Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
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Case Report

Volume 3, Number 4, August 2012, pages 219-222


Hemophagocytic Syndrome In Association With Systemic Lupus Erythematosus and Rheumatoid Arthritis

Figure

Figure 1.
Figure 1. Bone marrow biopsy showing hemophagocytic macrophages.

Table

Table 1. Revised Diagnostic Guidelines for Hemophagocytic Lymphohistiocytosis (HLH)
 
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