Figure 1. Bone marrow biopsy showing hemophagocytic macrophages.
| Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access |
| Article copyright, the authors; Journal compilation copyright, J Med Cases and Elmer Press Inc |
| Journal website http://www.journalmc.org |
Case Report
Volume 3, Number 4, August 2012, pages 219-222
Hemophagocytic Syndrome In Association With Systemic Lupus Erythematosus and Rheumatoid Arthritis
Figure
Table
| Diagnosis of HLH is established if either one or both of the criteria below are fulfilled |
|---|
| (1) A molecular diagnosis consistent with HLH |
| (2) Diagnostic criteria for HLH fulfilled (≥ 5 of the 8 criteria below) |
| Fever |
| Splenomegaly |
| Cytopenias affecting ≥ 2 of 3 lineages in peripheral blood: |
| hemoglobin < 90 g/L (in infants < 4 wk: hemoglobin < 100 g/L) |
| platelets < 100 x 109/L |
| neutrophils < 1.0 x 109/L |
| Hypertriglyceridemia and/or hypofibrinogenemia: |
| fasting triglycerides ≥ 3.0 mmol/L (≥ 265 mg/dL) |
| fibrinogen ≤ 1.5 g/L |
| Hemophagocytosis in bone marrow, spleen, lymph nodes or cerebrospinal fluid: |
| no evidence of malignancy |
| Low or absent natural killer cell activity (according to local laboratory reference) |
| Elevated ferritin (≥ 500 µg/L) |
| Soluble CD25 (namely, soluble interleukin-2 receptor) ≥ 2400 U/mL |