Case report: VEXAS syndrome: an atypical indolent presentation as sacroiliitis with molecular response to azacitidine.

VEXAS syndrome is a recently described autoinflammatory syndrome caused by the somatic acquisition of mutations in myeloid precursors and is frequently associated with hematologic malignancies, chiefly myelodysplastic syndromes. Disease presentation can mimic several rheumatologic disorders, delaying the diagnosis. We describe a case of atypical presentation resembling late-onset axial spondylarthritis, later progressing to a systemic inflammatory syndrome with chondritis, cutaneous vasculitis, and transfusion-dependent anemia, requiring high doses of steroids.

Disseminated intravascular coagulation score evolution in 48 h predicts early death in acute promyelocytic leukemia patients.

Introduction: Early death (ED) is the unsolved issue of acute promyelocytic leukemia (APL). The disseminated intravascular coagulation (DIC) score has been proposed as a marker of bleeding and death in APL; whether its temporal evolution predicts outcomes in APL is unknown. We evaluated whether an increasing score 48 h after diagnosis associates with ED.

Predictors of very early death in acute promyelocytic leukemia: a retrospective real-world cohort study.

Early death (ED) is still the major obstacle to cure in acute promyelocytic leukemia (APL). Most studies focus on 30-day ED; however, little is known on predictors of death before starting APL treatment (very early death - VED) and on predictors of 7-day ED, the period with most deaths due to thrombohemorrhagic diathesis. We hypothesized whether the severity of the coagulopathy of APL could predict VED and 7-day ED.

Chronic myeloproliferative diseases.

The chronic myeloproliferative disorders are a group of diseases in which there is an increased proliferation of one or more subtypes of myeloid cells; they include essential thrombocythemia (ET), polycythemia vera (PV), and primary myelofibrosis (PMF). In ET and PV the main neurologic manifestations are headaches, dizziness and macro- and microvascular, both venous and arterial, thrombosis and intracranial hemorrhages. Paresthesias and chorea also occur in PV.

[Biosimilars in oncology].

The development of biotechnology drugs represents one of the great advances in medical therapy and it was observed an exponential growth in its use. The resource to these drugs in Oncology and Hematology is no exception and it soon became an essential element of an integrated and directed therapy strategy. The expiry of the first biotechnology drugs patents has opened the door for the development and marketing of biosimilars, which entry in the Portuguese market was recently approved.

[Use of granulocyte growth factors: recommendations of the Portuguese Society of Hematology].

The administration of cytotoxic chemotherapy may be complicated by the emergence of neutropenia and febrile neutropenia, frequently determining hospital admission and intravenous treatment with broad spectrum antibiotics. Frequently, it is necessary to reduce the dose or to delay the administration of the cytotoxic drugs reducing the relative dose intensity of the chemotherapy regimen. Granulocyte growth factors stimulate the proliferation and differentiation of neutrophils and reduce the number of days of severe neutropenia and febrile neutropenia associated with cytotoxic chemotherapy.

[Immunologic reconstitution after allogeneic stem cell transplant].

Patients submitted to allogeneic stem cell transplantation have a prolonged immunodeficiency, extending beyond one year post-transplant. The immune reconstitution after an allogeneic stem cell transplant is dependent upon several factors, such as the age of the recipient, the degree of in vivo or in vitro T-cell depletion, the emergence and eventual treatment of graft versus host disease. While neutrophils and NK cells recover normally within 2 months of transplant, the reconstitution of B- and T-cell immunity is significantly slower.