Case of toxic epidermal necrolysis occurring after bone marrow transplantation accompanied by engraftment failure. 2019

Chika Asai, and Yuko Watanabe, and Junko Mukaijo, and Naoko Takamura, and Tomoko Okawa, and Yukie Yamaguchi, and Ayako Matsumura, and Kenji Matsumoto, and Michiko Aihara
Department of Environmental Immuno-Dermatology, Yokohama City University School of Medicine, Yokohama, Japan.

Toxic epidermal necrolysis (TEN) is a rare condition, causing life-threatening adverse cutaneous reactions. TEN occurrence after bone marrow transplantation (BMT) is a well-known phenomenon; however, to date, only a few cases have been reported in the published work. Here, we describe the case of a 53-year-old woman who experienced TEN after undergoing allogenic BMT for malignant lymphoma. Skin erosion spread across a maximum of 70% of the body surface area and severe mucosal lesions developed. Steroid pulse therapy, plasma apheresis and immunoglobulin therapy were administrated, which resulted in the complete resolution of TEN. However, she developed hemophagocytic lymphohistiocytosis and died 38 days after BMT, owing to rupture of the lower digestive tract complicated by multi-organ failure. In our case, engraftment failure occurred, and the peripheral white blood cell count was less than 100/μL during the TEN course, suggesting that the presence of only a few immune cells could cause TEN. Our findings showed that high mortality rates and widespread skin erosion could be regarded as the most important characteristics of TEN occurring after BMT.

UI MeSH Term Description Entries
D007410 Intestinal Diseases Pathological processes in any segment of the INTESTINE from DUODENUM to RECTUM. Disease, Intestinal,Diseases, Intestinal,Intestinal Disease
D008223 Lymphoma A general term for various neoplastic diseases of the lymphoid tissue. Germinoblastoma,Lymphoma, Malignant,Reticulolymphosarcoma,Sarcoma, Germinoblastic,Germinoblastic Sarcoma,Germinoblastic Sarcomas,Germinoblastomas,Lymphomas,Lymphomas, Malignant,Malignant Lymphoma,Malignant Lymphomas,Reticulolymphosarcomas,Sarcomas, Germinoblastic
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009102 Multiple Organ Failure A progressive condition usually characterized by combined failure of several organs such as the lungs, liver, kidney, along with some clotting mechanisms, usually postinjury or postoperative. MODS,Multiple Organ Dysfunction Syndrome,Organ Dysfunction Syndrome, Multiple,Organ Failure, Multiple,Failure, Multiple Organ,Multiple Organ Failures
D005260 Female Females
D006084 Graft Rejection An immune response with both cellular and humoral components, directed against an allogeneic transplant, whose tissue antigens are not compatible with those of the recipient. Transplant Rejection,Rejection, Transplant,Transplantation Rejection,Graft Rejections,Rejection, Graft,Rejection, Transplantation,Rejections, Graft,Rejections, Transplant,Rejections, Transplantation,Transplant Rejections,Transplantation Rejections
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012422 Rupture, Spontaneous Tear or break of an organ, vessel or other soft part of the body, occurring in the absence of external force. Ruptures, Spontaneous,Spontaneous Rupture,Spontaneous Ruptures
D013262 Stevens-Johnson Syndrome Rare cutaneous eruption characterized by extensive KERATINOCYTE apoptosis resulting in skin detachment with mucosal involvement. It is often provoked by the use of drugs (e.g., antibiotics and anticonvulsants) or associated with PNEUMONIA, MYCOPLASMA. It is considered a continuum of Toxic Epidermal Necrolysis. Drug-Induced Stevens Johnson Syndrome,Drug-Induced Stevens-Johnson Syndrome,Epidermal Necrolysis, Toxic,Lyell's Syndrome,Mycoplasma-Induced Stevens Johnson Syndrome,Mycoplasma-Induced Stevens-Johnson Syndrome,Nonstaphylococcal Scalded Skin Syndrome,Scalded Skin Syndrome, Nonstaphylococcal,Stevens Johnson Syndrome Toxic Epidermal Necrolysis,Stevens Johnson Syndrome Toxic Epidermal Necrolysis Spectrum,Stevens-Johnson Syndrome Toxic Epidermal Necrolysis,Stevens-Johnson Syndrome Toxic Epidermal Necrolysis Spectrum,Toxic Epidermal Necrolysis,Toxic Epidermal Necrolysis Stevens Johnson Syndrome,Toxic Epidermal Necrolysis Stevens Johnson Syndrome Spectrum,Toxic Epidermal Necrolysis Stevens-Johnson Syndrome,Toxic Epidermal Necrolysis Stevens-Johnson Syndrome Spectrum,Drug Induced Stevens Johnson Syndrome,Drug-Induced Stevens-Johnson Syndromes,Epidermal Necrolyses, Toxic,Lyell Syndrome,Lyell's Syndromes,Mycoplasma Induced Stevens Johnson Syndrome,Necrolyses, Toxic Epidermal,Necrolysis, Toxic Epidermal,Stevens Johnson Syndrome,Stevens-Johnson Syndrome, Drug-Induced,Stevens-Johnson Syndrome, Mycoplasma-Induced,Stevens-Johnson Syndromes, Drug-Induced,Syndrome, Lyell's,Syndrome, Mycoplasma-Induced Stevens-Johnson,Syndromes, Lyell's,Toxic Epidermal Necrolyses
D016026 Bone Marrow Transplantation The transference of BONE MARROW from one human or animal to another for a variety of purposes including HEMATOPOIETIC STEM CELL TRANSPLANTATION or MESENCHYMAL STEM CELL TRANSPLANTATION. Bone Marrow Cell Transplantation,Grafting, Bone Marrow,Transplantation, Bone Marrow,Transplantation, Bone Marrow Cell,Bone Marrow Grafting

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