:Hemophagocytic lymphohistocytosis (HLH) is a rare hyperinflammatory condition which may be primary or secondary to many diseases, including hematologic malignancies. Due to its life-threatening evolution, a timely diagnosis is paramount but challenging, since it relies on non-spe-cific clinical and laboratory criteria. The latter are often altered in other diseases, including autoimmune cytopenias (AIC), which in turn can be secondary to infections, systemic autoimmune or lym-phoproliferative disorders. In the present article, we describe two patients presenting at the emer-gency department with acute AICs subsequently diagnosed as HLH with underlying diffuse large B cell lymphoma. We discuss the diagnostic challenges in the differential diagnosis of acute cytope-nias in the internal medicine setting, providing a literature review of secondary HLH and AIC.

Secondary hemophagocytic lymphohistiocytosis and autoimmune cytopenias: Case description and review of the literature / B. Fattizzo, M. Ferraresi, J.A. Giannotta, W. Barcellini. - In: JOURNAL OF CLINICAL MEDICINE. - ISSN 2077-0383. - 10:4(2021), pp. 870.1-870.11. [10.3390/jcm10040870]

Secondary hemophagocytic lymphohistiocytosis and autoimmune cytopenias: Case description and review of the literature

B. Fattizzo
Primo
;
M. Ferraresi
Secondo
;
J.A. Giannotta
Penultimo
;
2021

Abstract

:Hemophagocytic lymphohistocytosis (HLH) is a rare hyperinflammatory condition which may be primary or secondary to many diseases, including hematologic malignancies. Due to its life-threatening evolution, a timely diagnosis is paramount but challenging, since it relies on non-spe-cific clinical and laboratory criteria. The latter are often altered in other diseases, including autoimmune cytopenias (AIC), which in turn can be secondary to infections, systemic autoimmune or lym-phoproliferative disorders. In the present article, we describe two patients presenting at the emer-gency department with acute AICs subsequently diagnosed as HLH with underlying diffuse large B cell lymphoma. We discuss the diagnostic challenges in the differential diagnosis of acute cytope-nias in the internal medicine setting, providing a literature review of secondary HLH and AIC.
autoimmune hemolytic anemia; immune thrombocytopenia; hemophagocytic lympho-histiocytosis; diffuse large B cell lymphoma
Settore MED/15 - Malattie del Sangue
Article (author)
File in questo prodotto:
File Dimensione Formato  
jcm-10-00870-v3-1.pdf

accesso aperto

Tipologia: Publisher's version/PDF
Dimensione 2.59 MB
Formato Adobe PDF
2.59 MB Adobe PDF Visualizza/Apri
Pubblicazioni consigliate

Caricamento pubblicazioni consigliate

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/904885
Citazioni
  • ???jsp.display-item.citation.pmc??? 3
  • Scopus 3
  • ???jsp.display-item.citation.isi??? 3
social impact