[Acute arterial thrombosis in hemophilia with inhibitory antibodies]. / Ostryi arterial'nyi tromboz pri ingibitornoi forme gemofilii.

The authors demonstrate an importance of personalized approach to perioperative hemostatic therapy in a 48-year-old patient with hemophilia A and inhibitory antibodies. Laparoscopic hernia repair and extraction of 15 decayed teeth were performed. Hemostatic therapy included AICC and rFVIIa. Postoperative period was complicated by acute thrombosis of splenic artery and partial spleen infarction. An essential factor in splenic artery thrombosis was increase in blood coagulation potential under rFVIIa administration and depletion of fibrinolytic system (prolongation of XIIa-dependent fibrinolysis from 25 to 75 min) and antithrombin III decrease up to 81%. Cancellation of hemostatic therapy under TEG control ensured fast regression of arterial thrombosis and preservation of spleen. Individual characteristics of patients (compensatory mechanisms of coagulation, comorbidities, clinical changes) should be considered when prescribing hemostatic therapy in hemophilia patients. Perioperative control of all possible coagulation tests (routine and integral) is required for individual selection of hemostatic therapy and decrease of the risk of hemorrhagic and thrombotic complications.

[Inhibitory and mild forms of hemophilia: diagnostic difficulties, surgical complications]. / Trudnosti diagnostiki i khirurgicheskie oslozhneniia pri razlichnykh formakh gemofilii.

AIM: To identify patients with hemophilia who have a high risk of postoperative hemorrhagic complications. MATERIAL AND METHODS: Prospective trial included 69 patients aged 18-71 years (median 29) with congenital hemophilia A and B. They underwent elective and emergency treatment for abdominal and thoracic pathology at the National Medical Research Center for Hematology in 2011-2016. Patients with mild and inhibitory forms of hemophilia were compared with those with severe and moderate forms of hemophilia. There were 50 (73%) patients with severe and moderate forms of hemophilia, 8 (11%) with inhibitory and 11 (16%) patients with mild form. Emergency operations were performed in 18 cases, elective - in 51. RESULTS: Inhibitory form of hemophilia is associated with 1.5 times higher (95% CI, 1.1-3.0) risk of hemorrhagic postoperative complications and death and 3,5 times higher (95% CI 1.7-5.9) risk of redo surgery compared with severe and moderate forms (p<0.05). Risk of hemorrhagic postoperative complications is also higher in patients with mild form of hemophilia compared with severe and moderate forms (1/6 vs. 1/50; p=0.05). CONCLUSION: The risk of postoperative hemorrhagic complications is significantly higher in inhibitory and mild hemophilia compared with severe and moderate forms and associated with hemostatic therapy defects and inadequate assessment of hemostatic disorders. Long-standing haemorrhagic syndrome should be followed by blood clotting system analysis including evaluation of procoagulant activity, presence of inhibitor, and thromboelastography. Decreased levels of albumin (by 2.9-8.6% in our trial) and cholesterol (by 6.5-54.8%) reflects impaired liver function and is sign of unfavorable prognosis. This finding should be considered for surgery and therapy of hemorrhagic manifestations.

A Single-center Experience in Splenic Diffuse Red Pulp Lymphoma Diagnosis.

The World Health Organization 2008 classification highlighted a new nosology-splenic diffuse red pulp lymphoma (SDRPL) with clinical and laboratory features similar to both splenic marginal zone lymphoma and hairy cell leukemia (HCL) and variant form of HCL. Experience of hematologists on the diagnosis and differential diagnosis of SDRPL is extremely limited. The aim of our report was to characterize the clinical and immunomorphologic features of SDRPL on our own observations. During 2013-2014, in National Research Center for Hematology, 87 spleen specimens removed from various B-cell lymphomas were analyzed. In four (4.6%) cases, the diagnosis SDRPL was made based on morphologic, immunohistochemical, immunophenotypic, molecular examination of spleen biopsies, blood and bone marrow samples. In all cases of SDRPL were observed significant splenomegaly, lymphocytosis from 56% to 94% (in two cases with leukocytosis 55.000 and 75.000 109/l). The circulating "villous" lymphocytes phenotype was CD20+ (bright), CD11c+/±, CD103 (weakly)+/±, LAIR-1+, CD25-, CD5-, CD10-, and CD23-. Mutation BRAFV600E was not detected. Bone marrow with minor lymphoid CD20+, CD25-, Annexin1-, Cyclin D1- cell infiltration. The average weight of the spleen was 3900 g (1450-9500 g), and morphologically, there was revealed lymphoid infiltration of red pulp with phenotype CD20+, DBA.44+, CD25-, Annexin1-, Cyclin D1-, CD103-, CD123-, CD27-, focal SD11c± and TRAP±. Now patients are observed in remission: two patients after splenectomy, two after splenectomy and cladribine+rituximab chemotherapy. SRDPL-a rare lymphoma that is suspected in the cases with significant splenomegaly and lymphocytosis with villous lymphocytes forms that have only a part of the classic markers HCL, with minor bone marrow infiltration. The standard diagnosis and treatment is splenectomy. Differential diagnosis of SMZL and HCL has clear criteria, but criteria of differentiation with variant HCL are still unknown.