"How I manage"

Cyclic thrombocytopenia (CTP) is a rare disease, which is characterized by periodic fluctuation of the platelet count. The pathogenesis of CTP is unknown and most likely heterogeneous. Patients with CTP are almost always misdiagnosed as having primary immune thrombocytopenia (ITP). The interval between ITP and CTP diagnosis can be many years. CTP patients often receive ITP-specific therapies including corticosteroids, thrombopoietin receptor agonists, rituximab, and splenectomy, which are followed by a transient increase in platelet count that is wrongly attributed to treatment effect with inevitable "relapse...

No abstract text is available yet for this article.

Women with inherited bleeding disorders, including carriers of hemophilia A and B, or with von Willebrand disease, have an increased risk of bleeding during pregnancy and delivery. The unborn child may also be affected by the bleeding disorder for which specific measures have to be considered. This requires a multidisciplinary approach, with a team that includes a hematologist, a pediatric hematologist, a clinical geneticist, an obstetrician-perinatologist, and an anesthesiologist. An optimal approach includes prepregnancy genetic counseling, prenatal diagnostic procedures, and a treatment plan for delivery for both the mother and child...

No abstract text is available yet for this article.

Targeted therapies for chronic lymphocytic leukemia (CLL) include venetoclax, the oral inhibitor of B-cell lymphoma-2, and inhibitors of kinases in the B-cell receptor signaling pathway, like Bruton tyrosine kinase and phosphatidylinositol 3 kinase. Randomized clinical trials clearly demonstrated improved progression-free survival with targeted therapy over chemoimmunotherapy in first-line and treatment of relapsed/refractory CLL. Comparative trials of venetoclax-based vs other targeted therapies have not been conducted...

Patients with myeloproliferative neoplasms (MPNs), such as polycythemia vera, essential thrombocythemia, and primary myelofibrosis, are at increased risk for arterial and venous thrombosis/thromboembolism. In particular, the risk of splanchnic venous thrombosis, such as portal vein thrombosis or Budd-Chiari syndrome, is significantly higher in patients with MPN than in the normal population. At the same time, MPN patients are at increased risk for severe bleeding. Therefore, the treatment of patients with MPN must be based on their suspected probability of thrombosis/thromboembolism and bleeding...

In this concise review, we discuss some common clinical challenges in the management of patients with cancer-associated venous thromboembolism (VTE), a frequent complication in patients with cancer that increases morbidity and mortality. While direct oral anticoagulants (DOACs) have been established in clinical practice for anticoagulation in patients with VTE without cancer, their efficacy and safety in patients with cancer have not been assessed in randomized controlled trials until recently. The choice of the appropriate anticoagulant agent in the era of DOACs to treat patients with cancer-associated VTE is based on balancing the risk of recurrence against the risk of bleeding, and potential drug-drug interactions...

Malignant pleural mesothelioma is a rare disease originating from mesothelial cells of the pleura and is related to asbestos exposure. The tumor is generally extended at the time of diagnosis and the treatment consists of a systemic palliative therapy. Radical approach is limited to very selected patients and is performed in expert centers but without validated schema. Radiotherapy alone is mainly used in palliative intent. Platinum-based chemotherapy in association with pemetrexed is the frontline standard of care and provides a 12-month overall survival...

Natural killer (NK) cell deficiency (NKD) is a subset of primary immunodeficiency disorders (PID) in which an abnormality of NK cells represents a major immunological defect resulting in the patient's clinical immunodeficiency. This is distinct from a much larger group of PIDs that include an NK cell abnormality as a minor component of the immunodeficiency. Patients with NKD most frequently have atypical consequences of herpesviral infections. There are now 6 genes that have been ascribed to causing NKD, some exclusively and others that also cause other known immunodeficiencies...

Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Most patients with mild and moderate VWD can be treated effectively with desmopressin. The management of severe VWD patients, mostly affected by type 2 and type 3 disease, can be challenging. In this article we review the current diagnosis and treatment of severe VWD patients. We will also discuss the management of severe VWD patients in specific situations, such as pregnancy, delivery, patients developing alloantibodies against von Willebrand factor and VWD patients with recurrent gastrointestinal bleeding...

Type 2 diabetes (T2D) is an evolving disease that requires therapeutic adjustments to maintain adequate glucose control in the long run. An increasing number of patients with T2D are treated with a metformin plus gliptin (DPP-4 Inhibitor) combination, especially those for whom a sulfonylurea is avoided because of a risk of hypoglycaemia. When this dual metformin-gliptin therapy becomes insufficient to reach or maintain adequate glucose control, three solutions may be considered : the addition of a gliflozin (SGLT2 inhibitor), the replacement of the gliptin by a glucagon-like peptide-1 receptor agonist or the addition of a basal insulin whose posology should be progressively up-titrated according to fasting glycaemia...

No abstract text is available yet for this article.

Recent advances in the treatment of follicular lymphoma (FL) have provided insight into molecular and biological influences on pathogenesis and prognosis. Additionally, numerous available treatment strategies for both newly diagnosed and relapsed disease require thoughtful consideration of patient selection to avoid the burden of overtreatment and toxicities. This review provides a broad overview on our approach to managing patients with low grade FL.

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Anticoagulant therapy is the most effective strategy to prevent arterial and venous thromboembolism, but treating older individuals is challenging, because increasing age, comorbidities, and polypharmacy increase the risk of both thrombosis and bleeding. Warfarin and non-vitamin K antagonist oral anticoagulants are underused and often underdosed in the prevention of stroke in older patients with atrial fibrillation because of concerns about the risk of bleeding. Poor adherence to anticoagulant therapy is also an issue for older patients with atrial fibrillation and those at risk of recurrent pulmonary embolism...

Wiskott Aldrich syndrome (WAS) is a primary immunodeficiency disease resulting in recurrent infections, eczema and microthrombocytopaenia. In its classical form, significant combined immune deficiency, autoimmune complications and risk of haematological malignancy necessitate early correction with stem cell transplantation or gene therapy. A milder form, X-linked thrombocytopaenia (XLT), shares similar bleeding risk from thrombocytopaenia but is not associated with other significant clinical features and is generally managed conservatively...

The benefits of homework on student learning and academic achievement, to a large extent, depend on the degree of student engagement. Motivational engagement (my intention or why I do the homework), cognitive engagement (how I get involved in homework), and behavioral engagement (how much homework I do, how much time I devote to it, how I manage that time) are key aspects that condition the quality of the process of doing homework, learning, and academic achievement. Prior academic achievement is one of the variables that seems to be positively related to student engagement (both due its motivational component and to the training to do homework)...

Diamond-Blackfan anaemia (DBA) is a rare inherited marrow failure disorder, characterized by hypoplastic anaemia, congenital anomalies and a predisposition to cancer as a result of ribosomal dysfunction. Historically, treatment is based on glucocorticoids and/or blood transfusions, which is accompanied by significant toxicity and long-term sequelae. Currently, stem cell transplantation is the only curative option for the haematological DBA phenotype. Whereas this procedure has been quite successful in the last decade in selected patients, novel therapies and biological insights are still warranted to improve clinical care for all DBA patients...

Drug therapy failure may occur with any medical intervention. It is badly accepted by both the physician and the patient. Drug failure may be attributed to the physician, the patient, the medication or the severity of the disease itself. Once drug failure is confirmed, causes should rapidly be identified in order to find a solution because several ones may often be offered.

No abstract text is available yet for this article.