Frostbite and Cold Agglutinin Disease: Coexistence of Two Entities Leading to Poor Clinical Outcomes
An 83-year-old woman was admitted to the emergency department for a 7-day history of fatigue and progressive cyanosis in the feet and hands after cold exposure despite physical protective measures. Upon arrival, the patient presented with necrotic cutaneous lesions in both hands and distal lower ext...
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doaj-25ab06ce96de4fb7af551b566b322c7f2021-06-30T23:37:15ZengMDPI AGMedicina1010-660X1648-91442021-06-015759259210.3390/medicina57060592Frostbite and Cold Agglutinin Disease: Coexistence of Two Entities Leading to Poor Clinical OutcomesNoel Lorenzo-Villalba0Emmanuel Andres1Javier Guerrero-Niño2Edward Nasco3Jessy Cattelan4Xavier Jannot5Marie-Pierre Ledoux6Service de Médecine Interne, Diabète et Maladies Métaboliques, Hôpitaux Universitaires de Strasbourg, 67000 Strasbourg, FranceService de Médecine Interne, Diabète et Maladies Métaboliques, Hôpitaux Universitaires de Strasbourg, 67000 Strasbourg, FranceService des Urgences, Hôpitaux Universitaires de Strasbourg, 67000 Strasbourg, FranceService de Médecine Interne, Diabète et Maladies Métaboliques, Hôpitaux Universitaires de Strasbourg, 67000 Strasbourg, FranceService de Médecine Interne, Diabète et Maladies Métaboliques, Hôpitaux Universitaires de Strasbourg, 67000 Strasbourg, FranceService de Médecine Interne, Diabète et Maladies Métaboliques, Hôpitaux Universitaires de Strasbourg, 67000 Strasbourg, FranceService d’Hématologie, Hôpitaux Universitaires de Strasbourg, 67000 Strasbourg, FranceAn 83-year-old woman was admitted to the emergency department for a 7-day history of fatigue and progressive cyanosis in the feet and hands after cold exposure despite physical protective measures. Upon arrival, the patient presented with necrotic cutaneous lesions in both hands and distal lower extremities. Upon admission, hemoglobin was 7.6 g/dL and laboratory tests were consistent with cold agglutinin disease (CAD), the presence of monoclonal IgM, and flow cytometry consistent with lymphoplasmacytic lymphoma, but MYD88 L265P mutation was negative. The patient required blood transfusion, resulting in stabilized hemoglobin and a decrease in markers of hemolysis. Treatment with aspirin 250 mg daily and intravenous iloprost 0.5 mL/h was initiated with a poor clinical response at day 4. Amputation was required. Plasma exchange was performed and chemotherapy with rituximab and bendamustine was initiated. The clinical course was marked by further necrosis, prompting discussions regarding an additional amputation that was not performed considering the high surgical risk and refusal by the patient. Supportive treatment was initiated, and the patient expired one month after hospital admission.https://www.mdpi.com/1648-9144/57/6/592frostbitecold agglutinin diseaseamputation |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Noel Lorenzo-Villalba Emmanuel Andres Javier Guerrero-Niño Edward Nasco Jessy Cattelan Xavier Jannot Marie-Pierre Ledoux |
spellingShingle |
Noel Lorenzo-Villalba Emmanuel Andres Javier Guerrero-Niño Edward Nasco Jessy Cattelan Xavier Jannot Marie-Pierre Ledoux Frostbite and Cold Agglutinin Disease: Coexistence of Two Entities Leading to Poor Clinical Outcomes Medicina frostbite cold agglutinin disease amputation |
author_facet |
Noel Lorenzo-Villalba Emmanuel Andres Javier Guerrero-Niño Edward Nasco Jessy Cattelan Xavier Jannot Marie-Pierre Ledoux |
author_sort |
Noel Lorenzo-Villalba |
title |
Frostbite and Cold Agglutinin Disease: Coexistence of Two Entities Leading to Poor Clinical Outcomes |
title_short |
Frostbite and Cold Agglutinin Disease: Coexistence of Two Entities Leading to Poor Clinical Outcomes |
title_full |
Frostbite and Cold Agglutinin Disease: Coexistence of Two Entities Leading to Poor Clinical Outcomes |
title_fullStr |
Frostbite and Cold Agglutinin Disease: Coexistence of Two Entities Leading to Poor Clinical Outcomes |
title_full_unstemmed |
Frostbite and Cold Agglutinin Disease: Coexistence of Two Entities Leading to Poor Clinical Outcomes |
title_sort |
frostbite and cold agglutinin disease: coexistence of two entities leading to poor clinical outcomes |
publisher |
MDPI AG |
series |
Medicina |
issn |
1010-660X 1648-9144 |
publishDate |
2021-06-01 |
description |
An 83-year-old woman was admitted to the emergency department for a 7-day history of fatigue and progressive cyanosis in the feet and hands after cold exposure despite physical protective measures. Upon arrival, the patient presented with necrotic cutaneous lesions in both hands and distal lower extremities. Upon admission, hemoglobin was 7.6 g/dL and laboratory tests were consistent with cold agglutinin disease (CAD), the presence of monoclonal IgM, and flow cytometry consistent with lymphoplasmacytic lymphoma, but MYD88 L265P mutation was negative. The patient required blood transfusion, resulting in stabilized hemoglobin and a decrease in markers of hemolysis. Treatment with aspirin 250 mg daily and intravenous iloprost 0.5 mL/h was initiated with a poor clinical response at day 4. Amputation was required. Plasma exchange was performed and chemotherapy with rituximab and bendamustine was initiated. The clinical course was marked by further necrosis, prompting discussions regarding an additional amputation that was not performed considering the high surgical risk and refusal by the patient. Supportive treatment was initiated, and the patient expired one month after hospital admission. |
topic |
frostbite cold agglutinin disease amputation |
url |
https://www.mdpi.com/1648-9144/57/6/592 |
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