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Purpura Fulminans Triggered by the Formation of Anti-Endothelial Autoantibodies in a Patient with Chronic Lymphocytic Leukemia

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Purpura Fulminans Triggered by the Formation of Anti-Endothelial Autoantibodies in a Patient with Chronic Lymphocytic Leukemia. / Beckmann, Lennart; Lennartz, Maximillian; Beitzen-Heineke, Antonia; Voigtländer, Minna; Rolling, Christina; Holstein, Katharina; Bokemeyer, Carsten; Amirkhosravi, Ali; Langer, Florian.

In: Ann Hematol Oncol, Vol. 9, No. 1, 1389, 17.03.2022.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Beckmann, L, Lennartz, M, Beitzen-Heineke, A, Voigtländer, M, Rolling, C, Holstein, K, Bokemeyer, C, Amirkhosravi, A & Langer, F 2022, 'Purpura Fulminans Triggered by the Formation of Anti-Endothelial Autoantibodies in a Patient with Chronic Lymphocytic Leukemia', Ann Hematol Oncol, vol. 9, no. 1, 1389. https://doi.org/10.26420/annhematoloncol.2022.1389

APA

Beckmann, L., Lennartz, M., Beitzen-Heineke, A., Voigtländer, M., Rolling, C., Holstein, K., Bokemeyer, C., Amirkhosravi, A., & Langer, F. (2022). Purpura Fulminans Triggered by the Formation of Anti-Endothelial Autoantibodies in a Patient with Chronic Lymphocytic Leukemia. Ann Hematol Oncol, 9(1), [1389]. https://doi.org/10.26420/annhematoloncol.2022.1389

Vancouver

Beckmann L, Lennartz M, Beitzen-Heineke A, Voigtländer M, Rolling C, Holstein K et al. Purpura Fulminans Triggered by the Formation of Anti-Endothelial Autoantibodies in a Patient with Chronic Lymphocytic Leukemia. Ann Hematol Oncol. 2022 Mar 17;9(1). 1389. https://doi.org/10.26420/annhematoloncol.2022.1389

Bibtex

@article{fc32fc2402cb427ca69c7a59686e3da4,
title = "Purpura Fulminans Triggered by the Formation of Anti-Endothelial Autoantibodies in a Patient with Chronic Lymphocytic Leukemia",
abstract = "Purpura fulminans (PF), a life-threatening disorder characterized bycutaneous microvascular thrombosis with secondary hemorrhagic infarction, isa rare complication of sepsis and may also be caused by severe protein C orprotein S deficiency.Here, we describe the case of a 58-year-old man who developed PF in closeassociation with the onset of chronic lymphocytic leukemia (CLL). PF initiallymanifested with an ecchymosis of the right upper leg in the absence of sepsisor disseminated intravascular coagulation. PF was likely triggered by antiendothelialIgG autoantibodies functionally interfering with the anticoagulantprotein C-protein S-thrombomodulin system on EA.hy926 endothelial cellsin a modified thrombin generation assay. Although plasma exchange andimmunosuppressive therapy with cyclophosphamide were temporarily effective,PF eventually progressed and the patient died from septic shock duringtreatment-associated neutropenia.In rare occasions, CLL may be associated with autoimmune-mediated PFrequiring prompt diagnosis and aggressive multimodal therapy.",
author = "Lennart Beckmann and Maximillian Lennartz and Antonia Beitzen-Heineke and Minna Voigtl{\"a}nder and Christina Rolling and Katharina Holstein and Carsten Bokemeyer and Ali Amirkhosravi and Florian Langer",
year = "2022",
month = mar,
day = "17",
doi = "10.26420/annhematoloncol.2022.1389",
language = "English",
volume = "9",
journal = "Ann Hematol Oncol",
issn = "2375-7965",
publisher = "Austin Publishing Group",
number = "1",

}

RIS

TY - JOUR

T1 - Purpura Fulminans Triggered by the Formation of Anti-Endothelial Autoantibodies in a Patient with Chronic Lymphocytic Leukemia

AU - Beckmann, Lennart

AU - Lennartz, Maximillian

AU - Beitzen-Heineke, Antonia

AU - Voigtländer, Minna

AU - Rolling, Christina

AU - Holstein, Katharina

AU - Bokemeyer, Carsten

AU - Amirkhosravi, Ali

AU - Langer, Florian

PY - 2022/3/17

Y1 - 2022/3/17

N2 - Purpura fulminans (PF), a life-threatening disorder characterized bycutaneous microvascular thrombosis with secondary hemorrhagic infarction, isa rare complication of sepsis and may also be caused by severe protein C orprotein S deficiency.Here, we describe the case of a 58-year-old man who developed PF in closeassociation with the onset of chronic lymphocytic leukemia (CLL). PF initiallymanifested with an ecchymosis of the right upper leg in the absence of sepsisor disseminated intravascular coagulation. PF was likely triggered by antiendothelialIgG autoantibodies functionally interfering with the anticoagulantprotein C-protein S-thrombomodulin system on EA.hy926 endothelial cellsin a modified thrombin generation assay. Although plasma exchange andimmunosuppressive therapy with cyclophosphamide were temporarily effective,PF eventually progressed and the patient died from septic shock duringtreatment-associated neutropenia.In rare occasions, CLL may be associated with autoimmune-mediated PFrequiring prompt diagnosis and aggressive multimodal therapy.

AB - Purpura fulminans (PF), a life-threatening disorder characterized bycutaneous microvascular thrombosis with secondary hemorrhagic infarction, isa rare complication of sepsis and may also be caused by severe protein C orprotein S deficiency.Here, we describe the case of a 58-year-old man who developed PF in closeassociation with the onset of chronic lymphocytic leukemia (CLL). PF initiallymanifested with an ecchymosis of the right upper leg in the absence of sepsisor disseminated intravascular coagulation. PF was likely triggered by antiendothelialIgG autoantibodies functionally interfering with the anticoagulantprotein C-protein S-thrombomodulin system on EA.hy926 endothelial cellsin a modified thrombin generation assay. Although plasma exchange andimmunosuppressive therapy with cyclophosphamide were temporarily effective,PF eventually progressed and the patient died from septic shock duringtreatment-associated neutropenia.In rare occasions, CLL may be associated with autoimmune-mediated PFrequiring prompt diagnosis and aggressive multimodal therapy.

U2 - 10.26420/annhematoloncol.2022.1389

DO - 10.26420/annhematoloncol.2022.1389

M3 - SCORING: Journal article

VL - 9

JO - Ann Hematol Oncol

JF - Ann Hematol Oncol

SN - 2375-7965

IS - 1

M1 - 1389

ER -