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Secondary Hemophagocytic Lymphohistiocytosis and Autoimmune Cytopenias: Case Description and Review of the Literature

Journal

JOURNAL OF CLINICAL MEDICINE
Volume 10, Issue 4, Pages -

Publisher

MDPI
DOI: 10.3390/jcm10040870

Keywords

autoimmune hemolytic anemia; immune thrombocytopenia; hemophagocytic lymphohistiocytosis; diffuse large B cell lymphoma

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HLH is a rare hyperinflammatory condition that can be difficult to diagnose and life-threatening. Patients may present with acute cytopenias, but differential diagnosis with other diseases such as AIC and lymphoproliferative disorders is necessary.
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-specific 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 lymphoproliferative disorders. In the present article, we describe two patients presenting at the emergency 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 cytopenias in the internal medicine setting, providing a literature review of secondary HLH and AIC.

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