TY - JOUR
T1 - Secondary hemophagocytic lymphohistiocytosis and autoimmune cytopenias
T2 - Case description and review of the literature
AU - Fattizzo, Bruno
AU - Ferraresi, Marta
AU - Giannotta, Juri Alessandro
AU - Barcellini, Wilma
N1 - Publisher Copyright:
© 2021 by the authors. Licensee MDPI, Basel, Switzerland.
PY - 2021/2/2
Y1 - 2021/2/2
N2 - :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.
AB - :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.
KW - Autoimmune hemolytic anemia
KW - Diffuse large B cell lymphoma
KW - Hemophagocytic lympho-histiocytosis
KW - Immune thrombocytopenia
UR - http://www.scopus.com/inward/record.url?scp=85105986372&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85105986372&partnerID=8YFLogxK
U2 - 10.3390/jcm10040870
DO - 10.3390/jcm10040870
M3 - Article
AN - SCOPUS:85105986372
VL - 10
JO - Journal of Clinical Medicine
JF - Journal of Clinical Medicine
SN - 2077-0383
IS - 4
M1 - 870
ER -