Articole semnate de acelasi autor in Revista Romana de Pediatrie:

Macrophage activation syndrome

Revista Romana de PEDIATRIE | Volumul LXI, Nr. 1, An 2012

ISSN 1454-0398 | e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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Ioan Gherghina, Dumitru Matei, Constantin Pascu, Mirela Covăcescu, Alexis Cochino and Georgiana Nache

REZUMAT

Methods. Short case presentation of a possible macrophage activation syndrome (MAS) secondary to acute viral infections and presentation of a literature review on this subject.

Results. Seven year old boy hospitalized for high fever, chills, and alteration of general state, hepatosplenomegaly, peripheral and central adenomegaly, sudden onset of anemia and thrombocytopenia. Lab results also showed elevated triglycerides and ferritin, moderately high ESR, high CRP, presence of infl uenza B virus in faryngian swab (PCR). Acute myeloid leukemia was excluded on the base of bone marrow biopsy. Treatment was started with 400 mg/kg intravenous immunoglobulin and resulted in normalization of clinical and biological picture, including mesenteric adenopathy remission. MAS secondary to infl uenza B was a diagnosis consistent with the picture of the clinic, biologic and evolution pattern. As described in the literature, MAS represent the macrophage tissue expansion (or of the hystiocites), which is often triggered by infection (EBV, CMV) or changes in drug therapy of chronic rheumatic diseases (Still’s disease, SLE, vasculitic syndromes). Pathogenic mechanism of MAS is characterized by proliferation of macrophages, correlated with decreased cytotoxic function of NK cells and cytotoxic lymphocytes – often determined by mutations in the gene that encodes perforina, PRF1, or mutations in MUNC 13-4, another gene that is involved in the release of perforine into the immune synapse with a target cell.

Conclusions. The occurrence of MAS complicating an underlying disease (vasculitis, SLE) or acute infection is a vital element of aggravation and imminent danger that should be immediately diagnosed and vigorously treated.

Key words: macrophage activation, child

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Ioan Gherghina

SINDROMUL DE ACTIVARE MACROFAGICA

ISSN 1454-0398 | e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

National Awards “Science and Research”

NEW! RJP has announced the annually National Award for "Science and Research" for the best scientific articles published throughout the year in the official journal.

ICMJE- Recommendations

Read the Recommendations for the Conduct, Reporting, Editing, and Publication of Scholarly work in Medical Journals.

Promoting Global Health

Ioan Gherghina, Dumitru Matei, Constantin Pascu, Mirela Covăcescu, Alexis Cochino and Georgiana Nache

REZUMAT

Metodă. Scurtă prezentare de caz a unui sindrom de activare macrofagică (MAS) secundar unei infecţii virale acute şi prezentare a unei revizuiri a literaturii de specialitate pe această temă.

Rezultate. Pacient în vârstă de 7 ani spitalizat pentru febră ridicată, frisoane, alterarea stării generale, hepatosplenomegalie, adenopatii periferice şi centrale, anemie şi trombocitopenie cu debut brusc. Rezultatele de laborator au arătat, de asemenea, trigliceride şi feritină crescute, VSH moderat crescut, CRP crescută, prezenţa virusului gripei B în exsudatul faringian (PCR). Leucemia acută mieloidă a fost exclusă pe baza biopsiei de măduvă osoasă. Tratamentul cu imunoglobuline intravenoase a fost început cu 400 mg/kg şi a dus la normalizarea tabloului clinic şi biologic, inclusiv la remiterea adenopatiei mezenterice. MAS secundar infecţiei cu virusul gripal B a fost un diagnostic în concordanţă cu aspectul clinic, biologic şi evolutiv al cazului. După cum este descris în literatura de specialitate, MAS reprezintă expansiunea ţesutului macrofagic (sau a histiocitelor), care este adesea declanşată de infecţie (EBV, CMV) sau de modificări în terapia unor boli reumatice cronice (boala Still, LES, sindroame vasculitice). Mecanismul patogenic al MAS este caracterizat prin proliferarea macrofagelor, corelată cu scăderea funcţiei citotoxice a celulelor NK şi a limfocitelor citotoxice – adesea determinate de mutaţii ale genei care codifică perforina, PRF1, sau mutaţii in MUNC 13-4, o altă genă care este implicată în eliberarea perforinei în sinapsa imunitară cu o celulă ţintă.

Concluzii. Apariţia MAS ca o complicaţie a unei boli subiacente (vasculită, LES) sau infecţii acute este un element de agravare şi de pericol iminent care ar trebui să fie imediat diagnosticat şi viguros tratat.

Cuvinte cheie: activare macrofagă, copil

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Ioan Gherghina