Dorota Zduńczyk

Medical University of Warsaw, Warszawa, Masovian Voivodeship, Poland

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Publications (7)6.35 Total impact

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    ABSTRACT: Wstęp: Zespół hemofagocytowy (HLH – limfohistiocytoza hemofagocytowa) to rzadki zespół objawów hiperzapalenia charakteryzujący się gorączką, splenomegalią, obecnością cytopenii, znacznej hiperferrytynemii, hemofagocytozy oraz hipertriglicerydemii lub hipofibrynognenemii. Objawy te są efektem samonapędzającej się „burzy cytokinowej”, która przy braku leczenia nieuchronnie prowadzi do zgonu. Do niedawna zespół ten był rozpoznawany jedynie u dzieci. Od kilku lat znacząco wzrosła świadomość jego występowania także u dorosłych–pacjenci z zespołem HLH są diagnozowani i leczeni w wielu ośrodkach hematologicznych. Cel: Celem pracy jest przedstawienie po raz pierwszy wyników HLH u pacjentów dorosłych w populacji polskiej. Materiał i metody: Dane dorosłych (�18. rż.) pacjentów z rozpoznaniem HLH zostały zebrane z 11 ośrodków. Wyniki:30pacjentów spełniało 5 kryteriów HLH-2004. Ponieważ istnieje możliwość oceny tylko 6 z 8 zalecanych kryteriów (aktywność limfocytów NK i stężenie sCD25 nie są wykonywane), dodatkowo do analizy włączono 8 pacjentów z dużym podejrzeniem klinicznym, którzy spełniali 4 kryteria. Wśród pacjentów, u których udało się znaleźć czynnik wywołujący HLH, u10był to wirus EBV (z czego u jednego pacjenta wykryto także chłoniaka PTCL, natomiast u kolejnego była to reaktywacja po allogenicznym przeszczepieniu komórek krwiotwórczych z powodu ALL). Oprócz tych dwóch chorych nowotwór wykryto u 14 pacjentów, najczęściej był to chłoniak (10/14), przeważnie pochodzący z limfocytów T (6/10), pozostałe 4 przypadki to DLBCL. U trzech pacjentów rozpoznano reumatologiczną odmianę HLH–zespół aktywacji makrofagów (MAS). Charakterystycznym odchyleniem jest bardzo wysoka hiperferrytynemia–mediana 13 365 ug/l (850–117 650). Obserwowano także hipertriglicerydemię322 mg/dl (74–865), zwiększoną aktywność AST 116 U/l (16–3338), ALT 88 U/l (18–1857) i LDH 674 U/l (195–13 160). Leczenie przyczynowe było oparte na schemacie HLH-2004 (etopozyd, deksametazon, cyklosporyna A) lub terapii choroby podstawowej. Spośród badanych chorych żyje obecnie 42%, mediana ich obserwacji wynosi 24 miesiące (1–92). Wnioski: Wysoka hyperferrytynemia powinna sugerować diagnostykę w kierunku HLH. Wśród potencjalnych przyczyn HLH zawsze należy poszukiwać nowotworu (zwłaszcza chłoniaka).
    Full-text · Conference Paper · Sep 2015
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    ABSTRACT: In patients with immunological disorders, adenovirus infections are associated with significant rates of morbidity and mortality. Only few hematological units use molecular virological methods, such as polymerase chain reaction, for surveillance of adenovirus infection, and treatment strategies have never been evaluated in multicenter clinical trials. This report describes the detection and treatment of human adenovirus (HAdVs) disseminated disease in the case of a 46-year-old immunocompromised female having myelodysplastic syndrome with refractory cytopenia with multilineage dysplasia: International Prognostic Scoring System 1. Serum and urine samples were tested for the presence of adenoviral DNA using the quantitative real-time polymerase chain reaction (PCR) assay. For additional confirmation, sequencing of PCR products was also performed. With real-time PCR, we detected HAdV DNA in both serum and urine samples. The viral level constantly decreased with applied oral ribavirin therapy. As the result of sequencing, HAdVs type 11 was determined. Surveillance of adenovirus by real-time PCR is useful in detecting and monitoring disseminated HAdV infection; it is a potential standard diagnostic approach that could assist clinicians to decide whether antiviral therapy ought to be administered.
    Full-text · Article · Oct 2011 · Archivum Immunologiae et Therapiae Experimentalis
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    ABSTRACT: In patients with immunological disorders, adenovirus infections are associated with significant rates of morbidity and mortality. Only few hematological units use molecular virological methods, such as polymerase chain reaction, for surveillance of adenovirus infection, and treatment strategies have never been evaluated in multicenter clinical trials. This report describes the detection and treatment of human adenovirus (HAdVs) disseminated disease in the case of a 46-year-old immunocompromised female having myelodysplastic syndrome with refractory cytopenia with multilineage dysplasia: International Prognostic Scoring System 1. Serum and urine samples were tested for the presence of adenoviral DNA using the quantitative real-time polymerase chain reaction (PCR) assay. For additional confirmation, sequencing of PCR products was also performed. With real-time PCR, we detected HAdV DNA in both serum and urine samples. The viral level constantly decreased with applied oral ribavirin therapy. As the result of sequencing, HAdVs type 11 was determined. Surveillance of adenovirus by real-time PCR is useful in detecting and monitoring disseminated HAdV infection; it is a potential standard diagnostic approach that could assist clinicians to decide whether antiviral therapy ought to be administered.
    Full-text · Article · Jan 2011
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    ABSTRACT: Hemophagocytic lymphohistiocytosis (HLH) is a rare (especially in adults) syndrome in which hyperinflammation leads to fever, hepatosplenomegaly, pancytopenia and when untreated leads to death. Characteristic laboratory findings include: hyperferritinemia, hypertrigliceridemia, hypofibrinogenemia, hemophagocytosis, low/absent NK-cell activity and high serum sCD25 levels. HLH in adults is in most cases secondary to viral infection (with an Epstein-Barr virus predominacy). A very rare case of T-cell lymphoma-induced HLH in an 80 year old patient is reported. This diagnosis was made when patient was already in critical condition and although after introducing treatment according to the HLH-2004 protocol (etoposide, cyclosporine A, corticosteroids) signs of improvement were observed, the outcome was fatal. Described case shows usefulness of more frequent ferritin concentration measurements in similar patients. Extremely high (> 10 000 μg/l) hyperferritinemia is a hallmark of HLH and may enable control of this syndrome which is 100% fatal without treatment. Full text available at: http://6096.indexcopernicus.com//fulltxt.php?ICID=971600
    Full-text · Article · Jan 2011
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    ABSTRACT: Epstein-Barr virus (EBV) is the major triggering factor for hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH). In patients with EBV-HLH, the EBV-infected T cells or natural killer cells are mostly mono- or oligoclonally proliferating, whereby hypercytokinemia plays a major role and causes hemophagocytosis, cellular damage, and dysfunction of various organs. This report describes the detection and treatment of EBV-associated HLH in the case of a 17-year-old male. Serum samples and skin swabs were tested for the presence of viral DNA using real-time PCR techniques. To confirm the molecular biological tests, electron microscopy was also performed. EBV DNA was detected with real-time PCR in both blood samples and skin swabs. The level of viral DNA constantly decreased during the applied therapy. The presence of the virus in the skin was confirmed by the appearance of herpes virus-like particles detected by electron microscopy in fluid taken from skin ulcerations. The results show that in terms of treatment, special therapeutic measures are required to control the cytokine storm generated by EBV and to suppress proliferating EBV genome-containing cells because the clinical course is often fulminate and results in a poor outcome. Therefore the potential of chemotherapy with a combination of steroids, etoposide, and cyclosporine to control HLH was assessed in the adolescent, who met the stringent diagnostic criteria for this reactive disorder of the mononuclear phagocyte system.
    Full-text · Article · Apr 2010 · Archivum Immunologiae et Therapiae Experimentalis
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    ABSTRACT: Background: Plerixafor is a CXCR4 receptor inhibitor, which was recently introduced for stem cell mobilization in myeloma and lymphoma patients prior to their transplantation. Since March 2009, we performed 16 mobilizations using plerixafor in combination with G-CSF in 10 patients with multiple myeloma, 3 Non-Hodgkin's lymphoma and 3 Hodgkin's lymphoma, who failed previous mobilization attempts with G-CSF in combination with chemotherapy. Methods and Findings: Our protocol consisted of daily s.c. injections of G-CSF (2 × 5 μg/kg) on days 1 through 7 and plerixafor (240 μg/kg) on day 4, 5 and 6. In three patients, plerixafor was added to chemotherapy-based mobilization regimen, in case when No. of CD34+ cell was too low to start cell collections. The median No. of circulating CD34+ cells after first administration of plerixafor was 23/μL (range 11-62) and in 13/16 patients it exceeded minimum of 15 cells/μL required to begin leukapheresis on that day. However, due to high peripheral blood leukocytosis (median 36.5 G/L; range, 11.4-72.5) the frequency of CD34+ cells was low (median 0.067%, range 0.030-0.215) that affected low collection efficiency of CD34+ cells. Moreover, this required collection and freezing of abundant No. of nucleated cells (median 9.3 × 10 8 NCs/kg, range 6.15-24.05). In our setting, high nucleated cell count translated into high volume of stem cell product (median 1260 mL; range 500-2050). Nevertheless, the final stem cell products contained median of 2.8 × 10 6 CD34+ cells/kg b.w. (range, 0.57-4.5 × 10 6) and in 12/16 patients (75%) it exceeded 2.0 × 10 6 CD34+ cells/kg b.w., which is required for stem cell transplantation. Eight patients have already been transplanted and median time to neutrophil (>0.5 G/L) recovery was 12 days (11-14) and platelet (>20 G/L) recovery was 14 days (10-25) that is satisfactory. Conclusions: Stem cell mobilization with plerixafor and G-CSF provides solution for majority of patients requiring autologous hematopoietic stem cell transplantation and failing mobilization with G-CSF in combination with chemotherapy. However, due to high leukocytosis, this protocol requires modification of stem cell collection and freezing procedures in order to avoid large volumes of stem cell product.
    No preview · Article · Jan 2010
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    ABSTRACT: A case of an 18-year-old male affected with hemophagocytic lymphohistiocytosis (HLH), in whom Epstein-Barr virus (EBV, HHV-4) was detected in skin rash, is presented. Macular mucocutaneous lesions were primarily observed. The lesions spread over the body and subsequently generalized maculo-papular rash with blister formation was observed. A combination of steroids, etoposide and cyclosporine were administered with positive therapeutic result.
    No preview · Article · Jan 2007