Scleral lenses for severe chronic GvHD-related keratoconjunctivitis sicca: a retrospective study by the SFGM-TC.

Chronic GvHD-related keratoconjunctivitis sicca (cGvHD-related KCS) can significantly alter the quality of life of patients after allogeneic hematopoietic stem cell transplantation. The aim of this work was to assess the efficacy and tolerability of scleral lenses to treat severe cGvHD-related KCS. In this retrospective, multicenter study, we included 60 consecutive patients diagnosed with cGvHD-related KCS and fitted with scleral lenses. Patients were evaluated at baseline and at 2 months with the following tests: the Ocular Surface Disease Index (OSDI) to assess quality of life, the Oxford score to grade corneal damage and the logarithm of minimal angle of resolution (Log MAR) scale to determine visual acuity. We observed improvement in quality of life in 58 patients (97%). All parameters improved at 2 months. We observed significant differences at 2 months compared with baseline for the mean OSDI (86 versus 30, respectively, P<0.001), the mean Oxford score (3.2 versus 1.3, respectively, P<0.001) as well as visual acuity (Log MAR of 0.33 versus 0.10, respectively, P<0.001). Treatment with scleral lenses was discontinued in only 5 patients (8%) with a median follow-up of 20.5 months (range: 2-125 months). Scleral lenses were very efficient and well tolerated in patients with severe cGvHD-related KCS.

A retrospective series of gut aspergillosis in haematology patients.

Gut invasive aspergillosis is an extremely rare infection in immunocompromised patients. The goal of this retrospective multicentre study is to report on cases of gut aspergillosis in haematology patients, including clinical presentation, risk factors, and outcome. Twenty-one patients from nine centres were identified. Eight had isolated gut aspergillosis, with no evidence of other infected sites, and 13 had disseminated aspergillosis. Thirteen patients had acute leukaemia. Nine were allogeneic stem cell transplant recipients. Clinical symptoms and imaging were poorly specific. The galactomannan antigenaemia test result was positive in 16/25 (64%) patients, including in four of the eight cases of isolated gut aspergillosis. Five of 21 patients had a dietary regimen rich in spices, suggesting that, in these cases, food could have been the source of gut colonization, and then of a primary gut Aspergillus lesion. The diagnosis was made post-mortem in six patients. The mortality rate in the remaining patients at 12 weeks was 7/15 (47%). Gut aspergillosis is probably misdiagnosed and underestimated in haematology patients, owing to the poor specificity of symptoms and imaging. Patients with a persistently positive galactomannan antigenaemia finding that is unexplained by respiratory lesions should be suspected of having gut aspergillosis in the presence of abdominal symptoms, and be quickly investigated. In the absence of severe abdominal complications leading to surgery and resection of the lesions, the optimal treatment is not yet defined.

[Acute respiratory distress revealing acute myeloblastic leukaemia: case report]. / Détresse respiratoire aiguë révélant une leucémie aiguë myéloblastique. À propos d'un cas.

We report on the case of a patient diagnosed with acute leukaemic transformation of chronic myelomonocytic leukaemia. Its development was characterised by blastic pulmonary localisation and response to corticosteroids. We discuss the etiologies of respiratory distress in acute myeloblastic leukaemia and the corticosteroid sensitivity of this myeloid disease.

[Rhinocerebral infections in immunosuppressed patients with hematological disorders]. / Infections rhinocérébrales chez l'immunodéprimé en hématologie.

Invasive rhinosinusitis, occurring almost exclusively in immunosuppressed patients, is defined by the presence of local inflammation with vascular and osseous necrosis with extensive soft tissue extension. Imaging and bacteriological and even histological (mycelial filaments) criteria have been established. Because it can evolve to death in patients with hematological disorders, accurate diagnosis is important but difficult to achieve due to the non-specific nature of signs and symptoms. Imaging plays an important role with CT of the paranasal sinuses and MR imaging of the brain for early diagnosis and treatment in order to improve prognosis. We will present the imaging features of three cases of rhinocerebral infections, with two cases of fungal infection and one case of invasive bacterial sinus infection.

Which role for rituximab in hairy cell leukemia? Reflections on six cases.

Hairy cell leukemia (HCL) is a rare, chronic, B-cell, lymphoproliferative disorder. Treatment has been revolutionized by the advent of interferon (IFN)-alpha and purine analogs (PA). First-line therapy with PA yields complete response rates of 75-100%, with many long-lasting remissions. In the event of profound neutropenia and/or infectious complications, a short sequence of IFN-alpha may precede PA treatment. Because of the excellent results achieved with PA therapy, the potential role of rituximab (an anti-CD20 monoclonal antibody that is highly effective against most B-cell lymphomas) in HCL has yet to be elucidated. Six HCL cases treated with rituximab are reported herein with a view to elucidating the potential role of the drug in HCL. The indications essentially consist of relapsing or refractory disease, avoiding the cumulative toxicity of PA, consolidation therapy in order to eradicate minimal residual disease, and first-line therapy for patients with contraindications to PA and IFN-alpha.

[Hematological disorders and hypereosinophilias]. / Hyperéosinophilies et affections hématologiques.

Hematological disorders are the third cause of hypereosinophilia, after allergic and parasitic diseases. Hematological disorders associated with hypereosinophilias can be classified as clonal, reactive or idiopathic, and recently the improvements of cytogenetic, molecular biology and immunology have allowed to revisit numerous cases previously diagnosed as idiopathic hypereosinophilic syndrome. Reactive eosinophilias are mainly associated with lymphoma or abnormal, often clonal T lymphoid population. Clonal eosinophilia is related either to various myeloid malignancies or to a genuine myeloproliferative disorder from the eosinophile lineage, the so-called chronic eosinophilic leukaemia. Chronic eosinophilic leukaemia can be associated with recurrent genes rearrangements involving PDGFRA, PDGFRB and FGFR1 or with clonal abnormalities not yet categorized. Idiopathic hypereosinophilic syndrome remains an exclusive diagnosis in presence of moderate or severe unexplained eosinophilia with target organ damage. The purpose of the diagnostic work-up of hypereosinophilic syndrome is to evidence either an abnormal T cell population or a clonal haematopoiesis. Imatinib mesylate dramatically improves chronic eosinophilic leukaemias associated with PDGFR abnormalities, while corticosteroids are still the main treatment for the other patients. In a near future, advances could arise from identification of new genes involved in clonal eosinophilia or in alternative therapy such as the anti-IL-5 antibodies.

[Thrombotic thrombocytopenic purpura: a case report]. / Le purpura thrombotique thrombocytopénique: à propos d'un cas.

We report a case of thrombotic thrombocytopenic purpura (TTP) in a 60 years-old woman with Sjogren's syndrome. Symptomatology on admission leads to evoke the diagnosis of TTP. Biological results allow to set the diagnosis. Actually, association of haemolytic anaemia, schizocytes and thrombocytopenia are in favour of TTP. Undetectable ADAMTS 13 activity (below 5%) confirms the diagnosis. In congenital TTP, plasma ADAMTS 13 is absent or severely reduced as a consequence of mutations in the two ADAMTS 13 gene. In acquired TTP, circulating antibodies inhibit plasma ADAMTS 13 activity. In those cases, further biological studies are needed to find a cause of TTP. Follow-up implies standard laboratory tests. Plasma exchanges are progressively tapered after normalization of platelets count.

New biological indicators to evaluate and monitor radiation-induced damage: an accident case report.

The aim of this work was to use several new biological indicators to evaluate damage to the main physiological systems in a victim exposed accidentally to ionizing radiation. Blood samples were used for biological dosimetry and for measurement of the plasma concentrations of several molecules: Flt3 ligand to assess the hematopoietic system, citrulline as an indicator of the digestive tract, and several oxysterols as lipid metabolism and vascular markers. The cytogenetic evaluation estimated the dose to the victim to be between 4.2 and 4.8 Gy, depending on the methodology used. Monitoring the Flt3 ligand demonstrated the severity of bone marrow aplasia. In contrast, the citrulline concentration showed the absence of gastrointestinal damage. Variations in oxysterol concentrations suggested radiation-induced damage to the liver and the cardiovascular system. These results were correlated with those from classic biochemical markers, which demonstrated severe damage to the hematopoietic system and suggested the appearance of subclinical damage to the liver and cardiovascular system. These results demonstrate for the first time the importance of a multiparameter biological approach in the evaluation of radiation damage after accidental irradiation.

[Hepatosplenic and kidneys candidasis complicating an acute myeloblastic leukemia. A case treated with voriconazole and caspofungin]. / Candidose hépato-spléno-rénale compliquant une leucémie aiguë myéloblastique. A propos d'un cas traité par l'association voriconazole et caspofungine.

We report the observation of hepato-splenic and kidneys candidiasis complicating the chemotherapy of a myeloblastic leukemia (LAM5b). Following the lack of effectiveness of a first line treatement, using amphotericine B liposomale and 5-fluorocytosine, implementation of an association of new molecules, a triazole of second generation (voriconazole) and an echinocandine (caspofungine) has allowed a successful result.

[Hodgkin's disease with bone marrow involvement. The role of positron tomography]. / Maladie de Hodgkin avec atteinte médullaire. Intérêt de la tomodensitométrie par émissions de positons.

INTRODUCTION: Hodgkin's disease with initially predominant bone marrow involvement is observed in 1% of cases. OBSERVATION: A case of Hodgkin's disease with massive bone marrow invasion, manifested by pancytopenia in the context of fever and alteration in general status of health, with little or non superficial peripheral lymph nodes. The bone marrow biopsy revealed extensive myelofibrosis with rare Reed-Sternberg cells. DISCUSSION: This case report is inscribed within the framework of the exceptional massive bone marrow forms of Hodgkin's disease, described by Duhamel et al. in 1979. We believe that metabolic imaging with 18F-FDG positron emission tomography (PET) is a valid examination in orienting diagnosis and post-therapy assessment.

[A 7-year survey of strains identified in blood cultures in a clinical hematology unit]. / Etude sur sept ans des isolats d'hémocultures dans un service d'hématologie clinique.

GOAL: This study had for aim to analyze the epidemiology of strains identified in blood cultures (hôpital d'instruction des armées Percy, Clamart, France, hematology unit) to compare the rate of identified micro-organisms with literature data, and to search for a possible correlation between antibiotherapy management and evolution of resistance profiles. MATERIAL AND METHODS: All the micro-organisms (N = 690) collected over seven years (January 1996 to December 2002), from blood cultures of hospitalized patients in conventional and sterile sector were studied. RESULTS: Gram positive cocci rate (GPC) was 62.6% and Gram negative bacilli (GNB) 31.3%. Evolution in time showed a decrease of GPC and an increase of GNB, notably the non fermenting Gram negative bacilli, leading to an equal rate by 2001-2002. The most frequently identified species were Staphylococcus epidermidis (36.4%), Escherichia coli (8.7%), Pseudomonas aeruginosa (6.8%), and Staphylococcus aureus (4.9%). The rate of methicillin-resistant staphylococci was 63.6%. Fifty-five percent of E. coli strains had a penicillinase phenotype. Pseudomonas aeruginosa resistance was 8.5, 8.5, 6.4 and 8.5%, respectively for ceftazidime, piperacillin-tazobactam, imipenem, and amikacin. CONCLUSION: This study showed a tendency to inversion of former bacteremia epidemiology with increasing negative Gram bacilli. It justifies the antibiotherapy protocols adopted in the hematology unit.

[Secondary medullary aplasia from accidental radiation:therapeutic options and evolution of the concept]. / L'aplasie médullaire secondaire à un accident d'irradiation:options thérapeutiques et évolution des concepts.

Bone marrow grafting following accidental irradiation exposure should be viewed in the perspective of a severe myeloablative syndrome linked to high medullary damage for a dose range higher than 6-8 Gy, resulting in very late or no recovery. Prognosis will depend on the presence or absence of radio-combined injuries, the toxicity of the transplant procedure, and the risk of rejection induced by insufficient percritical immunosuppression. It is in this context that new cell therapy modalities, which combine enhanced peripheral hematopoietic cell engraftment and high immunosuppressive conditioning regimen with low extrahematological toxicity, inducing early and stable mixed lymphomyeloid chimerism with minimal morbidity, can be considered. Such an approach is being evaluated in the treatment of patients with hematological malignancies at high risk of transplant-related mortality using conventional bone marrow methods.

Dicentric chromosome 3 associated with binucleated lymphocytes in atypical B-cell chronic lymphoproliferative disorder.

Binucleated lymphocytes on blood smear are known in PPBL characterized by stable and polyclonal lymphocytosis, polyclonal increase of serum IgM, HLA DR7 and strong correlation with additional i(3q) and premature chromosome condensation. In this disorder some reports of clonal Ig rearrangement suggest a follow up of these patients with immunological and genetic studies. Binucleated lymphocytes are rarely described in other clonal B-CLPD as B-CLL or marginal zone B-cell lymphoma (MZL). Chromosome 3 abnormality is never described in B-CLL but trisomy 3 represents the most consistent abnormality characterizing the MZL. We report in a man without previous medical history an unusual B-CLPD with monoclonal lymphocytosis CD5-, characteristic cytology (particularly binucleated lymphocytes) and chromosomic abnormality as dicentric chromosome 3 never previously described in B-CLPD. In this case lymphocytosis is persistent and stable over 24 months, cytologic immunologic and chromosomic abnormalities are unchanged. We discuss the nosologic place of this atypical B-CLPD closely related to PPBL and MZL with at the moment, after 24 months, a quiet evolution that imply nevertheless a careful follow up with regular cytologic, immunological and genetic studies to clarify the issue.

[Non-Hodgkin lymphoma mimicking renal carcinoma: apropos of 1 case of follicular lymphoma]. / Lymphome non hodgkinien simulant un carcinome rénal. A propos d'un cas de lymphome folliculaire.

Primary renal non-Hodgkin's lymphoma (NHL) is unusual, in contrast to the frequent renal involvement in disseminated NHL. We report a case of follicular lymphoma presenting initially as a renal mass. In the literature, twenty-seven similar cases have been described since 1980. The median age at diagnosis is 64 years with a male predominance. Clinical and radiological findings generally evoke renal carcinoma. Histologically, tumors are usually large B-cell lymphomas. The existence of renal non-Hodgkin lymphoma mimicking renal carcinoma must be recognized. Such lymphomas can either be primitive or be nodal with a renal presentation. Nephrectomy followed by chemotherapy permits long disease free survival.

Validity of anthropometric equations for the estimation of body density in adolescent athletes.

One hundred forty-one adolescent male (age: X +/- SD = 17.43 +/- 0.96 yr) and 133 adolescent female (16.51 +/- 1.39 yr) athletes were studied to determine the validity of available anthropometric equations for estimating body density (BD). Anthropometric measures were taken of eight skinfolds, nine circumferences, and four diameters. Body density, determined by underwater weighing (corrected for residual lung volume), was 1.080 +/- 0.010 g X ml-1 for the males and 1.066 +/- 0.010 g X ml-1 for the females. Cross-validation of 17 selected equations on the males revealed validity coefficients ranging from R2 = 0.29-0.67 and total-error scores ranging from 0.0062-0.0277 g X ml-1. Among the females, cross-validation of 15 selected equations revealed validity coefficients ranging from R2 = 0.31-0.67 and total-error scores ranging from 0.0064-0.0214 g X ml-1. While equations of either linear or quadratic form demonstrated acceptable accuracy in the estimation of male BD values, only quadratic equations displayed similar levels of accuracy among the females. Based on these results, as well as consideration of characteristics related to inter-tester error and the variance of predicted scores, the quadratic equations of Jackson and Pollock, Lohman, and Pollock et al., as well as the linear equation of Forsyth and Sinning, were found to be particularly appropriate for the estimation of BD in adolescent athletes.

Body composition and somatotype characteristics of junior Olympic athletes.

Body composition and somatotype were determined in Junior Olympic competitors to evaluate the structural characteristics concomitant to high proficiency in various athletic activities. Underwater weighings and anthropometric determinations of somatotype were performed on 145 male and 133 female adolescent participants in national meet competition in the sports of track and field, gymnastics, diving, and wrestling. The most frequent differences within either the male or female Junior Olympic samples involved the performers in throwing events (shot put, discus, and javelin), who were taller, heavier, fatter, and of unique somatotype when compared to all or most other competitors. Additional structural differences, generally of a lesser magnitude, also existed between other groups of Junior Olympians. Differences in body composition characteristics were also noted when Junior Olympians were compared with other adolescent athletes or non-athletes.