" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Conflict of interest" ] 3 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:1 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2096 "Ancho" => 3175 "Tamanyo" => 420638 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Change in main laboratory parameters and summary of key aspects and clinical outcomes of caplacizumab treatment. The horizontal line indicates treatment duration in days. (E) Change in ADAMTS13 activity (%). (A) Change in platelet count. (F) Change in von Willebrand factor (vWF). (B) Changes in lactate dehydrogenase (LDH). (C) Changes in haemoglobin. (D) Trends in total bilirubin. (G) Key aspects and clinical outcome of the patient. The horizontal line indicates the duration of treatment in days.</p> <p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">ADAMTS13, a disintegrin and metalloproteinase with thrombospondin type 1 motif, 13; vWF, von Willebrand factor; IgG, immunoglobulin G; LDH,: lactate dehydrogenase; PE, plasma exchange.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "M. Scully" 1 => "S.R. Cataland" 2 => "F. Peyvandi" 3 => "P. Coppo" 4 => "P. Knöbl" 5 => "J.A. Kremer Hovinga" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1056/NEJMoa1806311" "Revista" => array:7 [ "tituloSerie" => "N Engl J Med" "fecha" => "2019" "volumen" => "380" "numero" => "4" "paginaInicial" => "335" "paginaFinal" => "346" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/30625070" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Real-world data confirm the effectiveness of caplacizumab in acquired thrombotic thrombocytopenic purpura" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "L.A. Völker" 1 => "J. Kaufeld" 2 => "W. Miesbach" 3 => "S. Brähler" 4 => "M. Reinhardt" 5 => "L. Kühne" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1182/bloodadvances.2020001973" "Revista" => array:7 [ "tituloSerie" => "Blood Adv" "fecha" => "2020" "volumen" => "4" "numero" => "13" "paginaInicial" => "3085" "paginaFinal" => "3092" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/32634236" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0015" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Real-world data with the use of caplacizumab in the treatment of acquired thrombotic thrombocytopenic purpura: a single-center with homogeneous treatment experience" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "M. Jiménez" 1 => "S. Bobillo" 2 => "V. Pons" 3 => "C. Sánchez" 4 => "A. Pérez" 5 => "A. Molero" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/trf.17111" "Revista" => array:6 [ "tituloSerie" => "Transfusion" "fecha" => "2022" "volumen" => "62" "numero" => "11" "paginaInicial" => "2363" "paginaFinal" => "2369" ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "ADAMTS13 and VWF activities guide individualized caplacizumab treatment in patients with aTTP" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "L. Völker" 1 => "J. Kaufeld" 2 => "W. Miesbach" 3 => "S. Brähler" 4 => "M. Reinhardt" 5 => "L. Kühne" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1182/bloodadvances.2020001987" "Revista" => array:7 [ "tituloSerie" => "Blood Adv" "fecha" => "2020" "volumen" => "4" "numero" => "13" "paginaInicial" => "3093" "paginaFinal" => "3101" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/32634237" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0025" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Alternate-day dosing of caplacizumab for immune-mediated thrombotic thrombocytopenic purpura" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "L. Kühne" 1 => "J. Kaufeld" 2 => "L.A. Völker" 3 => "R. Wendt" 4 => "U. Schönermarck" 5 => "H. Hägele" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/jth.15637" "Revista" => array:7 [ "tituloSerie" => "J Thromb Haemost" "fecha" => "2022" "volumen" => "20" "numero" => "4" "paginaInicial" => "951" "paginaFinal" => "960" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/35000278" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000016100000006/v1_202309251317/S2387020623003455/v1_202309251317/en/main.assets" "Apartado" => array:4 [ "identificador" => "43311" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Scientific letters" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000016100000006/v1_202309251317/S2387020623003455/v1_202309251317/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020623003455?idApp=UINPBA00004N" ]
