: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. FerraresiSecondo
;J.A. GiannottaPenultimo
;
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.File | Dimensione | Formato | |
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