T. Comont

Newly diagnosed immune thrombocytopenia adults: clinical epidemiology, exposure to treatments and evolution. Results of the CARMEN multicenter prospective cohort

The clinical epidemiology of immune thrombocytopenia (ITP) is not well known in adults. This study was aimed at assessing the clinical epidemiology of incident ITP adults, the factors associated with chronicity and exposure to treatments. This study was conducted in the CARMEN registry, a multicentric prospective cohort aimed at including all newly diagnosed ITP adults in the French Midi‐Pyrénées region, South of France (3 million inhabitants) from June 2013. Descriptive analyses and multivariate logistic regression models were conducted. Out of 121 newly diagnosed ITP until December 2014, 113 patients were followed in the region and gave informed consent. Median age was 65 years. Half of the patients were female, 20.3% had a secondary ITP, 50.4% had a Charlsons score ≥1, median platelet count was 17 × 109/L; 50.9% had bleeding symptoms, including 2 severe gastrointestinal tract and 1 intracranial bleedings; 21.4% had another autoimmune disease and 20.3% experienced an infection within the six weeks before ITP onset. Persistency and chronicity rates were 68.2% and 58.7%, respectively. Antinuclear antibodies were associated with chronicity (OR: 2.89, 95% CI: 1.08‐7.74). Sixty‐eight (60.2%) patients were treated during the week following the diagnosis. Factors associated with the use of intravenous corticosteroids were secondary ITP and high bleeding score. Those associated with the use of intravenous immunoglobulin (IVIg) were a high bleeding score and low platelet count. In conclusion, severe bleeding is rare at ITP onset. Associated autoimmune diseases and recent infections were frequent. Antinuclear antibodies seem predictors of chronicity. Intravenous corticosteroids and IVIg were frequently used.

Platelet transfusion refractoriness in patients with acute myeloid leukemia treated by intensive chemotherapy

Platelet transfusion refractoriness (PTR) is a major adverse event in the management of acute myeloid leukemia (AML). In a series of 897 adult patients with AML receiving intensive chemotherapy, we identified 41 patients (4.8%) with PTR. PTR was more frequently observed in parous women, patients with extra-medullary disease, a low white blood cell count, an infection, or hemophagocytic syndrome. Among the 31 patients with anti-human leucocyte antigen (HLA) antibodies, an HLA-matched donor was identified for 18 patients (58.1%). Median time between diagnosis of PTR and the first HLA-matched transfusion was 12.5days. HLA-matched transfusions induced a significant increment in platelet counts in 37% of cases. Thrombopoietin receptor agonists were given to 10 patients but did not shorten the duration of thrombocytopenia, reduce severe bleeding, or early death. Grade 3-4 bleeding events during induction, early death caused by bleeding, and death caused by bleeding at any time were significantly greater in patients that had platelet transfusion refractoriness (22% vs. 4.1%, P<0.0001; 12.2% vs. 1.4%, P=0.0006; and 24.4% vs. 5.3%, P<0.0001; respectively). PTR during chemotherapy for AML significantly increased the risk of early and late deaths caused by a severe bleeding event. Improved understanding of platelet destruction is needed to design mechanism-based therapeutic strategies.

Re: Severe Primary Autoimmune Thrombocytopenia (ITP) in Pregnancy: a national cohort study Primary immune thrombocytopenia management during pregnancy. A French study

Sir, We welcome the letter by Algeri et al. and their insightful reflections about a particular subset of non-tubal ectopic pregnancies reported in our recently published series. As highlighted by the Italian group, cornual–interstitial pregnancies are especially challenging, from achieving the correct diagnosis to choosing the best treatment option. We should be aware that uncertainty in the diagnosis may potentially lead to overtreatment and unnecessary morbidity. Some of these cases will be nonviable pregnancies that will resolve spontaneously or be amenable to uterine curettage, as illustrated in the first and second cases presented. Moreover, a precise differential diagnosis with an angular pregnancy will avoid intervention in a normal ongoing pregnancy that is only implanted eccentrically in the uterine cavity. This was suspected following the second ultrasound in the third case presented, but later dismissed based on magnetic resonance imaging findings. Cases classified as angular pregnancies are not ectopic pregnancies and, as such, have not been included in our series. Nonetheless, in keeping with the experience reported, we are occasionally confronted with the same diagnostic dilemma. At our centre we do have access to three-dimensional/ mutiplanar transvaginal ultrasound and we agree that obtaining a true coronal view of the uterus is helpful in some cases. In our experience, for the asymptomatic and haemodynamically stable patient, sequential examinations performed by the same team of experienced examiners are essential to determine whether the pregnancy is growing towards the cavity or into the interstitial space with further ballooning of this region. Again here, there are no codified practice guidelines and the balance is thin between early intervention to minimise risks and an expectant approach to avoid an unnecessary procedure. We are glad to hear that ultrasoundguided injection will be considered as an option by more centres following the publication of our series and that this is acknowledged as an effort to provide evidence-based guidance in the choice of treatment options for nontubal ectopic pregnancies.&

Pretreatment with standard-dose intravenous methylprednisolone does not improve outcomes in newly diagnosed immune thrombocytopenia (ITP)

To assess the benefits and harms to initiate corticosteroids with intravenous methylprednisolone at a conventional dose (1 mg/kg/d) to treat adults with immune thrombocytopenia (ITP).

Severe haemophagocytic lymphohistiocytosis triggered by a visceral leishmaniasis in a patient with a Rosai-Dorfman disease

Dear Editor,Haemophagocytic lymphohistiocytosis (HLH) may be trig-gered by various infectious or non-infectious conditions (1).HLH has rarely been associated with visceral leishmaniasis(VL) (2), and as far as we know, never with a sinushistiocytosiswithmassivelymphadenopathy(Rosai-Dorfman(RD) disease (3)).We report a case of life-threatening HLH associated withVL in a patient suffering from a RD disease and highlight thepotential connections between HLH and histiocytosis.On24December2012,a64-year-oldmanpresentedwitha3-week history of high-grade fever, night sweats and weightloss. He was just returning from a 2-month stay in Moroccowhere he was born, but he had been living in France for30 years. Clinical examination showed bilateral cervicallymph nodes that were known for several years with recentenlargement and hepatosplenomegaly. Laboratory blood testsshowed haemoglobin 8 g/dL, platelets 8 G/L, leukocytes1.7 G/L, low fibrinogen 0.7 g/L, elevated ferritin, LDH,triglyceride and C reactive protein levels to 8000 ng/mL,1200 UI/L, 2.25 mmol/L and 54 mg/L andhypergammaglobulinemia of 21 g/L, respectively. CD4 Tcellcount was 245/mm