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LETTERS TO THE EDITORS

Acknowledgements

Disclosures

This study with project number of 91-3-31-19076 was approved and supported by Tehran University of Medical Sciences. All authors of this manuscript thank the vice chancellor of Tehran University of Medical Sciences.

The authors stated that they had no interests which might be perceived as posing a conflict or bias.

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5 Reiner AP, Schwartz SM, Frank MB et al. Polymorphisms of coagulation factor XIII subunit A and risk of nonfatal haemorrhagic stroke in young white women. Stroke 2001; 32: 2580–7. 6 Mosnier LO, Bouma BN. Regulation of fibrinolysis by thrombin activatable fibrinolysis inhibitor, an unstable carboxypeptidase B that unites the pathways of coagulation and fibrinolysis. Arterioscler Thromb Vasc Biol 2006; 26: 2445–53. 7 Willemse JL, Hendriks DF. A role for procarboxypepidase U (TAFI) in thrombosis. Front Biosci 2007; 12: 1973–87.

8 Trinh CH, Sh ElSayed W, Eshghi P et al. Molecular analysis of sixteen unrelated factor XIIIA deficient families from south-east of Iran. Br J Haematol 2008; 140: 581–4. 9 Tamaddon GH, Kazemi A, Rastegar G, Alla F, Hejazi S. Molecular basis of inherited factor XIII-A deficiency among patients from Sistan-Baluchestan. Zahedan Journal of Research in Medical Sciences. 2010; 11: 19–24. 10 Tokgoz S, Zamani A, Durakbasi-Dursun H et al. TAFI gene polymorphisms in patients with cerebral venous thrombosis. Acta Neurol Belg 2013; 113: 291–7.

A rare case of acquired haemophilia in a patient with chronic myelomonocytic leukaemia successfully treated with decitabine D . S H A H , * R . K U M A R † and S . G A I K A Z I A N † *Department of Internal Medicine; and †Department of Hematology Oncology, Oakland University William Beaumont School of Medicine, Royal Oak, MI, USA

Acquired haemophilia is a rare and fatal disorder; heightened awareness of this disease is required in cases of unexplained bleeding. Haematological malignancy is a potential aetiology of acquired haemophilia, and treating this underlying condition may result in a favourable outcome. We report a rare case of acquired haemophilia in a chronic myelomonocytic leukaemia (CMML) patient who responded dramatically after the initiation of decitabine. This is the first reported association between the hypomethylating agent decitabine and acquired haemophilia. A 58-year-old man presented to our hospital after he developed diffuse ecchymosis and spontaneous left Correspondence: Darshil Shah, Department of Internal Medicine, Oakland University William Beaumont School of Medicine, Beaumont Health System, 3601 W 13 Mile Road, Royal Oak, MI 48073, USA. Tel.: +1 248 551 5000; fax; +1 248 898 4680; e-mail: [email protected] Accepted after revision 7 October 2013 DOI: 10.1111/hae.12301 Haemophilia (2014), 20, e79--e112

iliopsoas haematoma. He was diagnosed with CMML-1 on a bone marrow examination 6 months prior. Bone marrow biopsy at that time demonstrated hypercellular marrow (60 70%) with marked monocytic (15%) hyperplasia. Myeloid blasts were within the normal range (2%), and the cytogenetics were normal. The patient was also diagnosed with rheumatoid arthritis during this time, had elevated cyclic citrullinated peptide levels (above 250), and was on chronic steroid therapy. His activated prothrombin time (aPTT) upon presentation was markedly prolonged (96.3 s; normal, 25–32 s), whereas the prothrombin time was normal (11.2 s). A mixing study failed to correct the aPTT prolongation. A quantitative assay of factor VIII (FVIII) inhibitors detected an inhibitor level of >200 BU. FVIII activity was

A rare case of acquired haemophilia in a patient with chronic myelomonocytic leukaemia successfully treated with decitabine.

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