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Defective α2 antiplasmin cross-linking and thrombus stability in a case of acquired factor XIII deficiency.
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Defective α2 antiplasmin cross-linking and thrombus stability in a case of acquired factor XIII deficiency.

Br J Haematol. 2017 May 17;:

Authors: Mitchell JL, Wright S, Kazi S, Watson HG, Mutch NJ

Abstract
Acquired factor XIII (FXIII) deficiency is a rare and life-threatening condition that is often misdiagnosed or missed completely. A 72-year-old woman presented with symptoms of major unprovoked bleeding but routine coagulation screening tests and platelet count were normal. Low activated FXIII (FXIIIa) activity levels and abnormal urea clot stability led to diagnosis of acquired FXIII deficiency. A modified Bethesda inhibitor titre of 1.6 Bethesda units/ml indicated the presence of a FXIII inhibitor. Bleeding responded to a single dose of FXIII concentrate and immunosuppression with prednisolone induced remission. A subsequent relapse was treated with combined prednisolone and Rituximab resulting in a prolonged, ongoing remission. Here we analyse the mechanisms underlying this idiopathic case of acquired FXIII deficiency. Prospective analysis of patient plasma revealed minimal FXIIIa activity and antigen in presentation and relapse samples. Thrombi formed from these samples lysed rapidly and showed an absence of cross-linked α2 AP. Western blotting revealed the presence of FXIII-B, indicating only FXIII-A and FXIII-A2 B2 were affected. FXIII activity and antigen levels normalised on remission. Our data suggest the presence of inhibitor-induced clearance of FXIII from plasma. As a consequence, reduced thrombus stability was evident due to defective α2 AP cross-linking, thereby explaining symptoms of excessive bleeding.

PMID: 28516512 [PubMed - as supplied by publisher]




The histone acetyltranseferase KAT8 regulates cell differentiation by suppression of MN1 in AML.
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The histone acetyltranseferase KAT8 regulates cell differentiation by suppression of MN1 in AML.

Br J Haematol. 2017 May 17;:

Authors: Zhao WB, Wang M, Gao S, Shaikh AS, Chen J, Li XZ

PMID: 28516501 [PubMed - as supplied by publisher]




Incidental finding of recent parvovirus infection.
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Incidental finding of recent parvovirus infection.

Br J Haematol. 2017 May 18;:

Authors: Leung E, McBride E

PMID: 28516484 [PubMed - as supplied by publisher]




Massive mediastinal enlargement due to extramedullary haematopoiesis in a patient with MYH9-related thrombocytopenia.
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Massive mediastinal enlargement due to extramedullary haematopoiesis in a patient with MYH9-related thrombocytopenia.

Br J Haematol. 2017 May 17;:

Authors: Zaninetti C, Boveri E, Melazzini F

PMID: 28516481 [PubMed - as supplied by publisher]