Macrophagic Activation Syndrome (MAS) Revealing Systemic Lupus Erythematosus

Sana Aourarh; Fatima Zahra Lazrak; Saida Eddyb; Adil Jahdaoui; Sanae Sayagh; Mustapha Ait Ameur; Mohamed Chakour

doi:doi:10.11648/j.ajlm.20200501.14

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Macrophagic Activation Syndrome (MAS) Revealing Systemic Lupus Erythematosus

Sana Aourarh, Fatima Zahra Lazrak, Saida Eddyb, Adil Jahdaoui, Sanae Sayagh, Mustapha Ait Ameur, Mohamed Chakour

Published in American Journal of Laboratory Medicine (Volume 5, Issue 1)

Received: 26 December 2019 Accepted: 15 January 2020 Published: 10 February 2020

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Abstract

Introduction: Macrophagic activation syndrome (SAM) or hemophagocytic lymphohistiocytosis is an anatomoclinical entity resulting from an inappropriate proliferation and activation of macrophagic cells. This rare but serious syndrome can be primary or secondary to certain pathologies dominated by infections and neoplasias. More rarely, it is secondary to connectivitis, in particular to systemic lupus erythematosus (LES). The aim of this work is to report the case of SAM revealing a systemic lupus erythematosus and to discuss the triggering factors. Observation: A 27-year-old patient who presents to the emergency room for arthralgia evolving for a week in a context of fever and deterioration of the general state hospitalized in the nephrology department for bicytopenia and functional renal failure. The hemogram showed anemia at 10.7 g/dL, normocytic, normochromic, aregenerative (reticulocytes at 18 G/L), hyperleukocytosis at 10.5 G/L and platelets at the lower limit of normal at 158 G/L (338 G/L in September 2006). In view of argenerative anemia, a myelogram was performed showing the presence of hemophagocytosis figures. The remainder of the biological examination revealed hyperferritinaemia at 1017 ng/mL with an increase in LDH 930 U/Let a CRP at 18.15 mg/L. The patient was transferred to the internal medicine department for etiological assessment, clinical and paraclinical examinations showed arthritis with deep ADP and pleural mass, leuconeutropenia with native DNA and positive antinuclear antibodies (ANA) as well as the coombs test which was positive, The diagnosis of SLE was retained in her on the criteria of the ACR.

Published in	American Journal of Laboratory Medicine (Volume 5, Issue 1)
DOI	10.11648/j.ajlm.20200501.14
Page(s)	28-31
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2020. Published by Science Publishing Group

Keywords

Hemophagocytic Lymphohistiocytosis, Systemic Lupus Erythematosus, Autoimmunity

References

[1]	Imashuku S. Differeiîtial diagnosis of heinophagocytic syndrome: underlying disorders and selection of the most effective treatment. Int J Hematol. 1997; 66: 135-5 1.
[2]	Kumakura S., Ishikura H., Kondo M., Murakawa Y., Masiida J., Kobayashi S. Autoimmune-associated lieinopliagocytic syndrome. Mod Rheumatol. 2004; 14: 205-15.
[3]	Dhote R., Simon J., Papo T., Detournay B., Sailler L., Andre M. H., et al. Reactive hemophagocytic syndrome in adult systeinic disease: report of twenty-six cases aiid literature review. Arthritis Rheuin. 2003; 49: 633-9.
[4]	Papo T., Andre M. H., Amoura Z., Lortholary O., Triboiit B., Guillevin L., et al. The spectrum of reactive heinophagocytic syndroine in systeinic lupus erythematosus. J Rheumatol. 1999; 26: 927-30.
[5]	Berrady R, Bono W, Le syndrome d’activation lympho-histiocytaire (SALH), Annales Françaises d’Anesthésie et de Réanimation 33 (2014) 26–32.
[6]	Wong KF, Chan JK. Reactive hemophagocytic syndrome – a clinicopathologic study of 40 patients in an Oriental population. Am J Med 1992; 93: 177–80.
[7]	Karras A., Hermine O. Syndrome d'activation inacrophagiq~ie. Rev Med Interne. 2002; 23: 768-78.
[8]	Wong K. F., Chan J. K. Reactive hemophagocytic syiîdroine-a cliiîicopatliologic study of 40 patients in Oriental population. Ain J Med. 1992; 93: 177-80.
[9]	Tsuda H. Hemophagocytic syndroine (HPS) in children and adults. Int J Heinatol. 1997; 65: 215-26.
[10]	Tiab M., Mechinaud F., Hamidou M., Gaillard F., Raffi F., Harousseau J. L. Syndromes hémophagocytaires: une série de 23 observations. Ann Med Interiie (Paris). 1996; 147: 138-44.
[11]	Henter J. l., Elinder G., Soder O., Ost A. Incidence in Sweden and clinical features of familial heinophagocytic lymphohistiocytosis. Acta Paediatr Scand. 1991; 80: 428-35.
[12]	Michot M, Hiéc M, Galicier L, Lambotte O, Michel M, Bloch-Queyratf C, Herminef O, Le syndrome d’activation lymphohistiocytaire de l’adulte, La Revue de médecine interne 34 (2013).
[13]	Kaito K, Kobayashi M, Katayama T, Otsubo H, Ogasawara Y, Sekita T, et al. Prognostic factors of hemophagocytic syndrome in adults: analysis of 34 cases. Eur J Haematol 1997; 59: 247–53.
[14]	Eminenegger U., Reiiners A., Frey U., Fux C., Bihl F., Seinela D., et al. Reactive macrophage activation syndrome: a simple screening strategy and its potential in early treatment initiation. Swiss Med Wkly. 2002; 132: 230-6.
[15]	Tsuda H., Shirono K. Ser~iinlipids in adult patients with hemophagocytic syndroine. Am J Hematol. 1996; 53: 285.
[16]	de Kergiienec C., Hillaire S., Molinie V., Gardin C., Degott C., Erlinger S., et al. Hepatic manifestations of heinophagocytic syndrome: a stiidy of 30 cases. Ain J Gastroenterol. 2001; 96: 852-7.
[17]	Janka GE, Hemophagocytic syndromes, Blood Rev 2007; 21: 245–53.

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Sana Aourarh, Fatima Zahra Lazrak, Saida Eddyb, Adil Jahdaoui, Sanae Sayagh, et al. (2020). Macrophagic Activation Syndrome (MAS) Revealing Systemic Lupus Erythematosus. American Journal of Laboratory Medicine, 5(1), 28-31. https://doi.org/10.11648/j.ajlm.20200501.14

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ACS Style

Sana Aourarh; Fatima Zahra Lazrak; Saida Eddyb; Adil Jahdaoui; Sanae Sayagh, et al. Macrophagic Activation Syndrome (MAS) Revealing Systemic Lupus Erythematosus. Am. J. Lab. Med. 2020, 5(1), 28-31. doi: 10.11648/j.ajlm.20200501.14

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AMA Style

Sana Aourarh, Fatima Zahra Lazrak, Saida Eddyb, Adil Jahdaoui, Sanae Sayagh, et al. Macrophagic Activation Syndrome (MAS) Revealing Systemic Lupus Erythematosus. Am J Lab Med. 2020;5(1):28-31. doi: 10.11648/j.ajlm.20200501.14

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@article{10.11648/j.ajlm.20200501.14,
  author = {Sana Aourarh and Fatima Zahra Lazrak and Saida Eddyb and Adil Jahdaoui and Sanae Sayagh and Mustapha Ait Ameur and Mohamed Chakour},
  title = {Macrophagic Activation Syndrome (MAS) Revealing Systemic Lupus Erythematosus},
  journal = {American Journal of Laboratory Medicine},
  volume = {5},
  number = {1},
  pages = {28-31},
  doi = {10.11648/j.ajlm.20200501.14},
  url = {https://doi.org/10.11648/j.ajlm.20200501.14},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajlm.20200501.14},
  abstract = {Introduction: Macrophagic activation syndrome (SAM) or hemophagocytic lymphohistiocytosis is an anatomoclinical entity resulting from an inappropriate proliferation and activation of macrophagic cells. This rare but serious syndrome can be primary or secondary to certain pathologies dominated by infections and neoplasias. More rarely, it is secondary to connectivitis, in particular to systemic lupus erythematosus (LES). The aim of this work is to report the case of SAM revealing a systemic lupus erythematosus and to discuss the triggering factors. Observation: A 27-year-old patient who presents to the emergency room for arthralgia evolving for a week in a context of fever and deterioration of the general state hospitalized in the nephrology department for bicytopenia and functional renal failure. The hemogram showed anemia at 10.7 g/dL, normocytic, normochromic, aregenerative (reticulocytes at 18 G/L), hyperleukocytosis at 10.5 G/L and platelets at the lower limit of normal at 158 G/L (338 G/L in September 2006). In view of argenerative anemia, a myelogram was performed showing the presence of hemophagocytosis figures. The remainder of the biological examination revealed hyperferritinaemia at 1017 ng/mL with an increase in LDH 930 U/Let a CRP at 18.15 mg/L. The patient was transferred to the internal medicine department for etiological assessment, clinical and paraclinical examinations showed arthritis with deep ADP and pleural mass, leuconeutropenia with native DNA and positive antinuclear antibodies (ANA) as well as the coombs test which was positive, The diagnosis of SLE was retained in her on the criteria of the ACR.},
 year = {2020}
}

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TY  - JOUR
T1  - Macrophagic Activation Syndrome (MAS) Revealing Systemic Lupus Erythematosus
AU  - Sana Aourarh
AU  - Fatima Zahra Lazrak
AU  - Saida Eddyb
AU  - Adil Jahdaoui
AU  - Sanae Sayagh
AU  - Mustapha Ait Ameur
AU  - Mohamed Chakour
Y1  - 2020/02/10
PY  - 2020
N1  - https://doi.org/10.11648/j.ajlm.20200501.14
DO  - 10.11648/j.ajlm.20200501.14
T2  - American Journal of Laboratory Medicine
JF  - American Journal of Laboratory Medicine
JO  - American Journal of Laboratory Medicine
SP  - 28
EP  - 31
PB  - Science Publishing Group
SN  - 2575-386X
UR  - https://doi.org/10.11648/j.ajlm.20200501.14
AB  - Introduction: Macrophagic activation syndrome (SAM) or hemophagocytic lymphohistiocytosis is an anatomoclinical entity resulting from an inappropriate proliferation and activation of macrophagic cells. This rare but serious syndrome can be primary or secondary to certain pathologies dominated by infections and neoplasias. More rarely, it is secondary to connectivitis, in particular to systemic lupus erythematosus (LES). The aim of this work is to report the case of SAM revealing a systemic lupus erythematosus and to discuss the triggering factors. Observation: A 27-year-old patient who presents to the emergency room for arthralgia evolving for a week in a context of fever and deterioration of the general state hospitalized in the nephrology department for bicytopenia and functional renal failure. The hemogram showed anemia at 10.7 g/dL, normocytic, normochromic, aregenerative (reticulocytes at 18 G/L), hyperleukocytosis at 10.5 G/L and platelets at the lower limit of normal at 158 G/L (338 G/L in September 2006). In view of argenerative anemia, a myelogram was performed showing the presence of hemophagocytosis figures. The remainder of the biological examination revealed hyperferritinaemia at 1017 ng/mL with an increase in LDH 930 U/Let a CRP at 18.15 mg/L. The patient was transferred to the internal medicine department for etiological assessment, clinical and paraclinical examinations showed arthritis with deep ADP and pleural mass, leuconeutropenia with native DNA and positive antinuclear antibodies (ANA) as well as the coombs test which was positive, The diagnosis of SLE was retained in her on the criteria of the ACR.
VL  - 5
IS  - 1
ER  -

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Author Information

Sana Aourarh

Department of Laboratory of Hematology of Faculty of Medicine, University Cadi Ayyad, University Hospital Mohammed VI, Marrakech, Morocco
Fatima Zahra Lazrak

Department of Laboratory of Hematology of Faculty of Medicine, University Cadi Ayyad, University Hospital Mohammed VI, Marrakech, Morocco
Saida Eddyb

Department of Laboratory of Hematology of Faculty of Medicine, University Cadi Ayyad, University Hospital Mohammed VI, Marrakech, Morocco
Adil Jahdaoui

Department of Laboratory of Hematology of Faculty of Medicine, University Cadi Ayyad, University Hospital Mohammed VI, Marrakech, Morocco
Sanae Sayagh

Department of Laboratory of Hematology of Faculty of Medicine, University Cadi Ayyad, University Hospital Mohammed VI, Marrakech, Morocco
Mustapha Ait Ameur

Department of Laboratory of Hematology of Faculty of Medicine, University Cadi Ayyad, Military Hospital Avicenna, Marrakech, Morocco
Mohamed Chakour

Department of Laboratory of Hematology of Faculty of Medicine, University Cadi Ayyad, Military Hospital Avicenna, Marrakech, Morocco

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APA Style

Sana Aourarh, Fatima Zahra Lazrak, Saida Eddyb, Adil Jahdaoui, Sanae Sayagh, et al. (2020). Macrophagic Activation Syndrome (MAS) Revealing Systemic Lupus Erythematosus. American Journal of Laboratory Medicine, 5(1), 28-31. https://doi.org/10.11648/j.ajlm.20200501.14

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ACS Style

Sana Aourarh; Fatima Zahra Lazrak; Saida Eddyb; Adil Jahdaoui; Sanae Sayagh, et al. Macrophagic Activation Syndrome (MAS) Revealing Systemic Lupus Erythematosus. Am. J. Lab. Med. 2020, 5(1), 28-31. doi: 10.11648/j.ajlm.20200501.14

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AMA Style

Sana Aourarh, Fatima Zahra Lazrak, Saida Eddyb, Adil Jahdaoui, Sanae Sayagh, et al. Macrophagic Activation Syndrome (MAS) Revealing Systemic Lupus Erythematosus. Am J Lab Med. 2020;5(1):28-31. doi: 10.11648/j.ajlm.20200501.14

Copy | Download

@article{10.11648/j.ajlm.20200501.14,
  author = {Sana Aourarh and Fatima Zahra Lazrak and Saida Eddyb and Adil Jahdaoui and Sanae Sayagh and Mustapha Ait Ameur and Mohamed Chakour},
  title = {Macrophagic Activation Syndrome (MAS) Revealing Systemic Lupus Erythematosus},
  journal = {American Journal of Laboratory Medicine},
  volume = {5},
  number = {1},
  pages = {28-31},
  doi = {10.11648/j.ajlm.20200501.14},
  url = {https://doi.org/10.11648/j.ajlm.20200501.14},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajlm.20200501.14},
  abstract = {Introduction: Macrophagic activation syndrome (SAM) or hemophagocytic lymphohistiocytosis is an anatomoclinical entity resulting from an inappropriate proliferation and activation of macrophagic cells. This rare but serious syndrome can be primary or secondary to certain pathologies dominated by infections and neoplasias. More rarely, it is secondary to connectivitis, in particular to systemic lupus erythematosus (LES). The aim of this work is to report the case of SAM revealing a systemic lupus erythematosus and to discuss the triggering factors. Observation: A 27-year-old patient who presents to the emergency room for arthralgia evolving for a week in a context of fever and deterioration of the general state hospitalized in the nephrology department for bicytopenia and functional renal failure. The hemogram showed anemia at 10.7 g/dL, normocytic, normochromic, aregenerative (reticulocytes at 18 G/L), hyperleukocytosis at 10.5 G/L and platelets at the lower limit of normal at 158 G/L (338 G/L in September 2006). In view of argenerative anemia, a myelogram was performed showing the presence of hemophagocytosis figures. The remainder of the biological examination revealed hyperferritinaemia at 1017 ng/mL with an increase in LDH 930 U/Let a CRP at 18.15 mg/L. The patient was transferred to the internal medicine department for etiological assessment, clinical and paraclinical examinations showed arthritis with deep ADP and pleural mass, leuconeutropenia with native DNA and positive antinuclear antibodies (ANA) as well as the coombs test which was positive, The diagnosis of SLE was retained in her on the criteria of the ACR.},
 year = {2020}
}

Copy | Download

TY  - JOUR
T1  - Macrophagic Activation Syndrome (MAS) Revealing Systemic Lupus Erythematosus
AU  - Sana Aourarh
AU  - Fatima Zahra Lazrak
AU  - Saida Eddyb
AU  - Adil Jahdaoui
AU  - Sanae Sayagh
AU  - Mustapha Ait Ameur
AU  - Mohamed Chakour
Y1  - 2020/02/10
PY  - 2020
N1  - https://doi.org/10.11648/j.ajlm.20200501.14
DO  - 10.11648/j.ajlm.20200501.14
T2  - American Journal of Laboratory Medicine
JF  - American Journal of Laboratory Medicine
JO  - American Journal of Laboratory Medicine
SP  - 28
EP  - 31
PB  - Science Publishing Group
SN  - 2575-386X
UR  - https://doi.org/10.11648/j.ajlm.20200501.14
AB  - Introduction: Macrophagic activation syndrome (SAM) or hemophagocytic lymphohistiocytosis is an anatomoclinical entity resulting from an inappropriate proliferation and activation of macrophagic cells. This rare but serious syndrome can be primary or secondary to certain pathologies dominated by infections and neoplasias. More rarely, it is secondary to connectivitis, in particular to systemic lupus erythematosus (LES). The aim of this work is to report the case of SAM revealing a systemic lupus erythematosus and to discuss the triggering factors. Observation: A 27-year-old patient who presents to the emergency room for arthralgia evolving for a week in a context of fever and deterioration of the general state hospitalized in the nephrology department for bicytopenia and functional renal failure. The hemogram showed anemia at 10.7 g/dL, normocytic, normochromic, aregenerative (reticulocytes at 18 G/L), hyperleukocytosis at 10.5 G/L and platelets at the lower limit of normal at 158 G/L (338 G/L in September 2006). In view of argenerative anemia, a myelogram was performed showing the presence of hemophagocytosis figures. The remainder of the biological examination revealed hyperferritinaemia at 1017 ng/mL with an increase in LDH 930 U/Let a CRP at 18.15 mg/L. The patient was transferred to the internal medicine department for etiological assessment, clinical and paraclinical examinations showed arthritis with deep ADP and pleural mass, leuconeutropenia with native DNA and positive antinuclear antibodies (ANA) as well as the coombs test which was positive, The diagnosis of SLE was retained in her on the criteria of the ACR.
VL  - 5
IS  - 1
ER  -

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