[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.

[Breast implant-associated anaplastic large-cell lymphoma: A case report and a review of literature]. / Assotsiirovannaia s implantatom anaplasticheskaia krupnokletochnaia limfoma molochnoi zhelezy: opisanie klinicheskogo sluchaia i obzor literatury.

Breast implant-associated anaplastic large-cell lymphoma will be identified as a separate nosological entity in the 2017 adapted WHO classification due to differences in its clinical presentations, pathogenesis, and prognosis with those of nodal and cutaneous anaplastic large-cell lymphomas. The paper gives a review of the literature and describes the authors' own clinical case of common breast implant-associated anaplastic large-cell lymphoma involving breast tissue, axillary lymph nodes, anterior chest muscles, and bone marrow. The treatment policy chosen by the authors could achieve complete remission.

[Perianal infection in patients with hemoblastosis: Risk factors and possibilities of prevention]. / Perianal'naya infektsiya u bol'nykh gemoblastozami: faktory riska i vozmozhnosti profilaktiki.

AIM: to identify poor prognostic factors for perianal infection (PI) in patients with hemoblastosis and to define an effective tactic for preventive and therapeutic measures. SUBJECTS AND METHODS: The prospective study enrolled 72 patients (37 men and 35 women; mean age, 47 years) with hemoblastosis that was complicated by the development of one of the following forms of PI: abscess, infiltrate, multiple ulcers. Different clinical and laboratory characteristics of the patients were examined to identify risk factors for PI. The species-specific concordance of microorganisms isolated from the anus and blood in the development of PI was assessed to record the latter as a source of sepsis. Treatment policy was defined according to the clinical form of PI. RESULTS: Acute myeloid leukemias and lymphomas were the most common background diseases in 30 (41.7%) and 22 (30.6%) patients, respectively. During induction chemotherapy cycles, perianal tissue infection occurred twice more frequently (66%) than totally at the onset of hemoblastosis (13%) and after achievement of remission (during consolidation and maintenance therapy) (21%; Fisher's exact test; p=0.01). PI in agranulocytosis was more than twice as common as in its absence: 69.4% vs 30.6% (p=0.01) and was responsible for sepsis in 9 (18%) of 50 patients. The main source of perianal tissue infection in patients with granulocytopenia was anal fissures and fistulas and ulcers of the anal canal: 44 (88%) cases of the 50 cases. In PI as an abscess, the average white blood cell count was 5 times higher (p=0.01) than that in PI as an infiltrate (or multiple ulcers): 6.6·109/l and 1.2·109 g/l. Abscess formation was observed in 16 (22.2%) patients and an indication for surgical drain. The inflammatory infiltrate was found to develop in 48 (66.7%) patients; multiple ulcers were seen in 8 (11.1%); in this group, parenteral antimicrobial therapy proved to be effective in 36 (78%) patients. 29 patients were operated on for anal fissures and fistulas at intercycle intervals. After continuing CT, PI recurrences were observed in 4 (9.1%) patients. In the operated versus medically treated patients, the risk of complications associated with abnormalities in the perianal area during continued CT was 5 times statistically significantly lower (odds ratio=0.2; 95% confidence interval 0.1 to 0.5; p=0.04; Cochran-Mantel test). CONCLUSION: Induction CT cycles, the status of granulocytopenia, and the presence of infection sources in the anal canal as an anal fissure, skin ulcerations, or a fistula should be considered as independent statistically significant prognostic risk factors for PI. The number of granulocytes determines the form of inflammation, the course of infection, and the chance of developing sepsis. The effective prevention encompassing surgical treatment for anal canal diseases reduces the risk of septic complications and the number of paraproctitis recurrences, contributing to the implementation of a planned CT program in patients with hemoblastosis.

[Clinical and morphological features of different types of Castleman's disease].

AIM: To study the clinical features of Castleman's disease (CD) and to elaborate therapeutic approaches in its different morphological types. SUBJECTS AND METHODS: The clinical and laboratory data were studied in 59 prospectively examined patients and 17 retrospectively examined ones with CD who had been treated at the Outpatient Department, Hematology Research Centre, in 1996 to 2014. There were a total of 37 men (median age, 36 years) and 39 women (median age, 34 years). The diagnosis was established from the results of histological and immunohistochemical examinations of removed lymph nodes (LN) or tumors in all the cases. RESULTS: A hyaline vascular variant (HVV) with local LN involvement was diagnosed in 38 (50%) patients; a plasma cell variant (PCV) was in 38 (50%); among the latter, 17 (22%) patients were found to have local involvement and 21 (28%) had generalized (multicentriC) involvement (multicentric Castleman's diseases (MCD)). Five (24%) patients with MCD were established to be infected with human herpesvirus type 8 (HHV-8). HVV was more frequently diagnosed in women (4%) than in men (29%); PCV was equally common in both men (47%) and women (53%); MCD was statistically significantly more frequently encountered in men (86%) than in women (14%) (p=0.05). The basic involvement areas in local HVV and PCV were peripheral (38%), mediastinal (29), retroperitoneal (18%), abdominal (9%), and small pelvic (6%) LNs. HVV and local PCV were benign and these were cured by surgical removal of LNs involved in the pathological process. MCD took its aggressive course with obvious constitutional symptoms, generalized lymphadenopathy, hepatosplenomegaly, hypergammaglobulinemia, autoimmune hemolysis, thrombocytopenia, and involvement of extranodal foci in the pathological process. MCD transformation to plasmablastic lymphoma was observed in 4 of the 5 HHV8-positive patients and followed by a poor outcome. The prognosis of untreated MCD was unfavorable. In a number of cases prednisolone monotherapy worsened prognosis and the MCD patients receiving timely multiple-drug R-CHOP or R-VD chemotherapy could achieve sustained remission (the 5-year overall survival was 55%). CONCLUSION: CD must be included into the differential diagnosis of lymphadenopathies. When specific treatment is performed, the prognosis of HVV and local PCV is favorable: the disease is surgically cured in 95% of cases. Multidrug chemotherapy according to the B-cell lymphoproliferative disease program is indicated for the treatment of MCD: sustained remission can be achieved by the use of R-CHOP or R-VD programs. The HHV-8-positive variants of MCD increase the probability of transforming the disease to incurable plasmablastic lymphoma. Overall, prognosis and therapy choice in HIV-negative patients with CD depend on the histological variant of the disease, the extent of a tumor, and HHV-8 infection.