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1.
Eur J Haematol ; 111(5): 706-714, 2023 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-37515415

RESUMEN

BACKGROUND AND OBJECTIVES: Autoimmune myelofibrosis (AIMF) is a rare cause of bone marrow fibrosis (BMF) occurring in the presence or absence of a defined autoimmune disease (secondary or primary AIMF, sAIMF/pAIMF, respectively). Unlike primary myelofibrosis (PMF), AIMF responds well to immunosuppressive therapy with a benign clinical course. Diagnostic criteria for AIMF in opposition to PMF have been lacking, though recent work has helped better characterise molecular and pathological features of AIMF, improving diagnostic precision. METHODS: Using a modern clinical and pathophysiological understanding of AIMF, we apply scoping review methodology and rigorous case-criteria to retrospectively analyse the case literature. We examine its patient-population, describing patient-associated factors, presentation, bone marrow pathology, genetics, treatment and outcomes. RESULTS: Fifty-five studies were identified, describing 139 AIMF patients. Patients were mostly young females (~4:1 ratio female:male, median age 40.8 years) and typically presented with cytopenias. Splenomegaly was rare. sAIMF was more common than pAIMF (~3:1 ratio), and most cases responded well to immunosuppressive therapy. CONCLUSIONS: Our results strengthen the emerging picture of AIMF's patient population, natural history and response to treatment. Further work should continue to use reproducible diagnostic criteria, and explore AIMF's pathophysiology, response to different therapies, and sequelae over larger timescales, as well as differences between pAIMF, sAIMF and PMF.


Asunto(s)
Enfermedades Autoinmunes , Mielofibrosis Primaria , Humanos , Masculino , Femenino , Adulto , Mielofibrosis Primaria/diagnóstico , Mielofibrosis Primaria/terapia , Mielofibrosis Primaria/complicaciones , Estudios Retrospectivos , Médula Ósea/patología , Enfermedades Autoinmunes/diagnóstico , Enfermedades Autoinmunes/terapia , Enfermedades Autoinmunes/complicaciones , Terapia de Inmunosupresión
2.
Cureus ; 16(3): e55796, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38586689

RESUMEN

Gastric diverticula are a rare phenomenon that is typically asymptomatic and encountered incidentally. Due to the relative proximity between the gastric diverticula and the left adrenal gland, they may mimic adrenal masses on computed tomography (CT). For this reason, the preferred diagnostic methods for gastric diverticula are upper gastrointestinal series or direct visualization on endoscopy. The present report describes an unusual case of a gastric diverticulum mimicking an abscess of the adrenal gland with the apparent spread of infection to the left lower lobe of the lung.

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