Hemorrhagic disorder due to an isoniazid-associated acquired factor xiii inhibitor in a patient with waldenstrom's macroglobulinemia

Krumdieck, Richard; Shaw, Denise R.; Huang, Shu T.; Poon, Man‐Chiu; Rustagi, Pradip K.

doi:10.1016/s0002-9343(05)80019-1

Cited by 20 publications

(21 citation statements)

References 45 publications

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“…Inhibitors arising from replacement therapy in congenital FXIII deficiency are rare, and inhibitors causing acquired FXIII deficiency are 10 times as rare as congenital FXIII deficiency [8]. Acquired FXIII deficiency has been reported with autoimmune disorders [9], and medications including phenytoin [10], isoniazid [11,12], penicillin [13] and procainamide [14]. FXIII inhibitors complicating amiodarone therapy have not been reported, and in our patient with inactive rheumatoid arthritis, the inhibitor persisted after discontinuing amiodarone.…”

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Prophylactic and perioperative replacement therapy for acquired factor XIII deficiency

Lim

Moffat

Hayward

2004

Journal of Thrombosis and Haemostasis

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To cite this article: Lim W, Moffat K, Hayward CPM. Prophylactic and perioperative replacement therapy for acquired factor XIII deficiency. J Thromb Haemost 2004; 2: 1017-19. Acquired factor (F)XIII deficiency is a rare coagulation disorder with limited information on using laboratory assays to guide management. We encountered unique monitoring and therapeutic challenges while managing a 73-year-old man with acquired FXIII deficiency. He presented with an extensive leg hematoma after receiving low-molecular-weight heparin for a suspected calf vein thrombosis. Two years earlier, he had developed a leg hematoma while on warfarin, which was discontinued. His medical history included inactive rheumatoid arthritis, stable angina, and paroxysmal atrial fibrillation on amiodarone. There was no family or prior bleeding history. He had previously undergone tonsillectomy, cholecystectomy and dental extractions without incident. Coagulation tests indicated an isolated deficiency of FXIII, with no FXIII activity detectable by the urea clot solubility assay [1,2] , twice weekly). A portacath was placed for home replacement therapy. Amiodarone was discontinued due to concerns about a drug-induced FXIII inhibitor.Six months later, the patient developed increasing angina. Angiography revealed a 90% stenosis of the left anterior descending coronary artery and a single coronary artery bypass graft was recommended. His FXIII inhibitor titer remained low (< 2 BU) and preoperative FXIII survival studies confirmed accelerated FXIII clearance, with more rapid decay of FXIII activity compared with FXIIIA antigen (Fig. 1A). Bypass surgery was done using off-pump, beating heart surgery to minimize risks of a postoperative coagulopathy. Perioperatively, FXIII levels were monitored using the Berichrom assay, as no other rapid commercial assays were available. He was given 2500 U FXIII (42 U kg ) immediately before and after surgery, and again on postoperative days 1-3, 5-7 and 9, before resuming his usual prophylaxis (2500 U twice weekly), which normalized his FXIII activity during surgery and maintained levels above 0.40 U mL )1 for 7 days postoperatively (Fig. 1B). No abnormal bleeding occurred and he recovered fully without complications. Over the subsequent 38 months, his FXIII inhibitor has remained < 2 BU and he has been maintained on lower doses of FXIII prophylaxis and aspirin 80 mg daily, without further angina or serious bleeding.He had a few minor bleeds when his FXIII prophylaxis was reduced to the point that no predose FXIII activity was detectable by urea clot solubility assays, but this resolved when his prophylaxis was adjusted to provide residual FXIII activity (1750 U, 29 U kg )1 weekly).FXIII is important for stabilizing fibrin and making clots less susceptible to plasmin lysis [5][6][7]. Inhibitors arising from replacement therapy in congenital FXIII deficiency are rare, and inhibitors causing acquired FXIII deficiency are 10 times as rare as congenital FXIII deficiency [8]. Acquired FXIII deficiency has been report...

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Prophylactic and perioperative replacement therapy for acquired factor XIII deficiency

Lim

Moffat

Hayward

2004

Journal of Thrombosis and Haemostasis

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“…Faktör XIII bir transglutaminaz olup, pıhtılaşma sisteminin son basamağında görev almaktadır (5,6,7). Fibrin molekülleri arasında kovalent bağ oluşturarak fibrinin mekanik ve kimyasal etkenlere karşı dirençli hale gelmesinde önemli bir görevi vardır (5,8).…”

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“…İzoniazid, penisilin, fenitoin gibi bazı ilaçların alımını izleyerek faktör XIII'e karşı gelişen IgG antikorlarına bağlı olarak edinsel faktör XIII eksikliği gelişebilir. Kronik karaciğer hastalığı, üremi, lenfoproliferatif ve myeloproliferatif hastalıkların seyri sırasında da hafif şiddette akkiz faktör XIII eksikliği görülebilir (6,7).…”

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“…Kanama çoğu kez travmadan hemen sonra başlar, bazen de 12-36 saat sonra görülebilir. Hasta kadınlarda tekrarlayan spontan düşükler olabilir (7,12). Karimi ve ark.…”

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“…Hastamızda TDP ile kısa vadede iyileşme sağlanmıştır. Literatürde 3-4 haftada bir 2-3 ml/kg TDP transfüzyonu ile etkili hemostaz sağlanabileceği bildirilmektedir (7,12). Hastamızda da aylık TDP tedavisi düzenlenmiştir.…”

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Recurrent Spontaneous Intracerebral Haemorrhage Due To Factor XIII Deficiency

Öcek¹,

Bilgin²,

Bilgir³

et al. 2014

Türk Beyin Damar Hast Derg

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ÖZETPrimer intraserebral kanama (İSK)'nın en sık nedeni hipertansiyon (HT)dur (1). Bu etkenler içinde hematolojik nedenler oldukça azdır. Hematolojik nedenler içinde de nadir görülen Faktör XIII eksikliğine bağlı İSK geçiren bir olguyu sunmaya değer bulduk. 36 yaşında kadın hasta hastanemize ani gelişen bulantı, kusma, bilinç bozukluğu, sağ hemipleji nedeni ile yatırıldı. Beyin bilgisayarlı tomografisi(BBT)de sol frontoparietal bölgede hematom izlendi. BT-anjio normaldi. Diğer laboratuvar testleri normal iken FXIII erime testi pozitifti. Antiödem ve taze donmuş plazma (TDP) tedavisi uygulandı. Motor afazi ve hafif sağ hemiparezi ile taburcu edildi. Hastanın bir ay sonraki nörolojik muayenesi olağandı. 3 ay sonra şiddetli baş ağrısı nedeniyle yapılan BBTde sol parietal bölgede yeni bir hematom izlendi. Tekrar TDP verildi. Bu kez nominal afaziyle taburcu edildi. Spontan ve tekrarlayan ISK olgularında hematolojik nedenler mutlaka akla getirilmelidir. Hematolojik nedenlerden koagülasyon bozukluklarına bağlı İSKların tedavisi mümkün ve hayat kurtarıcıdır. Anahtar Sözcükler: İntraserebral kanama, koagülasyon bozuklukları, Faktör XIII eksikliği, taze donmuş plazma. RECURRENT SPONTANEOUS INTRACEREBRAL HAEMORRHAGE DUE TO FACTOR XIII DEFICIENCY ABSTRACTWhile hypertension is the most common cause of primer ıntracerebral hemorrhage, various rare causes can be cited including hematologic diseases. In this report, a case with recurrent intracerebral hemorrhage due to a rare coagulation defect, factor XIII deficiency is presented. Thirty six year old female patient was admitted to the neurology clinic with sudden onset nausea, vomiting, unconsciousness and right hemiplegia. A large hematoma was seen at the left frontoparietal region on computed brain tomography (CT). CT angiography was normal. All the laboratory tests were normal, except factor XIII clot solubility test being positive. Antiedema and fresh frozen plasma therapy (FFP) were given for several days. After stabilization of the clinical picture, the patient was discharged with motor aphasia and slight right hemiparesis. One month later the patient was seen with normal neurological examination. However, three months later she was admitted to the hospital again with severe headache. CT was repeated and in the parietal lobe, a new hematoma was seen. She was treated with FFP in the same way and discharged 2 weeks later with nominal aphasia. In spontaneous and recurrent ICH cases, hematologic causes must be kept in mind albeit very rare, especially in the young. İt is important to identify coagulation disorders among hematologic causes as their therapy is possible and life saving.

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