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Peripheral T cell lymphomas are rare malignancies with aggressive course, with several different subtype described in the 2016 WHO classification. Their distribution across the world is heterogenous, with marked difference between Western and Asian country. Their clinical presentation often comprise extra-nodal involvement, B symptoms and immune system disorder which can lead to wrong diagnosis orientation. Make a right diagnosis need a experienced pathologist in close collaboration with clinical datas. Peripheral T cell lymphomas are in general associated with poor prognosis when treated with anthracyclines-based regimen, and several studies and trials focused on the use of intensified regimen or novel targeted agents, whose proper indication still remain to be clarified., (Copyright © 2020. Published by Elsevier Masson SAS.)

Modern day treatment of blood cancers is characterised by the introduction of targeted therapies. Alone or in combination with more traditional treatments, administered in a hospital or an outpatient setting, these new medicines possess specific mechanisms of action and particular side effects in addition to or instead of those of traditional treatments. To understand the changes to the care management of patients taking these new treatments, it is necessary to describe their mechanism of action and their side effects., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

We report a case of KSHV/EBV associated germinotropic lymphoproliferative disorder (LPG) in a 49-year-old African patient, without immunosuppression. LPG is a rare entity arising in immunocompetent patients in opposition to other lymphoproliferative disorders associated to Kaposi sarcoma-associated herpes virus (KSHV). The disease presents itself as localized lymphadenopathy with an infiltration of germinal centers by plasmablastic cells coinfected by KSHV and EBV (Epstein-Barr Virus). After treatment, the outcome is favorable. Differential diagnosis in our case, due to the presence of clusters of Hodgkin-like cells in the mantle zone, included lymphocyte rich classic Hodgkin lymphoma (LHCRL) and nodular lymphocyte predominant Hodgkin lymphoma (LHNPL). Finally, we highlight the differential diagnostic criteria of KSHV lymphoproliferative diseases., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Thousands of autologous and at less extent allogeneic hematopoietic stem cells (HSC) bags are cryopreserved in France. The majority of autologous HSC grafts are used within a year after collection. However, many bags are still unused and cryopreserved for many years. In France and on a European scale, the ever-growing number of cryopreserved bags represents a real economic health concern. Indeed, the cost of storage is about 100€ per bag and per year. In addition, quality and therapeutic value of these long-term cryopreserved grafts needs to be evaluated. In the attempt to harmonize clinical practices between different French transplantation centers, the French Society of Bone Marrow Transplantation and Cell Therapies (SFGM-TC) set up its fourth annual series of workshops which brought together practitioners from its member centers across France. These workshops took place in September 2013 in Lille. In this article, we addressed the issue of the destruction of long-term cryopreserved grafts be them autologous or allogeneic and provide recommendations regarding their destruction., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

In the attempt to harmonize clinical practices between different French transplantation centers, the French Society of Bone Marrow Transplantation and Cell Therapy (SFGM-TC) set up the third annual series of workshops which brought together practitioners from all member centers and took place in October 2012 in Lille. Here we report our results and recommendations regarding the management of common issues related to the donor: pre-transplant pregnancy and monoclonal gammopathy., (Copyright © 2013. Published by Elsevier SAS.)

In the attempt to harmonize clinical practices between different French transplantation centers, the French Society of Bone Marrow Transplantation and Cell Therapy (SFGM-TC) set up the third annual series of workshops which brought together practitioners from all member centers and took place in October 2012 in Lille. Here we report our results and recommendations regarding the management of pre-transplant donor's cytomegalovirus, Epstein-Barr virus, Toxoplasma gondii, or syphilis IgM positive serology test., (Copyright © 2013. Published by Elsevier SAS.)

Introduction: The recent discovery of the Janus Kinase 2 (JAK2(V617F)) mutation, an indicator of clonal expansion, has widely modified the diagnostic work-up of myeloproliferative disorders. However, histopathologic criteria of the WHO classification, focused on megakaryocytes abnormalities, have taken a central role in the diagnosis of essential thrombocytemia (ET), particularly to distinguish it from the prefibrotic myelofibrosis (PMF) evolving to a clinic myelofibrosis unlike true ET. The aim of our study is to evaluate inter-observer reproducibility and prognostic implications of these pathological criteria comparing diagnoses with clinical and biological follow-up., Methods: Forty-four patients presenting with isolated thrombocytemia were retrospectively evaluated. All patients were initially diagnosed with ET based on the PVSG classification from 1997. The initial bone marrow biopsy specimens were re-evaluated using the Thiele pathological classification: true ET, prefibrotic myelofibrosis (PMF) and early myelofibrosis. Patients were followed for a median of 6 years., Results: Our population includes three patients with polycythemia vera and 41 patients with true ET. Based on clinical and biological follow-up, diagnoses were changed to idiopathic myelofibrosis in four of six patients with "early" myelofibrosis (66.7%), but in only one of 14 patients with PMF (7.15%). In contrast, the diagnosis of true ET was not changed in the 21 cases. Inter-observer reproducibility for pathological classification was 100%., Conclusion: In this study, our diagnostic methodology based on the clinico-biological follow-up, which has not been evaluated in previous studies, calls into question the diagnostic value of the pathological criteria in PMF., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

JACIE (Joint accreditation committee for ISCT Europe and EBMT) is a comprehensive quality management system built in under the auspices of two European professional societies. The singularity of JACIE is its coverage of all the main actors - clinical wards as well as collection and processing facilities - that contribute to a hematopoietic stem cell transplantation program. Deployment of JACIE started ten years ago. A recent retrospective analysis of the large-size european registry of autologous and allogenic hematopoietic stem cell transplantations demonstrates that one of the factors affecting the overall survival of recipients of allogenic transplantation is the status of the transplant program regarding JACIE accreditation. This provides one of the first demonstrations that introduction of a quality management system can contribute to the overall survival of patients treated with a highly specific and complex medical procedure.

Twelve years ago, rituximab, a chimeric monoclonal anti-CD20 antibody, became available for the treatment of relapsing follicular lymphoma. It has resulted in an improvement of approximately 20% in survival for nearly all B cell lymphomas. In follicular lymphoma, rituximab combined with chemotherapy is indicated as first-line treatment when treatment is necessary. It is also an interesting option, alone or in combination with chemotherapy for the treatment of relapses, after verifying the persistence of CD20 expression on the surface of malignant cells. As a maintenance treatment after chemotherapy with or without rituximab, it improves relapse-free survival and overall survival. The standard treatment for high-grade diffuse lymphoma, regardless of age and regardless of predicted severity, is now the combination of CHOP chemotherapy and rituximab. The combination of fludarabine, cyclophosphamide, and rituximab is currently the reference treatment for chronic lymphoid leukemia as first-line treatment for patients with few comorbidities and for those with relapses. The role of this antibody in the treatment of more serious forms of lymphoma is under evaluation. The administration of rituximab, alone or in combination, in the treatment of low-grade non-follicular lymphoma must be decided as a function of existing protocols in multidisciplinary consultations. New-generation anti-CD20 antibodies, designed to improve still further the already remarkable efficacy/toxicity ratio, are now taking the road that rituximab opened.

The management of non-Hodgkin's lymphoma (NHL) has changed considerably over the last 15 years with the recognition of independent prognostic factors leading to the development of a predictive model of outcome, the International Non-Hodgkin's Lymphoma Prognostic Factors Index (IPI) and more recently gene array studies. Since then, the therapeutic approaches have tried to improve the outcome of patients with adverse prognostic factors with the building of intensified regimens rather than the classical CHOP regimen. The introduction of monoclonal antibodies (MoAb) into the management of NHL has dramatically improved the response rates and rituximab is now approved for the treatment of diffuse large B-cell lymphoma and follicular lymphoma. Subsequently, MoAb have been conjugated to radioisotopes to target radiotherapy to tumor sites and improve overall survival. The advantages of these radiolabeled antibodies are not only to enhance the therapeutic potency of MoAb by targetting specifically CD20+ tumour cells but also to protect the neighbouring normal organs from cytotoxicity. Finally, the initial staging as well as the evaluation of the response after treatment has been better defined with the introduction of positron emission tomography (PET) into the assessment of NHL. The accuracy of PET imaging in the detection of residual disease and extranodal localizations appears to be the most helpful non-ivasive modality in NHL. The aim of this review is to focus on recent and most significant improvements in the management of lymphomas.

Allogeneic stem cell transplantation is able to cure many hematologic malignancies, through, at least partially, a graft versus disease effect of the donor's immune system transfer. However, the toxicity of this technique limits its use to selected patients. The aim of non-myeloablative stem cell transplantation is to reduce the toxicity of the conditioning regimen while allowing the engrafement of donor's stem cells and the immunological antitumoral activity of the donor's immune system. Several reports have already demonstrated the validity of this concept. This new multi-step therapeutic strategy is complex, raises many questions and deserves further studies to be fully applicable to a greater number of patients.

The neutropenic patient, whatever the cause of neutropenia (drug related, secondary to marrow involvement by malignant disease or to cytotoxic treatment) is at high risk of bacterial and fungal infection. The first symptom (and often the only one) of infection is fever. The guidelines for management of the febrile neutropenic patient are precise and simple and have been widely accepted for more than 30 years allowing a dramatic decrease of the infectious mortality during episodes of neutropenia.

Purpose: Cryptococcus is an opportunistic infection that affects immunodepressed patients and is a classical complication of AIDS-stage HIV infection. The aim of this study was to investigate Cryptococcus neoformans infections in patients with hematological malignancies., Methods: Six cases have been described of cryptococcosis detected in Nantes, France over the past 10 years in patients with hematological malignancies., Results: This infection has been found particularly in the context of lymphoproliferative disorders (chronic lymphocytic leukemia, Waldenström macroglobulinemia, Hodgkin's disease, and non-Hodgkin's lymphoma), and also following cytotoxic therapy. In four cases, the patients were treated with fludarabine, which rapidly caused long-duration marked lymphocytopenia, notably in CD4 cells. Cell-mediated immunity plays a major role in systemic defense against C. neoformans. It therefore seems that fludarabine favors the spread of cryptococcal infections., Conclusion: In the context of lymphoproliferative syndromes treated with cytotoxic drugs, in particular fludarabine, it appears important to take into account the possible presence of cryptococcal infection in the presence of respiratory, neurological or cutaneous disorders, so that a correct diagnosis can be made and the appropriate treatment administered.

Introduction: The outcome of neutropenic patients with Pseudallescheria boydii infection is poor., Exegesis: We report the first case of Pseudallescheria boydii hip arthritis in a patient treated for acute lymphoblastic leukemia. In vitro susceptibility testing showed that the strain was resistant to amphotericin B, fluorocytosine and nystatin, but susceptible to itraconazole. The patient received oral itraconazole (600 mg/day) and clinical symptoms initially resolved. Two months later, after a course of chemotherapy and high-dose steroids while receiving oral itraconazole treatment, the patient developed fever, skin lesions and disseminated lung infiltrates due to Pseudallescheria boydii and finally died., Conclusion: This case illustrates the severity of fungal infections due to Pseudallescheria boydii despite a presumably well-conducted antifungal therapy.

Patients with hematology disease in post-chemotherapy medullary aplasia are susceptible to life-threatening bronchopulmonary mycoses, especially aspergillosis. Other less common pulmonary mycoses are also observed. We report 4 cases due to Penicillium purpurogenum, Acromonium strictum and Scedosporium apiospermum (n = 2) infections. These unusual fungi appear to be emerging as pathogens in this population. They have common ubiquitous, opportunistic and low pathogenicity characteristics in immunocompetent subjects. The major risk factor is neutropenia. Isolating one of these fungi in an immunodepressed patient modifies management protocols since certain unusual fungi such as Scedosporium are resistant to amphotericin B.

As a frequent complication of immunosuppression, lymphoproliferative disorders affect approximately 2 % of allorgan transplant recipients. Most of them are EBV-associated-B-cell-lymphoproliferations. Other types of non Hodgkin's lymphomas and Hodgkin's disease, as observed in general population, have only rarely been reported in this group of patients. We report three cases of Hodgkin's disease, which were diagnosed in two renal transplant recipients and one heart transplant patient. They were associated with Epstein-Barr virus, as demonstrated by immunohistochemistry and in situ hybridization. EBV is frequently associated with Hodgkin's disease in the general population, and always implicated in AIDS-related Hodgkin's disease. However in transplant patients the rarity of Hodgkin's disease argues against a direct oncogenic role of this virus.

In the absence of systemic chemotherapy after the diagnosis of primary granulocytic sarcoma (PGS), all patients will subsequently develop acute myelogenous leukemia (AML). The authors describe a case of PGS of the head of the pancreas found at laparotomy in a 32-year-old man. The patient received early after surgery, two courses of high-dose chemotherapy and, with a follow-up of 2 years, never developed AML. This rare observation illustrates the necessity of further early systemic chemotherapy after surgical excision of PGS.

The authors report three cases of fusarial infection in neutropenic patients with hematologic malignancies. The first patient was affected by a cutaneous extensive fusariosis. The second patient developed a fusarial lung infection during a multiple organ failure following allogenic bone marrow transplantation. The third patient who presented with refractory acute myelogenous leukemia, developed fusarial skin lesions, and died from pulmonary failure. The treatment of fusarial infection is disappointing and requires amphotericin B, in association with hematopoietic growth factors. The role of new agents, or combination chemotherapy remains to be determinated. The recovery of adequate neutrophil levels is the most important factor in the resolution of fusarial infection.

Objectives: To evaluate the toxicity and effectiveness of piperacillin+tazobactam and amikacin compared with a reference treatment with ceftazidime and amikacin given as first line therapy in neutropenic patients with fever., Methods: A multicentric randomized trial was conducted in 222 adults who had fever (38 degrees C for > 3 h) during a period of aplasia (white cell count < 0.5.10(9)/l for 22.9 +/- 10.4 days) induced by chemotherapy for acute leukaemia (68.1%) or by bone marrow autograft for lymphoma, myeloma or solid tumour (30.3%). 109 patients were assigned to the piperacillin (12 g/d)/tazobactam (1.5 g/d)+amikacin group and 113 to the ceftazidime (3 g/d)+amikacin group. Evaluation criteria were the frequency of apyrexia after a 72-hour antibiotic regimen and major infectious events defined as death due to infection and severe infections causing a delay in the chemotherapy protocol., Results: Data obtained in 188 patients who fulfilled all the protocol criteria were evaluated. The episode of fever was controlled better with the piperacillin/tazobactam+amikacin combination (apyrexia achieved in 60.6% of the patients vs 44.7% in the ceftazidime+amikacin group, p = 0.028) and there were fewer superinfections (23% vs 41% respectively, p < 0.008). Tolerance was similar in the two groups. In vitro, 56% of the strains resistant to piperacillin and isolated prior to treatment were sensitive to the piperacillin/tazobactam combination. Among the strains isolated (41 Gram-, 61 Gram+), 72% were sensitive to ceftazidime and 84% were sensitive to the piperacillin/tazobactam combination. There were 16 deaths due to infection (8.5%) with no difference according to antibiotic regimen. There was no difference in toxicity., Conclusion: Tolerance was similar in the two groups. A combined regimen of piperacillin/tazobactam can be proposed as first line treatment for neutropenic patients with fever.

Prophylactic systemic antibiotherapy with ceftriaxone (CRO) alone was tested in aplastic patients receiving total gut decontamination and treated in protected environment. To enter the study, the patients had to be afebrile when their polymorphonuclear (PMN) count fell under 500/cumm. Seventy eight therapeutic aplasias (after allogeneic or autologous bone marrow transplant conditioning regimens or high dose chemotherapy) form the basis of this report. The median duration of aplasia was 19 D (11-93 D). Forty-three patients received during 51 aplasias one single injection of CRO per day as soon as PMN count was under 500/cumm. In 23 cases (45%) the patients remained afebrile until the end of aplasia. There were 3 Staphylococcus epidermidis bacteremias (6%), 3 bacteriologically documented fevers (6%) and 1 Cryptococcus septicemia. Twenty-nine of these aplasias were part of a randomized study between group A (prophylactic CRO) and group B (non prophylactic CRO: 27 cases). In group A, there were significantly more aplasias without fever (34.5% vs 4%), and less bacteremias (10% vs 48%). Fever appeared later in group A (mean 12.5 D vs 6 D). No death was recorded during the whole study. Thus, in protected environment, prophylactic systemic antibiotherapy could still lessen the risk of bacterial infections. The side effects and the cost of such a procedure appeared to be diminished by a monoantibiotherapy.

Six patients (aged 39 to 55 years) with stage III myelomas were treated with IV high dose melphalan (HDM) 140 mg/m2. They had previously received only one or two courses of conventional chemotherapy. The extrahematological tolerance of the procedure was good. The duration of neutropenias (less than 500/mm3) was 14 to 24 days. Complete remission (CR) was obtained in 4 cases but one patient died in CR of invasive aspergillosis. In the other 3 cases, bone marrow was cryopreserved without ex vivo treatment and reinfused after a 2nd course of HDM. These patients remain in apparent CR 12, 20 and 34 months after the diagnosis although one of them had a pure meningeal relapse treated with a second autograft. These results suggest that CR can be obtained with HDM but at the expense of profound myelosuppression. Long term disease control can be obtained but the role of the 2nd course of HDM with autologous bone marrow transplantation warrants further study.

Prophylactic systemic antibiotic therapy with ceftriaxone alone was tested in aplastic patients receiving total gut decontamination and treated in a protected environment. Only patients who were afebrile when their polymorphonuclear (PMN) count fell below 500/cumm were admitted to the study. Seventy-eight episodes of therapeutic aplasia (consecutive to allogeneic or autologous bone marrow transplant conditioning regimens or to high dose chemotherapy) form the basis of this report. The median duration of aplasia was 19 days (range 11-93 days). Twenty patients received, during 22 episodes of aplasia, one single injection of ceftriaxone per day as soon as their PMN count was below 500/mm3. In 13 cases (59%) the patients remained afebrile until the end of aplasia, and no bacteriemia was detected. The second part of the study was randomized between group A (prophylactic ceftriaxone: 29 cases) and group B (no prophylactic ceftriaxone: 27 cases). Patients in group A had significantly more episodes of afebrile aplasia (34.5% vs 4%) and less bacteriemias (10% vs 48%) than those in group B. Also fever developed later in group A (mean: 12.5 vs 6 days). No death was recorded throughout the study. Thus, in a protected environment prophylactic systemic antibiotic therapy could still lessen the risk of bacterial infection. Using one single antibiotic seemed to reduce the side-effects and cost of the prophylactic treatment.

A case of eosinophilic fasciitis (EF) associated with Hodgkin disease is described. In eight previously published cases, EF was associated with immunologic diseases, such as autoimmune marrow aplasia. The relation between EF and Hodgkin disease should be studied by investigating the physiopathologic meaning of eosinophils in both conditions.

Acute febrile neutrophilic dermatosis, so called Sweet's Syndrome is a distinct dermatological disease defined by constant clinical and histological features: Eruption of painful, tender, raised erythematous plaques of face and neck with fever. Dense dermal infiltration with mature neutrophil polymorphs. Sweet's Syndrome may occur during the course of chronic or acute haematological diseases such as chronic myelogenous leukemia or acute non lymphoblastic leukemia. In all cases, the counts of Neutrophil Polymorphs were normal or above normal limits. We report a case of Sweet's Syndrome occurring during the aplastic period induced by the treatment of an acute myelo monocytic leukemia, and discuss the responsibility of white blood cells transfusion in genesis of typical histological aspect.