Surgical management of endometrial cancer in patient with musculocontractural Ehlers-Danlos Syndrome harboring pathogenic variants in CHST14 (mcEDS-CHST14): A case report

Article

Author: Kobara, Hisanori ; Ida, Koichi ; Shiozawa, Tanri ; Yamaguchi, Tomomi ; Shioya, Yuta ; Kosho, Tomoki ; Miyamoto, Tsutomu ; Ando, Hirofumi

IntroductionMusculocontractural Ehlers-Danlos syndrome (mcEDS) is a rare autosomal recessive connective tissue disorder caused by systemic depletion of dermatan sulfate. Symptoms characteristic of mcEDS include multiple contractures, fragile skin with subcutaneous bleeding, and hypermobile joints, which suggest difficulty in perioperative management. However, safe surgical techniques and perioperative management of this disorder remain unknown because of its rarity. We report a patient with mcEDS who developed endometrial cancer and underwent surgery, with emphasis on perioperative management.Case reportA female patient, who had been genetically diagnosed with mcEDS-CHST14 at the age of 25, presented with symptoms characteristic of mcEDS, including: congenital contractures of fingers and clubfeet, recurrent joint dislocation, progressive foot and spinal deformities, and large subcutaneous hematomas. At age 33, she had been diagnosed with atypical endometrial hyperplasia, and scheduled to undergo total abdominal hysterectomy. To address the risk of massive hemorrhage during the surgery due to mcEDS-related tissue fragility, plasma-derived factor VIII concentrate was prophylactically administered before surgery. During the surgery, neither fragility, hyperextensibility, nor hemorrhagic tendency of the uterus or adnexa observed was observed. The surgery was uneventful, with blood loss of 180 mL and operative time of 2 h and 54 min. However, on the sixth postoperative day, a 2.5-cm dissection was noted at the site of skin incision. Subcutaneous fluid accumulation developed under the skin incision and it persisted for 1.5 months. The postoperative pathological diagnosis was endometrioid carcinoma grade 1, stage IA.ConclusionThe present case suggested that the prophylactic use of factor VIII was effective for the prevention of hemorrhage during surgery. However, delayed wound healing of the skin and subcutaneous tissues was considered a subject for future improvement.

01 Jan 2022·Clinical and Applied Thrombosis/HemostasisQ4 · MEDICINE

Clinical Efficacy and Safety of Fanhdi^®, a Plasma-Derived VWF/Factor VIII Concentrate, in von Willebrand Disease in Spain: A Retrospective Study

Q4 · MEDICINE

Article

Author: Jiménez-Yuste, Víctor ; Núñez, Ramiro ; Nieto Hernández, Maria del Mar ; Marzo Alonso, Cristina ; Varó Castro, Maria José ; Palomo Bravo, Ángeles ; Benítez Hidalgo, Olga ; Coll, Julia ; Galmes, Bernardo J. ; Alvarez-Román, María Teresa ; Mir, Roser ; Hernández García, Carmen ; Carrasco, Marina ; Pérez González, Noelia Florencia ; García Candel, Faustino ; Gonzalez-Porras, Jose Ramon

Objective To evaluate the efficacy and safety of a plasma-derived factor VIII concentrate containing von Willebrand Factor (pdVWF/FVIII) in standard clinical practice in von Willebrand Disease (VWD) patients. Methods A retrospective, multicentric, observational study of VWD patients treated with Fanhdi®, a pdVWF/FVIII concentrate, from January 2011 to December 2017 was conducted at 14 centers in Spain. Efficacy and safety were evaluated for acute bleeding episodes, for prevention of bleeding in surgeries, and for secondary long-term prophylaxis. Results Seventy-two eligible patients, type 1, 2, 3 VWD (25%/38.9%/36.1%) were treated for spontaneous and traumatic bleeding (140 episodes, n = 41 patients), to prevent surgical bleeding (69 episodes, n = 43 patients); and for secondary long-term prophylaxis (18 programs, n = 13 patients). Replacement therapy with pdVWF/FVIII showed an excellent to good clinical efficacy in 96.7% of the bleeding episodes, 100% during surgical procedures and 100% during prophylaxis. No adverse events (AEs), nor serious AEs related to the product were observed. Conclusions Fanhdi® was effective, safe and well tolerated in the management of bleeding episodes, the prevention of bleeding during surgeries, and for secondary long-term prophylaxis in VWD patients.

01 Jan 2022·Haemophilia

Efficacy and safety evaluation of Fanhdi^®, a plasma‐derived factor VIII/ von Willebrand factor concentrate, in Von Willebrand's disease patients undergoing surgery or invasive procedures: A prospective clinical study

Letter

Author: Jimenez‐Yuste, Victor ; Alvarez‐Roman, Maria Teresa ; Grifols, Carlota ; Páez, Antonio ; Haya, Saturnino ; Torres, Mireia ; Martin‐Salces, Monica ; Núñez, Laura ; Mairal, Esther ; Federici, Augusto B.

100 Deals associated with Plasma-derived factor VIII (Octapharma AG)

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Indication	Country/Location	Organization	Date
Hemophilia A	Slovakia	Octapharma AG	-
Hemophilia A	Denmark	Octapharma AG	-
Hemophilia A	Czechia	Octapharma AG	-
Hemophilia A	Netherlands	Octapharma AG	-
Hemophilia A	Latvia	Octapharma AG	-

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Study	Phase	Population	Analyzed Enrollment	Group	Results	Evaluation	Publication Date


ISTH2021	Not Applicable	Hemophilia A	283	Third-generation Recombinant Factor VIII	cczykfaege(ibhggfwvhe) = rzffuotxsq qyizqfucuc (kvhrlmsdcn ) View more	-	17 Jul 2021
				Plasma-derived Factor VIII	cczykfaege(ibhggfwvhe) = nkaghztmeo qyizqfucuc (kvhrlmsdcn ) View more
NIS-Previq (ISTH2019)	Not Applicable	Hemophilia A	34	simoctocog alfa	jfailrtwrc(bomznlaetj) = rxujckyftf eithvrycnh (fbbvaosccb ) View more	-	10 Jun 2019
				plasma-derived factor VIII concentrates	jfailrtwrc(bomznlaetj) = ujoedodyyj eithvrycnh (fbbvaosccb ) View more

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