J Rheum Dis.  2015 Jun;22(3):180-185. 10.4078/jrd.2015.22.3.180.

Three Cases of Secondary Hemophagocytic Lymphohistiocytosis Associated with Systemic Erythematosus Lupus

• Lim E
• Kim YG
• Choi WS
• Jung YS
• Han JH
• Bae CB
• Jung JY
• Kim HA
• Suh CH

• KMID: 2222884
• DOI: http://doi.org/10.4078/jrd.2015.22.3.180

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder characterized by fever, pancytopenia, hyperferritinemia, and phagocytosis of hematopoietic cells in bone marrow, liver, or lymph nodes. HLH can occur during the course of systemic lupus erythematosus (SLE), but can also be a presenting manifestation. Because development of pancytopenia occurs in less than 10 percent of SLE cases, investigation for HLH is necessary when otherwise unexplained pancytopenia persists despite adequate treatment. We experienced three cases of secondary HLH associated with SLE. Among the three patients, two patients developed HLH during the clinical course of SLE. The other patient who presented with pancytopenia was first diagnosed with HLH, and later with SLE. In her case, HLH turned out to be a presenting manifestation of SLE. We report on three successfully treated cases, and discuss the prevalence, characteristics, treatments, and prognosis of secondary HLH associated with SLE.

Keyword

Hemophagocytic lymphohistiocytosis; Systemic lupus erythematosus; Pancytopenia

MeSH Terms

Bone Marrow
Fever
Humans
Liver
Lupus Erythematosus, Systemic
Lymph Nodes
Lymphohistiocytosis, Hemophagocytic*
Pancytopenia
Phagocytosis
Prevalence
Prognosis

Figure

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  Figure 1. Bone marrow aspirate smear showing macrophage phagocyting (A) neutrophils and platelets and (B) platelets and red blood cells. The border of macrophage is shown in white arrows (Wright stain, ×1,000).

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