Hepatitis B And C In Hematopoietic Stem Cell Transplant

Although the risk of acquisition of hepatitis B or hepatitis C virus through blood products has considerably reduced since the last decade, some infected patients are candidates to stem cell transplantation. Others may have no alternative than an infected donor. In all these cases, recipients of transplant are prone to short and long term liver complications. The evolution of liver tests under chemotherapy before transplant may give useful information to anticipate on the risk of hepatitis reactivation after transplant, both for HBv and HCv. More than sixty percent of the patients who are HBsAg-positive before transplant reactivate after transplant, and 3% develop acute severe liver failure. Because both viral replication and immune reconstitution are the key factors for reactivation, it is crucial to closely follow liver function tests and viral load during the first months of transplant, and to pay a special attention in slowly tapering the immunosuppression in these patients. Lamivudine reduces HBv viremia, but favors the emergence of HBv polymerase gene mutants and should be individually discussed. Both in case of HBv or HCv hepatitis reactivation with ALT ≥ 10N concomitantly to an increase in viral load at time of immune reconstitution, steroids should be given. In case there is no alternative than a HBv or HCv positive geno-identical donor, the risk of viral hepatitis, including acute liver failure and late complications, should be balanced with the benefit of transplant in a given situation

Subcutaneous panniculitis-like T-cell lymphoma presenting with diffuse cutaneous edema in a 2-year-old child

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Primary Prophylaxis of Invasive Fungal Diseases in Allogeneic Stem Cell Transplantation: Revised Recommendations from a Consensus Process by Gruppo Italiano Trapianto Midollo Osseo (GITMO)

Abstract This document updates and expands the recommendations on primary prophylaxis of invasive fungal diseases (IFD) in allogeneic hematopoietic stem cell transplantation (allo-HSCT) recipients, published in 2009 by the Gruppo Italiano Trapianto Midollo Osseo (GITMO). A consensus process was undertaken to describe and evaluate current information and practice regarding risk stratification and primary antifungal prophylaxis during the pre-engraftment and postengraftment phases after allo-HSCT. The revised recommendations were based on the evaluation of recent literature including a large, prospective, multicenter epidemiological study of allo-HSCT recipients conducted among the GITMO transplantation centers during the period of 2008 to 2010. It is intended as a guide for the identification of types and phases of transplantation at low, standard, and high risk for IFD, according to the underlying disease, transplantation, and post-transplantation factors. The risk stratification was the critical determinant of the primary antifungal approach for allo-HSCT recipients

Molecular analysis of Fanconi anemia: the experience of the Bone Marrow Failure Study Group of the Italian Association of Pediatric Onco-Hematology

Fanconi anemia is an inherited disease characterized by congenital malformations, pancytopenia, cancer predisposition, and sensitivity to cross-linking agents. The molecular diagnosis of Fanconi anemia is relatively complex for several aspects including genetic heterogeneity with mutations in at least 16 different genes. In this paper, we report the mutations identified in 100 unrelated probands enrolled into the National Network of the Italian Association of Pediatric Hematoly and Oncology. In approximately half of these cases, mutational screening was carried out after retroviral complementation analyses or protein analysis. In the other half, the analysis was performed on the most frequently mutated genes or using a next generation sequencing approach. We identified 108 distinct variants of the FANCA, FANCG, FANCC, FANCD2, and FANCB genes in 85, 9, 3, 2, and 1 families, respectively. Despite the relatively high number of private mutations, 45 of which are novel Fanconi anemia alleles, 26% of the FANCA alleles are due to 5 distinct mutations. Most of the mutations are large genomic deletions and nonsense or frameshift mutations, although we identified a series of missense mutations, whose pathogenetic role was not always certain. The molecular diagnosis of Fanconi anemia is still a tiered procedure that requires identifying candidate genes to avoid useless sequencing. Introduction of next generation sequencing strategies will greatly improve the diagnostic process, allowing a rapid analysis of all the genes

Therapeutic decision making in BMT/SCT for severe aplastic anemia

Etiology Idiopathic (most frequent type of aplastic anemia) Chemical and physical agents Infections (e.g., viral hepatitis, EBV, HIV) Congenital Disease stagin

Therapeutic decision making in BMT/SCT for severe aplastic anemia

Etiology page 197 Disease staging 198 Results with conventional therapy 198 Results with allogeneic transplant 201 Indications for allogeneic transplant 203 Pretransplant workup 203 Monitoring posttransplant 204 Further reading 204 Etiology “Idiopathic” (most frequent type of aplastic anemia) Chemical and physical agents (a) Agents that regularly produce aplasia if dose is sufficient cytotoxic drugs ionizing radiation benzene and related agents inorganic arsenic (b) Agents that occasionally produce aplasia (idiosyncratic) chloramphenicol sulfonamides, penicillins, tetracyclines quinacrine diphenylhydantoin tolbutamide phenylbutazone, indomethacin carbamazepine acetylsalicylic acid chlorpromazine gold compounds penicillamine hair dyes solvents, DDT cimetidine 3. Infections (e.g., viral hepatitis, EBV, HIV) 4. Congenital Fanconi anemia Shwachman-Diamond syndrome Amegakaryocytic thrombocytopenia Disease staging Results with conventional therapy The two main therapeutic options for patients with severe aplastic anemia are combination immune suppressive treatment or allogeneic transplantation, if a suitable donor is available. A protocol for combination immune suppressive treatment is as follows (Frickhofen et al., 2003): Methylprednisolone, 5 mg/kg on days 1 to 8 (single oral dose or IV infusion over 30 minutes); then prednisolone, 1 mg/kg/day for days 9 to 14; then taper the dose until day 29. The incorporation of steroids is important as it prevents or ameliorates serum sickness. The dose used in EBMT protocols is somewhat lower: 1 to 2 mg/kg/day for 2 weeks, 0.5 mg/kg during the third week, tapering to 0 during the fourth week. Antithymocyte globulin (ATG) (e.g., ATGAM, Pharmacia & Upjohn/ Pfizer, produced in horses), 20 mg/kg IV over 6 hours on days 1 to 8. […