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.

Full-Dose Azacitidine in 5 Days Versus 7 Days With a Weekend Break in Myelodysplastic Syndromes: A Retrospective Cohort Study.

Introduction: Apart from transplantation, only azacitidine demonstrated a survival benefit in a phase III study in higher-risk myelodysplastic syndromes (MDS). The approved regimen is 75 mg/m/day for 7 consecutive days, imposing a logistic challenge for outpatient weekend administration. Schedules with 5 days and 7 days with a weekend break (5 + 2) have been used for convenience despite the lack of strong scientific support.

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.

Cerebrovascular Complications of Anemia.

Purpose Of The Review: Anemia has been called the fifth cardiovascular risk factor. It is one of the most prevalent pathologies worldwide. In this article, we aimed to perform a narrative review of the main cerebrovascular complications of anemia and its influence on stroke prognosis.

Spontaneous Tumor Lysis Syndrome: A Rare Presentation in Plasmablastic Lymphoma.

Tumor lysis syndrome is an oncological emergency, which can ultimately lead to death if not recognized early and treated accordingly. The institution of adequate prophylactic measures can decrease its incidence and severity; but very rarely, a highly aggressive neoplasm such as acute lymphoblastic leukemia or Burkitt's lymphoma can present with spontaneous tumor lysis syndrome (sTLS). We present the case of a 58-year-old male with newly diagnosed plasmablastic lymphoma with a retroperitoneal bulky mass invading the bladder, who presented with severe sTLS and was admitted to an intensive care unit due to acute renal failure and hyperkalemia requiring emergent renal replacement therapy.

Cerebrovascular manifestations in hematological diseases: an update.

Patients with hematological diseases often experience cerebrovascular complications including ischemic stroke, intracerebral and subarachnoid hemorrhage, microbleeds, posterior reversible encephalopathy syndrome, and dural sinus and cerebral vein thrombosis (CVT). In this update, we will review recent advances in the management of cerebrovascular diseases in the context of myeloproliferative neoplasms, leukemias, lymphomas, multiple myeloma, POEMS, paroxysmal nocturnal hemoglobinuria (PNH), thrombotic thrombocytopenic purpura (TTP), and sickle-cell disease. In acute ischemic stroke associated with hematological diseases, thrombectomy can in general be applied if there is a large vessel occlusion.

Correction to: Update on management and progress of novel therapeutics for R/R AML: an Iberian expert panel consensus.

After publication of this paper, the authors determined that an additional information in the funding section was missing.

Update on management and progress of novel therapeutics for R/R AML: an Iberian expert panel consensus.

A significant proportion of adult patients with acute myeloid leukemia (AML) fail to achieve complete remission or will relapse later on after achieving it. Prognosis for relapsed or refractory (R/R) AML patients remains discouraging, with the main curative option still relying on hematopoietic stem cell transplant (HSCT) for those who are eligible. Beyond morphological bone marrow and peripheral blood assessment, evaluation of patient performance status and comorbidities, as well as genetic/molecular characterization, is crucial to make an accurate diagnosis and prognosis, which will be useful to select the most appropriate treatment.

Osteomyelitis Caused by Carbapenemase-Producing Klebsiella Pneumoniae: A Diagnosis to Consider in Patients with Hematologic Malignancies and Stem Cell Transplant Recipients.

BACKGROUND Osteomyelitis (OM) due to carbapenemase-producing Klebsiella pneumoniae (CPKp) is a very rare but severe condition, particularly among patients with hematologic malignancies and stem cell transplant recipients, who are especially at risk of developing nosocomial infections caused by this bacterium. CASE REPORT We describe 2 cases of acute and chronic OM by CPKp in adults with hematologic disorders. Patient 1, with acute lymphoblastic leukemia, developed bacteremia due to multidrug CPKp after induction chemotherapy.

Complete androgen insensitivity syndrome caused by a novel splice donor site mutation and activation of a cryptic splice donor site in the androgen receptor gene.

The androgen insensitivity syndrome is an X-linked recessive genetic disorder characterized by resistance to the actions of androgens in an individual with a male karyotype. We evaluated a 34-year-old female with primary amenorrhea and a 46,XY karyotype, with normal secondary sex characteristics, absence of uterus and ovaries, intra-abdominal testis, and elevated testosterone levels. Sequence analysis of the androgen receptor (AR) gene revealed a novel splice donor site mutation in intron 4 (c.