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Copyright of Transfusiology & Haematology Today / Transfuze a Hematologie Dnes is the property of Czech Medical Association of JE Purkyne and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Transfusiology & Haematology Today / Transfuze a Hematologie Dnes is the property of Czech Medical Association of JE Purkyne and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Obinutuzumab is a new anti-CD20 monoclonal antibody predominantly used in the treatment of patients with chronic lymphocytic leukaemia and follicular lymphoma. In our case report, we describe successful treatment using obinutuzumab in a patient with Waldenström macroglobulinemia. This therapy was used as 4th line-treatment and led to complete bio-chemical and clinical response of Waldenström macroglobulinemia. Obitunuzumab could thus be useful option even in such a rare disease. [ABSTRACT FROM AUTHOR]

This article describes a rare case of Waldenström's macroglobulinemia with autoimmune myopathy and presents a short overview of other possible autoimmune manifestations associated with the presence of monoclonal immunoglobulin (MIg). In our patient, Waldenström's macroglobulinemia manifested as chronic muscle pain and anaemia. The patient suffered from back muscle pains for about 3 years before being dia-gnosed with WM, with progression of myalgia and pain of the upper and lower limbs in the last year prior to dia-gnosis. Serum myoglobin and creatinkinase (CK) levels were significantly increased. Polymyositis was not confirmed. The dia-gnosis of WM was established in 2014, with typical infiltration with lympho-plasmacytoid lymphoma in the bone marrow and the presence of IgM lambda paraproteinemia in the serum. The patient started treatment with the anti-CD20 monoclonal antibody rituximab, cyclophosphamide and dexamethasone and received 8 cycles from 7/2014–2/2015. The IgM disappeared, the immunofixation results normalised as did myoglobin, CK and haemoglobin after the 5th cycle and there was significant improvement of muscle pain. The patient achieved a complete remission with no signs of relapse/progression at the last follow-up in 12/2019 with normal or only mildly elevated CK and myoglobin. [ABSTRACT FROM AUTHOR]

Erdheim-Chester disease (ECD) is a rare form of histiocytosis, in present time ECD is classified as Langerhans-cell histiocytosis. It encompasses a spectrum of disorders ranging from asymptomatic bone lesions to multisystemic, life-threatening variants with CNS involvement. ECD prognosis is very variable. In this article, ECD treatment possibilities are widely discussed. The first line treatment is usually cladribine and the second line treatment is anakinra. ECD therapy is only partially effective with 50% treatment responses in various modalities at the maximum. ECD pathogenesis understanding has been widely improved with the discovery of MAPK pathway mutations, that have led to the use of targeted therapy (MEK and BRAF inhibitors) trials, with major efficacy. In the year 2020 BRAF and MEK inhibitors are recomended as first line treatment for severe CNS impairment, in other ECD cases the BRAF and MEK inhibitors are used as the second-line therapy. [ABSTRACT FROM AUTHOR]

Still's disease (Adult-onset Still's disease, AODS) is a very rare systemic inflammatory disease accompanied by a triad of spiking fever, maculopapular exanthema and arthralgia. To date, there is no specific laboratory or imaging test available for AODS. We report the case of a 65-year-old male who suffered from fever and arthralgia. Initial laboratory tests showed elevated CRP, leukocytosis and anaemia. Metabolically active abdominal lymphadenopathy was present. We excluded many potential differential diagnoses and the diagnosis of lymphoma was not confirmed. After an extensive workup, the patient was diagnosed with AOSD based on the Yamaguchi criteria. The first line therapy was prednisone at a dose of 1mg/kg, after which all AODS symptoms disappeared including fever. After tapering the prednisone dose, the AOSD relapsed. The patient then received anakinra, 100 mg daily as second line treatment and achieved a good response, but it was necessary to continue this treatment. Our patient has now been treated with anakinra from 2016-to date and remains asymptomatic. In this article, we describe our AOSD case and provide a brief overview of typical AOSD clinical symptoms and treatment options. [ABSTRACT FROM AUTHOR]

Necrobiotic xanthogranuloma is an uncommon non-Langerhans cell histiocytosis involving skin and extra cutaneous tissues. Necrobiotic xanthogranuloma is most frequently associated with MGUS, myeloma and may also occur in other lymphoproliferative disorders. Its pathogenesis remains unclear. Necrobiotic xanthogranuloma is usually a chronic and slowly progressive disease. Several treatments have been proposed and have yielded variable results. The aim of this case report and systematic review is to summarize all reported treatments of necrobiotic xanthogranuloma of the skin, with or without underlying malignant conditions. Treatment options include corticosteroids, thalidomide, high-dose intravenous immunoglobulins (IVIG), chlorambucil, cyclophosphamide, fludarabine, rituximab, melphalan, infliximab, cladribine, laser therapy, radiotherapy and other possibilities. The treatment options are at most only partially effective; the best response is achieved with IVIG therapy. In our case, treatment with cyclophosphamide, dexamethasone and bortesomib did not lead to any significant response. We then used IVIG and electron beam irradiation achieving a partial response and stabilization of this rare disease.. [ABSTRACT FROM AUTHOR]

Copyright of Transfusiology & Haematology Today / Transfuze a Hematologie Dnes is the property of Czech Medical Association of JE Purkyne and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Transfusiology & Haematology Today / Transfuze a Hematologie Dnes is the property of Czech Medical Association of JE Purkyne and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Transfusiology & Haematology Today / Transfuze a Hematologie Dnes is the property of Czech Medical Association of JE Purkyne and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Aim: To evaluate the results of negative pressure wound therapy (NPWT) in the management of post-operative wound infections (Szilagyi's classification grade II and III) after elective arterial revascularization. Patients and methods: Retrospective analysis of patients' data. All patients who were treated between 2015 and 2018 using NPWT for wound infections after elective arterial procedures on lower extremities were included. Results: Between 2015 and 2018 a total of 13 patients have been treated using NPWT after elective infrainguinal arterial revascularization. There were nine men and four women of an average age of 72 years. Nine patients were treated for Szilagyi grade II infection (69.2%) and four patients for Szilagyi grade III infection (30.8%). In 11 cases there was an early postoperative infection (84.6%). The most common bacterial agent was Staphylococcus aureus. The minimum follow-up was 12 months. During the follow-up, the patency of arterial reconstructions was 92.3% and in 84.6% complete wound healing was achieved. The treatment success rate in the artificial graft patients' group was 88.9%. Conclusion: NPWT is an effective treatment of wound infections in patients after arterial lower extremity revascularization. The method shows promising results in the management of artificial graft infection as well. [ABSTRACT FROM AUTHOR]

Copyright of Transfusiology & Haematology Today / Transfuze a Hematologie Dnes is the property of Czech Medical Association of JE Purkyne and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Transfusiology & Haematology Today / Transfuze a Hematologie Dnes is the property of Czech Medical Association of JE Purkyne and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Allogeneic stem cell transplantation (SCT) is considered to be the treatment of choice for many haematological disorders, especially haematological malignancies. We present here our long-term experience with allogeneic SCT in cohort of 533 pts. We analysed 533 pts who underwent allogeneic SCT at our centre from November 1996 to June 2017. The diagnoses were as follows: AML (187 pts; 35%), ALL (73 pts, 14%), lymphomas (66 pts, 12%), MDS + MPN (52 pts, 10%), CML (74 pts, 14%), CLL (47 pts, 9%), other diagnoses (34 pts, 6%). The median age was 45 years (range 18–64). The types of donors and grafts used were as follows: HLA identical sibling, n = 257 (48%); unrelated donor, n = 276 (52%); PBSCs, n = 492 (92%); BM, n = 41 (8%). Median follow-up from SCT was 26.6 months, median follow-up from SCT for surviving pts was 83.2 months. Myeloablative conditioning (MAC) was used in 253 pts (47%), reduced intensity conditioning (RIC) was used in 280 pts (53%). Disease status at SCT was remission in 351 pts (66%) and active disease in 182 pts (34%). Median time from diagnosis to SCT was 7.4 months. The overall response rate after allogeneic SCT was 82%, including CR in 78% of pts and PR in 4% of pts. The incidence of acute and chronic GvHD was 42% and 40% resp. Non-relapse mortality (NRM) was 18%, 19% and 20% at 1 year, 2 years and 4 years from SCT resp. Relapse incidence was 22%, 27% and 31% at 1 year, 2 years and 4 years from SCT resp. Median PFS was 31.5 months, median OS was 85.6 months. Gender, type of donor, type of graft, time from diagnosis to SCT and type of conditioning did not significantly influence PFS and OS in our cohort of pts. Patients with AML had significantly shorter PFS compared to pts with other diagnoses (median PFS 18.9 months versus 43.2 months, p = 0.031, HR 1.32). Patients with active disease at SCT had significantly shorter PFS compared to pts in remission at SCT (median PFS 9.6 months versus 73.7 months, p < 0.001, HR 1.79). On multivariate analysis, diagnosis of AML versus other diagnoses (median OS 29.7 months versus 197.2 months, p < 0.001, HR 1.63) and active disease at SCT versus remission at SCT (median OS 25.0 months versus 186.5 months, p < 0.001, HR 1.93) were significant predictors of poor OS. Allogeneic transplantation remains the standard treatment option predominantly for various haematological malignancies. According to our results, the type of diagnosis and disease status at allogeneic SCT are important prognostic factors for PFS and OS. Patients in remission of their disease at the time of allogeneic SCT have significantly longer PFS and OS. [ABSTRACT FROM AUTHOR]

Copyright of Transfusiology & Haematology Today / Transfuze a Hematologie Dnes is the property of Czech Medical Association of JE Purkyne and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Transfusiology & Haematology Today / Transfuze a Hematologie Dnes is the property of Czech Medical Association of JE Purkyne and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Transfusiology & Haematology Today / Transfuze a Hematologie Dnes is the property of Czech Medical Association of JE Purkyne and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Transfusiology & Haematology Today / Transfuze a Hematologie Dnes is the property of Czech Medical Association of JE Purkyne and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Česká a Slovenská Neurologie a Neurochirurgie is the property of Czech Medical Association of JE Purkyne and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Transfusiology & Haematology Today / Transfuze a Hematologie Dnes is the property of Czech Medical Association of JE Purkyne and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)