113 research outputs found

    APLASTIC ANEMIA AND VIRAL HEPATITIS

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    <p class="MsoNormal" style="margin: 0cm 0cm 0pt; line-height: 200%; text-align: justify;"><span style="font-size: small;"><span style="font-family: Times New Roman;">Acquired aplastic anemia(aAA) is a severe <span style="mso-ansi-language: EN-GB;" lang="EN-GB">and rare disease, characterized by hematopoietic bone marrow failure and peripheral cytopenia. The pathophysiology is immune mediated in most cases, activated T1 lymphocytes have been identified as effector cells . The disease can be successfully treated with combined immunosuppressive therapy or allogeneic hematopoietic stem cell transplantation.</span></span></span></p><p class="MsoNormal" style="margin: 0cm 0cm 0pt; line-height: 200%; text-align: justify;"><span style="mso-ansi-language: EN-GB;" lang="EN-GB"><span style="font-size: small;"><span style="font-family: Times New Roman;">Hepatitis-associated aplastic anemia (HAA)<span style="mso-spacerun: yes;">  </span>is a syndrome of bone marrow failure following the development of acute seronegative hepatitis. HAA syndrome most often affects young males who presented severe pancytopenia two to three months after an episode of acute hepatitis. The clinical course of hepatitis is more frequently benign but a fulminant severe course is also described. The bone marrow failure can be explosive and severe and it is usually fatal if untreated, no correlations have been observed between severity of hepatitis and AA.</span></span></span></p><p class="MsoNormal" style="margin: 0cm 0cm 0pt; line-height: 200%; text-align: justify;"><span style="mso-ansi-language: EN-GB;" lang="EN-GB"><span style="font-size: small;"><span style="font-family: Times New Roman;">In none of the<span style="mso-spacerun: yes;">  </span>studies a specific virus could be identified and most cases are seronegative for known hepatitis viruses. The clinical characteristics<span style="mso-spacerun: yes;">  </span>and response to immunotherapy indicate a central role for immune-mediated mechanism in the pathogenesis of HAA. The initial target organ of the immune<span style="mso-spacerun: yes;">  </span>response is the liver as suggested by the time interval between hepatitis and the onset of bone marrow failure.</span></span></span></p><p class="MsoNormal" style="margin: 0cm 0cm 0pt; line-height: 200%; text-align: justify;"><span style="mso-ansi-language: EN-GB;" lang="EN-GB"><span style="font-size: small;"><span style="font-family: Times New Roman;">Liver histology is characterized by T cell infiltrating the parenchyma as reported in acute hepatitis. </span></span></span></p><p class="MsoBodyText" style="margin: 0cm 0cm 0pt;"><span lang="EN-GB"><span style="font-size: small; font-family: Times New Roman;">Recently in HAA it has been demonstrated intrahepatic<span style="mso-spacerun: yes;">  </span>and blood lymphocytes with<span style="mso-spacerun: yes;">  </span>T cell repertoire similar to that of confirmed viral acute hepatitis. The expanded T cell clones return to a normal distribution after response to immunosuppressive treatment, suggesting the antigen or T cell clearance. Therapeutic options are the same as acquired aplastic anemia.</span></span></p&gt

    Recombinant factor VIIa for the management of severe hemorrhages in patients with hematologic malignancies

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    Seven patients with hematologic malignancies were treated with recombinant activated factor VII (rFVIIa) for severe bleeding episodes complicating diagnostic procedures or high-dose chemotherapy associated or not with stem cell transplantation. All patients were thrombocytopenic and refractory to standard support. After administration of rFVIIa, 2 complete responses, 3 partial responses and 2 failures were documented

    Rituximab in post allogeneic hematopoietic stem cell transplantation membranous nephropathy: a case report.

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    Membranous nephropathy (MN) post allogeneic hematopoietic stem cell transplantation (HSCT) is a rare complication with few long-term outcome data. We describe the clinical course and outcome of an adult female patient who developed MN after allogeneic HSCT for follicular non-Hodgkin's lymphoma. MN was treated with methylprednisolone as first-line therapy, then she was changed to rituximab for a relapse. After treatment with rituximab, we observed a progressive decrease of proteinuria and normalization of serum albumin. Seven months after treatment, she remains in remission. No adverse reactions to rituximab were observed throughout follow-up

    Development of cellulitis caused by Aeromonas hydrophila in allogeneic hematopoietic transplantation: a case report.

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    The Aeromonas species are Gram-negative bacteria that are commonly found in aquatic environments. Aeromonas hydrophila is the most frequently isolated in humans and has been responsible for a variety of infections: gastrointestinal syndromes, skin and soft tissue infections, sepsis and other less common syndromes

    Aspergillus niger infection in patients with haematological diseases: a report of eight cases

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    In this paper we analysed clinical, laboratory characteristics and outcome of patients with haematological diseases who developed an Aspergillus niger infection, in a multicentre study involving 14 Italian Haematological Divisions during a 10-year period. The study recorded 194 consecutive microbiologically documented aspergilloses, eight of which (4%) were due to A. niger, and were observed only in five of the participating centres. The primary localization of infection was lung in seven cases and paranasal sinus in one case. Seven patients died at the end of follow-up. The death was mainly attributable to A. niger progression in six of them. Our study that collected the largest number of cases of A. niger infection in haematological malignancies confirms that this infrequent complication is characterized by a high mortality rate

    Hepatitis B reactivation characterized by HBsAg negativity and anti-HbsAg antibodies persistence in haematopoietic stem cell transplanted patient after lamivudine withdrawal

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    HBV reactivation is associated with high mortality rates in hematopoietic stem cell transplantation (HSCT) and prophylactic lamivudine (LMV) treatment is suggested to prevent this phenomenon. However, the duration of LMV treatment in HSCT patients is not fully defined and the time of immune recovery is considered the best parameter for a drug to be safely interrupted. In patients undergoing allogeneic HSCT, the time of immune recovery is not easy to define and may take years after transplantation and prolonged LMV treatments, which can lead to drug-resistant viral strains

    Fibrin glue directly applied on damaged bladder mucosa during cystoscopy is highly effective to treat severe, refractory, haemorrhagic cystitis after allogeneic transplant

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    Fibrin glue directly applied on damaged bladder mucosa during cystoscopy is highly effective to treat severe, refractory, haemorrhagic cystitis after allogeneic transplant M.C. Tirindelli (1), G. Flammia (1), L. Cudillo (2), T. Dentamaro (3), A. Picardi (2), O. Annibali (1), A. Tendas (3), L. Cupelli (3), C. Nobile (1), F. Marchesi (1), R. Cerretti (2), M. Mirabile (2), G. De Angelis (2), K. Girardi (1), P. De Fabritiis (3), G. Avvisati (1), W. Arcese (2) (1)University Campus Bio-Medico (Rome, IT); (2)Tor Vergata University (Rome, IT); (3)S. Eugenio Hospital (Rome, IT) Background: Hemorrhagic Cystitis (HC) occurring after hemat- opoietic stem cell transplant (HSCT) significantly affects quality life of patients, prolongs hospitalization and in some cases can become a life-threatening complication. Its management has not been established. Fibrin Glue (FG) is a hemostatic agent derived from human plasma with proven efficacy in repairing damaged tissues. Study design and methods: This study included patients who met the following criteria: grade ≥3 HC not responding to hyper- hydration, bladder irrigation, antiviral treatments and transfusion support. FG was obtained using Vivostat system, an automatic method for processing and applying FG. During conventional cystoscopy and maintaining bladder distension by a CO2 insuf- flator, FG was accurately sprayed through a specific applicator on bleeding mucosa. FG polymerized on contact and set over several days. The response to the treatment was defined com- plete (CR) for disappearance of hematuria, partial (PR) for at least one grade regression of HC and no response (NR). Results: From Jan 06 to Oct 09, 626 patients undergoing an autologous (n=428) or allogeneic (n=198) HSCT were regis- tered at the RTN. No autologous HSCT recipients developed HC of severe grade, whereas 18 of 198 patients (9%) undergo- ing an allogeneic HSCT met the criteria to enter the study. These 18 patients (6 M, 12 F) with a median age of 32.5 years (range, 18-53) had been submitted to a HSCT from HLA identical sib. (n=4), unrelated CB (n=4), MUD (n=2) or related haploiden- tical donor (n=8) for different hematological malignancies. All patients, deeply immunosuppressed with positive BKV viruria >7x106 copies/ml, developed a very severe HC, refractory to all current treatments including antiviral therapy. At time of FG application, HC persisted for a median of 16 days (range, 7-65) and was grade 3 and 4 in 14 and 4 patients, respectively. The number of FG applications was 1 in 15 patients, 2 in 2 and 3 in 1 patient for a median of 11 mL (range, 6.3-16.2) of glue. The treatment was successful in 16 out of 18 patients (89%). All 14 patients with grade 3 HC responded and the response was complete in 12 (86%) and partial in 2 (14%), while of the 4 patients with grade 4 HC: 1 achieved CR, 1 PR and 2 NR. No patient died of HC. Conclusions: FG therapy is a feasible, safe, easy repeatable, not invasive, small time consuming, lightly expensive and highly effective procedure in treating severe, refractory post- transplant HC
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