Hematopoietic Recovery using Multi-Cytokine Therapy in 8 Patients Presenting Radiation-Induced Myelosuppression after Radiological Accidents.

Treatment of accidental radiation-induced myelosuppression is primarily based on supportive care and requires specific treatment based on hematopoietic growth factors injection or hematopoietic cell transplantation for the most severe cases. The cytokines used consisted of pegylated erythropoietin (darbepoetin alfa) 500 IU once per week, pegylated G-CSF (pegfilgrastim) 6 mg × 2 once, stem cell factor 20 µg.kg-1 for five days, and romiplostim (TPO analog) 10 µg.kg -1 once per week, with different combinations depending on the accidents. As the stem cell factor did not have regulatory approval for clinical use in France, the French regulatory authorities (ANSM, formerly, AFSSAPS) approved their compassionate use as an investigational drug "on a case-by-case basis". According to the evolution and clinical characteristics, each patient's treatment was adopted on an individual basis. Daily blood count allows initiating G-CSF and SCF delivery when granulocyte <1,000/mm3, TPO delivery when platelets <50,000/mm3, and EPO when Hb<80 g/L. The length of each treatment was based on blood cell recovery criteria. The concept of "stimulation strategy" is linked to each patient's residual hematopoiesis, which varies among them, depending on the radiation exposure's characteristics and heterogeneity. This paper reports the medical management of 8 overexposed patients to ionizing radiation. The recovery of bone marrow function after myelosuppression was accelerated using growth factors, optimized by multiple-line combinations. Particularly in the event of prolonged exposure to ionizing radiation in dose ranges inducing severe myelosuppression (in the order of 5 to 8 Gy), with no indication of hematopoietic stem cell transplantation.

[Association between multiple myeloma and acute myeloid leukemia secondary to myelodysplastic syndrome]. / Association d'un myélome multiple et d'une leucémie aiguë myéloïde secondaire à un syndrome myélodysplasique.

We report a rare case of association of two distinct hematologic malignancies: refractory cytopenia with multilineage dysplasia associated with del(5q) and symptomatic multiple myeloma associated with del(17p) and del(13q). After 16 months, the patient presented an acute leukemic transformation of the myelodysplasic syndrome.

[Light-chain escape from plateau phase]. / Échappement des chaînes légères de la phase de plateau.

We report the first case of light-chain escape from plateau phase occurring after 7 years of evolution of multiple myeloma in a patient who had never received new molecules. A very good partial response was obtained.

[Signification of Epstein-Barr virus detection in the cerebrospinal fluid of a patient with human immunodeficiency virus related Burkitt lymphoma]. / Signification de la détection du virus d'Epstein-Barr dans le liquide céphalorachidien d'un patient suivi pour un lymphome de Burkitt lié au virus de l'immunodéficience humaine.

The significance of Epstein-Barr virus detection in the cerebrospinal fluid of patients with Burkitt lymphoma is poorly studied. We report the case of a patient with immunodeficiency associated Burkitt lymphoma in complete remission who presented 5 months after the end of treatment, an isolated optic neuritis. Lumbar puncture found lymphocytic meningitis and the viral load of Epstein-Barr virus was 234,000 copies per milliliter in the cerebrospinal fluid. These symptoms could be explained by Epstein-Barr virus meningoencephalitis but the detection of MYC rearrangements in the cerebrospinal fluid confirms the diagnosis of Burkitt lymphoma cerebral relapse. The detection of the Epstein-Barr virus DNA in the cerebrospinal fluid should be interpreted with caution.

[Plasmacytoïd dendritic cells acute leukemia: a case report]. / Leucémie aiguë dérivée des cellules dendritiques plasmacytoïdes : à propos d'un cas.

We describe a case of a patient hospitalized in haematology unit for treatment to blastic plasmacytoid dendritic cell neoplasm. Apart skin lesions found at diagnosis in 83% of patients, few elements suggest the diagnosis. Cytology is not characteristic and no cytogenetic abnormality is specific to the LpDC, the karyotype shows generally at least three cytogenetic abnormalities. Definitive diagnosis rests to identification of a blastic population with immunophenotype CD4+ CD56+. This leukemia is chemosensitive but the relapse rate is important and the survival time is 16 months.

Impact of rituximab and/or high-dose therapy with autotransplant at time of relapse in patients with follicular lymphoma: a GELA study.

PURPOSE: The treatment of patients with follicular lymphoma has changed with the introduction of high-dose therapy (HDT) with autologous stem-cell transplant then with rituximab. The effect of these two strategies on the outcome of relapsing patients with follicular lymphoma has never been compared. PATIENTS AND METHODS: We analyzed two cohorts of patients treated in two successive randomized studies with the same treatment, cyclophosphamide, doxorubicin, teniposide, and prednisolone plus interferon, to evaluate the role of rituximab and HDT in salvage therapy after first disease progression or relapse. RESULTS: Of the 364 patients included in these two studies, 254 progressed or relapsed and constitute the population of this analysis. Among them, 98 had been treated with HDT, including 33 of them after rituximab-containing salvage regimen, and 69 with rituximab alone or combined with chemotherapy but without HDT. Patients' characteristics at diagnosis were similar in all subgroups. If event-free survival was identical for patients treated within Groupe d'Etude des Lymphomes Folliculaires (GELF) -86 or GELF-94 studies, overall survival was longer in GELF-94 study. HDT was associated with a statistically significant benefit in terms of event-free survival from relapse and survival after relapse (SAR). Rituximab was associated with a greater benefit than HDT for these two end points. When both treatments were combined, patients treated with rituximab-containing salvage regimen followed by HDT had 5-year SAR more than 90%. CONCLUSION: In follicular lymphoma, for patients treated with first-line chemotherapy the combination of a salvage regimen containing rituximab with or without HDT leads to a dramatic improvement of long-term outcome.

Intensive chemotherapy followed by hematopoietic stem-cell rescue for refractory and recurrent primary CNS and intraocular lymphoma: Société Française de Greffe de Moëlle Osseuse-Thérapie Cellulaire.

PURPOSE: The prognosis of relapsing primary CNS lymphoma (PCNSL) is poor. We report the results of a prospective multicenter trial of intensive chemotherapy followed by autologous hematopoietic stem-cell rescue (IC + HCR) in immunocompetent adult patients with PCNSL or intraocular lymphoma (IOL) after failure of high-dose methotrexate-based treatment. PATIENTS AND METHODS: Salvage treatment consisted of two cycles of high-dose cytarabine and etoposide (CYVE). Intensive chemotherapy combined thiotepa, busulfan, and cyclophosphamide. Forty-three patients (median age, 52 years; range, 23 to 65 years) were included, with relapse (n = 22), refractory disease (n = 17), or a partial response to first-line treatment (n = 4). The response to CYVE was not assessable in three cases because of treatment-related death. Twenty patients (47%) were chemosensitive to CYVE: 15 of them proceeded to IC + HCR. IC + HCR was also administered to 12 patients who did not respond to CYVE. All but one of the 27 patients who underwent IC + HCR entered complete remission. RESULTS: With a median follow-up of 36 months, the median overall survival was 18.3 months in the overall population, and 58.6 months among patients who completed IC + HCR. The respective median progression-free survival (PFS) times after IC + HCR were 11.6 and 41.1 months. The 2-year overall survival probability was 45% in the whole population and 69% among the 27 patients who received IC + HCR. The 2-year PFS probability was 43% among all the patients and 58% in the IC + HCR subpopulation. CONCLUSION: IC + HCR is an effective treatment for refractory and recurrent PCNSL.

[Efficacy of rituximab in hairy cell leukemia]. / Efficacité du rituximab dans la leucémie à tricholeucocytes.

INTRODUCTION: Rituximab is an alternative treatment for hairy cell leukemia, when the standard treatments are unavailable. CASE: An 82-year-old woman was diagnosed with hairy cell leukemia. The severity of her neutropenia ruled out purine analogs, while heart disease and age both contraindicated use of interferon. Rituximab, in four weekly treatments, was effective from the first treatment and the positive response was sustained thereafter. DISCUSSION: Rituximab may be used to treat hairy cell leukemia, especially for patients refractory to purine analogs or after relapse or in the case of severe neutropenia, when interferon is contraindicated.

[Spontaneous hematoma of the iliac psoas muscle in chronic myeloid leukemia. A case report]. / Hématome spontané du muscle psoas iliaque au cours de la leucémie myéloïde chronique. A propos d'un cas.

Spontaneous hematoma in the ilio-psoas muscle is an uncommon condition, usually observed as a complication of anticoagulation or hemophilia. Clinically, the onset is marked by violent pain in the territory of femoral nerve and/or psoitis. The diagnosis is confirmed by echography or CT-scan. The most serious complications are loss of self-sufficiency and neuro-muscular after-effect. Surgery is recommended in patients with neurological suffering, followed by early physiotherapy. We report the case of a 42-year-old man, with an ilio-psoas muscle hematoma, revealing chronic myeloid leukemia, without any hemostasis disorder.

[Hairy-cell leukemia: 30 cases and a review of the literature]. / La leucémie à tricholeucocytes. Mise au point à partir de l'étude de 30 observations.

From 1978 to 2002, 30 patients presenting hairy-cell leukemia were seen in two different hospitals. We reviewed clinical, biological and therapeutic data. At diagnosis, the median age was 67.8 years; 47% were clinically asymptomatic, 12 patients had anaemia, 15 thrombocytopenia (platelets<100,000/mm3). A treatment was required for 29 patients. At the end of the study, 27 patients are alive and none died because of the disease. This study confirms the good prognosis of hairy-cell leukemia, especially since the advent of new therapeutics such as purine analogs. Based on the results in this series, we examined the different aspects of the disease referring to diagnosis, treatment and prognosis.