Ostalo
Systemic Lupus Erythematosus Progressing to Non-Hodgkin’s Lymphoma Complicated by Fatal Hemophagocytic Syndrome: Case Report
Ivica Jeremić
; Institute of Rheumatology
Slobodanka Đorđević-Kontić
Miloš Nikolić
Mirjana Šefik-Bukilica
Nada Vujasinović-Stupar
Branka Bonači-Nikolić
; Department of Allergy and Clinical Immunology Clinical Centre of Serbia Faculty of Medicine, University of Belgrade Koste Todorovića 2 11000 Belgrade Serbia
Sažetak
Hemophagocytic syndrome (HPS) may be provoked by
infections, malignancies and autoimmune diseases. We report on a
56-year-old woman with long-lasting systemic lupus erythematosus
(SLE) who presented with malar rash, inflammatory livedo reticularis,
fever, weight loss, pancytopenia and mild splenomegaly with cervical
lymphadenopathy. She had criteria for SLE flare-up (malar rash, high
antinuclear antibody titer, complement consumption, pathological
urinary sediment, and retinal vasculitis). Despite high-dose glucocorticoid
therapy, pancytopenia and fever worsened. Important elevations
of triglycerides and ferritin were also found. Bone marrow aspirate
demonstrated hemophagocytosis, which confirmed the coexistence
of HPS and SLE. The treatment with glucocorticoids, immunoglobulins,
cyclophosphamide, filgrastim and antimicrobial therapy was unsuccessful.
After one month, the patient developed Pneumocystis jirovecii
pneumonia with fatal outcome. Bone marrow biopsy, taken 5 days
before death, showed high grade diffuse large B-cell (CD20+, Ki-67+)
non-Hodgkin’s lymphoma (DLBCL). We are the first to report the association
of both SLE and non-Hodgkin’s lymphoma complicated by HPS.
We showed that, based on clinical and laboratory data, it was difficult
to distinguish the early phase of HPS from SLE flare-up and new-onset
DLBCL. Therapy of such a complex case of HPS has not been standardized,
and opportunistic infections remain a difficult issue.
Ključne riječi
hemophagocytic syndrome; systemic lupus erythematosus; lymphoma
Hrčak ID:
89078
URI
Datum izdavanja:
16.4.2012.
Posjeta: 457 *