A Case of Macrophage Activation Syndrome Developed in Female Adolescent with Systemic Lupus Erythematosus

Seung Woo Keum; Min Jae Kim; E Young Bae; Seung Beom Han; Nack-Gyun Chung; Dae-Chul Jeong; Jin Han Kang

doi:10.4078/jrd.2014.21.2.96

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Keum, Kim, Bae, Han, Chung, Jeong, and Kang: A Case of Macrophage Activation Syndrome Developed in Female Adolescent with Systemic Lupus Erythematosus

Case Report

J Rheum Dis 2014;21(2):96-100.

Published online: 31 October 2014

DOI: https://doi.org/10.4078/jrd.2014.21.2.96

A Case of Macrophage Activation Syndrome Developed in Female Adolescent with Systemic Lupus Erythematosus

Seung Woo Keum, Min Jae Kim, E Young Bae, Seung Beom Han, Nack-Gyun Chung, Dae-Chul Jeong, Jin Han Kang

Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea

Corresponding to: Dae-Chul Jeong, Department of Pediatrics, Seoul St. Mary's Hospital, 222, Banpo-daero, Seocho-gu, Seoul 137-701, Korea. E-mail: dcjeong@catholic.ac.kr

Received 20 January 2013 Revised 20 June 2013 Accepted 20 June 2013

This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Macrophage activation syndrome (MAS) is a severe complication in patients with autoimmune disease. We should consider MAS in patients with autoimmune disease, who present with newly developed fever, and MAS needs proper management due to grave outcome. We report a case of MAS in a 15-year-old adolescent girl, who was newly diagnosed with systemic lupus erythematosus 1 month before the diagnosis of MAS. Her MAS was improved by intensive treatment, including etoposide.

Keywords: Macrophage activation syndrome, Systemic lupus erythematosus, Adolescent

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Figure 1.

Clinical course of an adolescent girl diagnosed with macrophage activation syndrome developed in systemic lupus erythematosus.

Table 1.

Preliminary guidelines for macrophage activation syndrome as a complication of juvenile systemic lupus erythematosus (10)

Clinical Criteria

1. Fever (＞38°C)

2. Hepatomegaly (≥3 cm below the costal arch)

3. Splenomegaly (≥3 cm below the costal arch)

4. Hemorrhagic manifestations (purpura, easy bruising, or mucosal bleeding)

5. CNS dysfunction (irritability, disorientation, lethargy, headache, seizures, or coma)

Laboratory criteria

1. Cytopenia affecting 2 or more cell lineages (WBC count ≤4,000/mm³, Hb ≤9 g/dL, or PLT count ≤150,000/mm³)

2. Increased AST (＞40 U/L)

3. Increased LDH (＞567 U/L)

4. Hypofibrinogenemia (fibrinogen ≤150 mg/dL)

5. Hypertriglyceridemia (triglycerides ＞178 mg/dL)

6. Hyperferritinemia (ferritin ＞500 μ g/L)

Histopathologic criterion

Evidence of macrophage hemophagocytosis in the bone marrow aspirate

The diagnosis of macrophage activation syndrome: at least 1 clinical criterion and at least 2 laboratory criteria. Bone marrow aspiration for evidence of macrophage hemophagocytosis may be required only in doubtful cases. AST: aspartate aminotransferase, CNS: central nervous system, Hb: hemoglobin, LDH: lactate dehydrogenase, PLT: platelet, WBC: white blood cell.

Table 2.

Diagnostic guidelines for hemophagocytic lymphohistiocytosis (11)

1. A molecular diagnosis consistent with hemophagocytic lymphohistiocytosis

2. Fulfilled five or more criteria out of the eight criteria below

1) Fever

2) Splenomegaly

3) Cytopenias (≥2 of 3 lineages in the peripheral blood): neutrophil count ＜1,000/mm³, Hb ＜9 g/dL (in infants ＜4 weeks: Hb ＜10 g/dL), PLT count ＜100,000/mm³

4) Hypertriglyceridemia (fasting triglycerides ≥265 mg/dL) and/or hypofibrinogenemia (fibrinogen ≤150 mg/dL)

5) Hemophagocytosis in bone marrow or spleen or lymph nodes without evidence of malignancy

6) Low or absent NK cell activity (according to local laboratory reference)

7) Ferritin ≥500 μ g/L

8) Soluble CD25 (soluble IL-2 receptor) ≥2,400 U/mL

The diagnosis of hemophagocytic lymphohistiocytosis can be established, if one of either 1 or 2 is fulfilled. Hb: hemoglobin, PLT: platelet.

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