Thrombosis is a serious and extremely dangerous disease that has a negative impact on the quality and longevity. Antiphospholipid syndrome (APS) is a pathology characterized by recurring venous, arterial, microvasculature thrombosis, pregnancy pathology with loss of the fetus and the synthesis of antiphospholipid antibodies. A high risk of thrombotic complications is also observed in patients with myeloproliferative neoplasms (MPN). This article presents a description of three clinical cases of Ph - negative myeloproliferative diseases, occurring in conjunction with APS. In all cases, recurrent thrombosis allowed to suspect the presence of two diseases - MPN and APS.
Purpose of the study. A comparative evaluation of the effectiveness of different therapeutic strategies in patients with polycythemia vera (PV) and essential thrombocythemia (ET). Materials and methods. Patients with PV or ET, diagnosed according to the criteria WHO 2016 were included in the study. The primary endpoint - 6 months of therapy (clinical-hematological and molecular responses). The secondary endpoint - 12 months of therapy (clinico-hematologic, molecular, histological responses). Sixty three patients were included in the analysis: the first group consisted of 33 patients who received the therapy with ce-pegiterferone alpha-2b (ce-pegalpha-INF-α-2b), 10 of them received previous treatment; the second group - 23 patients btained hydroxycarbamide; the third group - 7 patients were treated with recombinant interferon alpha therapy (rINFα). In comparison groups, differences in age were revealed: patients receiving hydroxycarbamide therapy were older. Phlebotomy occurred in 36% of patients in the first group, 9% in the second group, and 14% in the third group. Results. By the 6th month of therapy, 43% of the patients receiving the ce-pegalpha-INF-α-2b had complete clinical-hematologic response, 36% had partial clinical-hematologic remission and stabilization of the disease was established in 21% cases. No disease progression occured. By the 12th month of therapy, statistically significant differences in terms of efficacy between the different therapeutic groups (p = 0.2462, Fisher's exact test). In all three groups, the allelic load of JAK2V617F decreased: from 50 to 19%, from 22.3 to 15.8%, from 50 to 7.19%, respectively. The lower the allele load positively correlated with better response to therapy, which was observed in all analyzed groups. Hematologic adverse events (AEs) were more frequently observed in patients receiving ce-pegalpha-INF-α-2b therapy. Local reactions developed on 3-7 days of therapy as a hyperemic macula at the injection site. Both these reactions and hair loss did not influence on patient’s condition. In the second group (patients with hydroxycarbamide therapy) there were changes in the skin and mucous membranes: dry skin, stomatitis, and in older patients new keratomas appeared. The flu-like syndrome was the most common adverse event associated with the therapy of ce-pegalpha-INF-α-2b, which fully relived during the first month of therapy. There was only one case with the flu-like syndrome we observed at the 11th month of therapy. As a rule, the biochemical blood test changes did not influence on patient’s condition, were mostly associated with dietary violations, had a tendency to self-resolution and did not require medical interventions. Serious AEs were reported in one case - pulmonary embolism in a patient treated with rINFα. The reasons for the therapy discontinue in group 1: toxic hepatitis, intolerance, by the request of the patient, inadequate efficacy of therapy; in group 2: skin toxicity, in group 3: thromboses. The conclusion. Treatment of ce-pegalpha-INF-α-2b in patients with PV and ET is highly effective - the most patients pbtained clinical and hematological responses. There were no statistically significant differences in these parameters in comparison with hydroxycarbamide and rINFα. The use of the ce-pegalpha-INF-α-2b had an acceptable safety profile. The estimated therapeutic dose should be calculated according to body weight. To reduce the frequency of hematologic AE, titration of the drug dose is required.
Background: Castleman' s disease (CD) is rare lymphoproliferative disorder with long asymptomatic current and high risk of transformation in malignant lymphoma. For last years, diagnostics of CD improved and uniform classification of an illness was accepted. Depending on morphological features allocate 3 variant of CD: hyaline-vascular (HVV), plasma cell (PCV) and mixed cell. Most cases of the ÑD involve a single or localized group of lymph nodes and can be asymptomatic. In these cases the histologic picture can be characterized as a HVV or mixed cell and PCV morphology. Less often the disease proceeds with multiple lymphoid regions (multicentric CD, MCD) and is usually accompanied by various systemic inflammatory symptoms (fever, weight loss and night sweats). In these cases histologic picture in the involved lymph nodes meet mix cell or PCV of CD. The role of a human herpes virus-8 (HHV-8) in pathogenesis of MCD is studied. It is known that HHV-8 codes in a genome of a cell of the person some regulatory proteins and cytokines, first of all a virus homolog interleukin-6 which stimulates development of human IL-6 and endothelial factor of growth that conducts to a neoangiogenesis in not tumoral lymph nodes. HHV-8-positive MCD allocate separately in due to its extremely aggressive course and a high risk of transformation into HHV-8-positive plasmablastic lymphoma. Aim: Explore the features of a clinical current and of therapeutic approaches at different variants CD. Patients and Methods: In Center for Hematology since 1996 to the present time observed clinical and morphological features of 76 pts with CD. Clinical data were obtained during the retrospective analysis of 17 outpatient clinical records and prospective supervision over 59 pts. The diagnosis was established from results of histological and immunohistochemical examinations of removed lymph nodes in all cases. Frequency analyses were performed using z-tests with SAS 9.1 software. Results: HVV with local lymph nodes involvement was diagnosed in 38 (50%) pts. The average age of pts was 40 years The PCV was also observed in 38 (50%) cases, among them in 17 (22%) pts were found to have local involvement and 21 (28%) had multicentric involvement. Five (24%) pts with MCD were established to be infected with human herpesvirus type 8. Male (37 pts) and female (39 pts) are suffering with an identical frequency. However, HVV is more often diagnosed for female, than at male (71% to 29% respectively), PCV equally often meets both at male, and at female (47% of male, 53% of female) and, at last, at MCD the share of male statistically significantly is more than share of female (86% against 14%, p=0,05) (Figure 1). The basic involvement areas in local HVV and PCV were peripheral (38%), mediastinal (29%), retroperitoneal (18%) abdominal (9%) and small pelvic (6%) lymph nodes (95% CI). Local HVV and PCV proceed benign and in 94.5% of cases are cured by surgical removal of lymph nodes involved in the pathological process, in 5.5% of cases with inoperable cases require followed by radiation therapy. MCD was significantly more common in male, occurs aggressively with severe constitutional symptoms, generalized lymphadenopathy, hepatosplenomegaly, hypergammaglobulinemia, autoimmune hemolysis, thrombocytopenia, and involvement of extranodal foci in the pathological process. MCD transformation to plasmoblastic lymphoma was observed in 4 of 5 HHV8-positive pts and followed by a poor outcome. The prognosis of untreated MCD was unfavorable. In a number of cases prednisolone monotherapy worsened and the MCD pts receiving timely R-CHOP or R-VD chemotherapy could achieve sustained remission (the 5-year OS was 55%). Conclusion: The prognosis of HVV and local PCV is favorable: the disease is surgically cured in 95% of cases. Chemotherapy according to the program of Non-Hodgkins Lymphomas indicated for the treatment of MCD: sustained remission can be achieved by the use of R-CHOP or R-VD programs. HHV8-positive MCD characterized by high risk disease transformation to incurable plasmablastic lymphoma. Overall, prognosis and therapy choice in HIV-negative pts with CD depend on the histological type of the disease, the extent of a tumor and HHV-8 infection. Figure 1. Gender distribution for three variants of CD; there is male-female imbalance in HVV and MCD groups (z-test, ð<0.05); analysis based on initial diagnosis data. Figure 1. Gender distribution for three variants of CD; there is male-female imbalance in HVV and MCD groups (z-test, ð<0.05); analysis based on initial diagnosis data. Disclosures No relevant conflicts of interest to declare.
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